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                                  THE
                          CASE HISTORY SERIES


                       CASE HISTORIES IN MEDICINE
                                   BY
                         RICHARD C. CABOT, M.D.
                  Third edition, revised and enlarged


                      CASE HISTORIES IN PEDIATRICS
                                   BY
                        JOHN LOVETT MORSE, M.D.
                  Second edition, revised and enlarged


                     ONE HUNDRED SURGICAL PROBLEMS
                                   BY
                         JAMES G. MUMFORD, M.D.
                            Second Printing


                      CASE HISTORIES IN NEUROLOGY
                                   BY
                           E. W. TAYLOR, M.D.
                            Second Printing


                      CASE HISTORIES IN OBSTETRICS
                                   BY
                      ROBERT L. DENORMANDIE, M.D.
                             Second Edition


                  CASE HISTORIES IN DISEASES OF WOMEN
                                   BY
                         CHARLES M. GREEN, M.D.

                             NEUROSYPHILIS
               MODERN SYSTEMATIC DIAGNOSIS AND TREATMENT
        Presented in one hundred and thirty-seven Case Histories
                                   BY
                      E. E. SOUTHARD, M.D., SC.D.
                                  AND
                          H. C. SOLOMON, M.D.

    Being Monograph Number Two of the Psychopathic Hospital, Boston,
  Massachusetts. (Monograph Number One was A Point Scale for Measuring
    Mental Ability by Robert M. Yerkes, James W. Bridges and Rose S.
       Hardwick. Published by Warwick and York. Baltimore 1915.)

[Illustration: METCHNIKOFF WASSERMANN EHRLICH SCHAUDINN NOGUCHI]




                             NEUROSYPHILIS
               MODERN SYSTEMATIC DIAGNOSIS AND TREATMENT
        PRESENTED IN ONE HUNDRED AND THIRTY-SEVEN CASE HISTORIES


                                    BY

                       E. E. SOUTHARD, M.D., Sc.D.,

       Bullard Professor of Neuropathology, Harvard Medical School;
   Pathologist, Massachusetts Commission on Mental Diseases; Director,
 Psychopathic Department, Boston State Hospital; Vice-President, American
                     Medico-Psychological Association

                                   AND

                           H. C. SOLOMON, M.D.,

 Instructor in Neuropathology and in Psychiatry, Harvard Medical School;
   Special Investigator in Brain Syphilis, Massachusetts Commission on
 Mental Diseases; Acting Chief-of-Staff, Psychopathic Department, Boston
                              State Hospital


                         WITH AN INTRODUCTION BY

                       JAMES JACKSON PUTNAM, M.D.,

  Professor Emeritus of Diseases of the Nervous System, Harvard Medical
                                  School


           BY VOTE OF THE TRUSTEES OF THE BOSTON STATE HOSPITAL

                           MONOGRAPH NUMBER TWO

                                  OF THE

               PSYCHOPATHIC HOSPITAL BOSTON, MASSACHUSETTS


                                  BOSTON

                         W. M. LEONARD, PUBLISHER

                                   1917




                           _Copyright, 1917.
                           By W. M. Leonard_




                                  =In=

                             MASSACHUSETTS

                              A STATE THAT

                       BOTH TOLERATES AND FOSTERS

                                RESEARCH

------------------------------------------------------------------------




                                PREFACE


This book is written primarily for the general practitioner and
secondarily for the syphilographer, the neurologist, and the
psychiatrist. Our material is drawn chiefly from a psychopathic
hospital, that modern type of institution in which the mental problems
of general medical practice come to a diagnostic head weeks, months, or
years before the asylum is thought of.

It is this peculiar nature of psychopathic hospital material—a
concentrated essence of the most difficult daily problems of general
practice—that brings together such an apparent _mélange_ of cases as are
here described, ranging from mild single-symptom diseases like
extraocular palsy up to genuine magazines of symptoms as in general
paresis; from feeblemindedness, apparently simple, up to apparently
simple dotage, both feeblemindedness and dotage really syphilitic; from
the mind-clear tabetic to the maniacal or deluded subject who looks
physically perfectly fit; from the early secondaries to the late
tertiaries or so-called quaternaries; from peracute to the most chronic
of known conditions; from the most delicate character changes to the
profoundest ruin of the psyche.

Although the bulk of our case-material is drawn from general practice
through the thinnest of intermediary membranes, the psychopathic
hospital, yet we have tried to depict the whole story by presenting
enough autopsied cases from district state hospitals to show exactly
what treatment has to face. Nor have we hesitated to insert cases in
which treatment has failed.

In addition to (_a_) the Psychopathic Hospital, Boston, group of
incipient, doubtful, obscure, or complicated cases (the early clinical
group) and (_b_) the Danvers State Hospital, Hathorne, group of
longer-standing, committed, fatal cases (the finished or autopsied
group) we present (_c_) a miscellaneous group of cases, including many
from private neurological or psychiatric practice. No doubt those
familiar with Boston medicine will see traces of the teaching of our
former chiefs, notably Professors James Jackson Putnam and Edward Wyllys
Taylor. We are obliged to them for some well-observed cases.

We have dedicated our work to the Commonwealth, but perhaps we should
more specifically ascribe to the Massachusetts Commission on Mental
Diseases (formerly the State Board of Insanity) the spirit that
permitted our special study of neurosyphilis treatment. To these
authorities, who have countenanced and encouraged a somewhat costly
piece of special work since 1914, we offer our thanks, hoping that other
states will be one by one stimulated to the state-endowment of research.
States doing full duty by research can be counted on one hand.

To our Psychopathic Hospital colleagues and the internes, and especially
to Drs. Myrtelle M. Canavan and Douglas A. Thom of the Commission’s
Pathological Service, we also offer our best thanks.

The Danvers traditions are tangible here: cases of Drs. A. M. Barrett,
H. A. Cotton, H. W. Mitchell, H. M. Swift, and others are presented. We
have been especially aided by the more recent work of Dr. Lawson G.
Lowrey.

Nor should we have been able to present our samples of brain correlation
without drawing on the collection arranged and analyzed by Dr. Annie E.
Taft, Custodian, Harvard Department of Neuropathology. The photographs,
part of a collection of brain photographs now numbering over 10,000
representing 700 brains of all sorts, were made by Mr. Herbert W.
Taylor.

The Wassermann testing work has been done by Dr. W. A. Hinton of the
State Board of Health. Dr. Hinton himself wrote out the text description
of the Wassermann method. The method of his laboratory is held to the
standards of control set by previous chiefs, viz. by Professor F. P.
Gay, who brought immunological methods direct from the laboratory of
Bordet (whose method the Wassermann method essentially is), Prof. W. P.
Lucas, and the late Dr. Emma W. D. Mooers, who had assisted Plaut in his
first work with the Wassermann method in Kraepelin’s Munich Clinic.

The material combed by us to secure this illustrative series amounts to
over 2000 cases of syphilis of the nervous system, including over 100
autopsies in all types of case. We have presented these with very
varying fulness, chiefly to illustrate the contentions at the heads of
the case-descriptions.

In using the book, we suggest early reference to the Summary and Key,
where for convenience are placed numerous cross-references permitting
extended illustration of almost every proposition from several cases.

We have not made a large feature of the Medicolegal and Social section.
This kind of thing well deserves a volume by itself, with all the legal
and social-service implications drawn out in their amazing richness and
detail. The social service slogan, “A paretic’s child is a syphilitic’s
child” has already accomplished a great deal of good in our local world.
Some day we may not be compelled to _drive_ the paretic’s spouse and
offspring to the Wassermann serum test! The general practitioner must
help here.

A note on the Treatment section. This is manifestly not the last word or
even, we hope, our own last word, since the systematic work of the
Massachusetts Commission must be kept up for some years to get a
reliable verdict. Some of the results give rise to greater optimism than
has prevailed in asylum circles, especially re general paresis. We are
confident that _no one can now successfully make a differential
diagnosis between the paretic and the diffuse non-paretic forms of
neurosyphilis in many phases of either disease_, even with all
laboratory refinements. If this be so, it is _improper not to give the
full benefits of modern treatment to all cases in which the diagnosis
remains doubtful_ between the paretic and the diffuse non-paretic forms
of neurosyphilis. We ourselves advocate modern treatment, not only in
the diffuse, but also in early paretic forms of neurosyphilis.

It would have been out of place in a book in this Case History Series to
have dealt extensively with the history of our topic. We have
compensated inadequately for this lack by a few remarks at the head of
the Summary and Key. We are, like all others in the field, under the
inevitable obligation to Nonne of Hamburg, whose great work has gone
into three editions, the second of which has appeared in English
translation (Nonne’s Syphilis of the Nervous System, C. R. Ball,
translator). Mott’s work, embodied in a large volume of the Power-Murphy
System of Syphilis, has also been attentively consulted, as well as the
various systematic works on neurology and psychiatry. The topic of
Neurosyphilis is getting wide and appropriate attention in this country
through special journals, both those dealing with nervous and mental
diseases, and those dealing with syphilis. Syphilis is in a sense the
making of psychiatry and will go far to pushing psychiatry into general
practice.

At the last moment we have been led to deviate from our plan of
presenting only local cases familiar and accessible to us. In a section
on Neurosyphilis and the War, we present excerpts and digests of
English, French, and German cases of neurosyphilis that have appeared in
association with the war. Our own country has not suffered greatly as
yet either from the lighting up of neurosyphilis under martial stress or
from the immediate or remote effects of syphilis obtained in the unholy
congress of Mars and Venus. Space forbids a large collection of these
martial cases, but, as will be seen, a fair sample of problems is
presented.

Speaking for the moment as the senior author of this book, I wish to say
that, were it not for the energy, industry, and ingenuity of the junior
author, Dr. H. C. Solomon, the book would not have been written. Nor, in
all probability, would the systematic work of the Commonwealth on
neurosyphilis and its treatment ever have been begun. I can also accord
the highest praise to Mrs. Maida Herman Solomon for her social-service
work in this new field.

Perhaps, in closing, we owe an apology to John Milton for our borrowings
from the two Paradises. Had he known much about syphilis, Milton might
have written still stronger mottoes for us.

                                                          E. E. SOUTHARD

    74 FENWOOD ROAD
  _Boston, Massachusetts_




                           TABLE OF CONTENTS


                                                                    PAGE

 SECTION I. THE NATURE AND FORMS OF SYPHILIS OF THE NERVOUS SYSTEM
   (NEUROSYPHILIS). CASES 1 TO 8                                      17

 CASE

   1. Paradigm: protean symptoms, nervous and mental. Autopsy, with
        meningeal, parenchymatous, and vascular lesions.              17

   2. Tabes dorsalis (tabetic neurosyphilis). Autopsy                 31

   3. General paresis (paretic neurosyphilis). Autopsy                37

   4. Cerebral thrombosis (vascular neurosyphilis). Autopsy           42

   5. Juvenile paresis (juvenile paretic neurosyphilis). Autopsy      45

   6. Extraocular palsy (focal meningeal neurosyphilis). Autopsy      50

   7. Gumma of brain (gummatous neurosyphilis). Autopsy               53

   8. _Meningitis hypertrophica cervicalis_ (gummatous
        neurosyphilis). Autopsy                                       56


 SECTION II. THE SYSTEMATIC DIAGNOSIS OF THE FORMS OF NEUROSYPHILIS
   CASES 9 TO 38                                                      63

 CASE

   9. Neurasthenia _versus_ neurosyphilis                             63

  10. Paretic neurosyphilis _versus_ manic-depressive psychosis       68

  11. Neurosyphilis _versus_ manic-depressive psychosis               71

  12. Dementia praecox _versus_ neurosyphilis. Autopsy                74

  13. Neurosyphilis: negative Wassermann reaction (W. R.) of serum    77

  14. Diffuse neurosyphilis: six tests apt to run mild                80

  15. Paretic neurosyphilis: six tests strong                         85

  16. Taboparesis (tabetic neurosyphilis): tests like those of
        paresis                                                       92

  17. Paretic _versus_ diffuse neurosyphilis: confusion _re_ tests    97

  18. Vascular neurosyphilis: positive serum, negative fluid W. R.   101

  19. Seizures in diffuse neurosyphilis                              103

  20. Seizures in paretic neurosyphilis                              106

  21. Aphasia in paretic neurosyphilis                               111

  22. Aphasia in paretic neurosyphilis                               115

  23. Remission in paretic neurosyphilis                             117

  24. Remission in diffuse neurosyphilis                             122

  25. _Paresis sine paresi_                                          126

  26. Paretic neurosyphilis. Autopsy                                 131

  27. Gummatous neurosyphilis. Operation                             137

  28. Extraocular palsy (cranial neurosyphilis)                      140

  29. Tabes dorsalis (tabetic neurosyphilis): six tests apt to run
        mild                                                         141

  30. Tabetic neurosyphilis, clinically atypical                     143

  31. Cervical tabes                                                 146

  32. Erb’s syphilitic spastic paraplegia                            147

  33. Syphilitic muscular atrophy                                    149

  34. Neurosyphilis of the secondary period                          151

  35. Juvenile paretic neurosyphilis: optic atrophy                  154

  36. Juvenile paretic neurosyphilis                                 157

  37. Simple feeblemindedness, syphilitic                            159

  38. Juvenile tabes                                                 161

 SECTION III. PUZZLES AND ERRORS IN THE DIAGNOSIS OF NEUROSYPHILIS
   (INCLUDING NON-SYPHILITIC CASES). CASES 39–82                     165

 CASE

  39. Paretic _versus_ diffuse neurosyphilis. Autopsy                165

  40. Paretic _versus_ vascular neurosyphilis, cerebellar. Autopsy   169

  41. Paretic _versus_ vascular neurosyphilis, cerebellar. Autopsy   172

  42. Tabetic combined with vascular neurosyphilis. Autopsy.         175

  43. Tabetic neurosyphilis: mental symptoms, non-paretic. Autopsy   177

  44. Cerebral gliosis. Autopsy                                      180

  45. Neurasthenia _versus_ neurosyphilis                            183

  46. Hysteria. Neurosyphilis of the secondary period                185

  47. Manic-depressive psychosis _versus_ paretic neurosyphilis      187

  48. Cerebral tumor                                                 190

  49. Early post-infective paretic neurosyphilis                     192

  50. Atypical paretic neurosyphilis, hemitremor. Autopsy            197

  51. Paretic neurosyphilis. Autopsy                                 199

  52. Manic-depressive psychosis _versus_ paretic neurosyphilis      202

  53. Syphilitic(?) exophthalmic goitre. Autopsy                     205

  54. Argyll-Robertson pupils                                        209

  55. Argyll-Robertson pupils: pineal tumor. Autopsy                 212

  56. Neurosyphilis(?) with negative spinal fluid                    216

  57. Disseminated syphilitic encephalitis, seven months
        post-infective. Autopsy                                      218

  58. “Pseudoparesis”                                                222

  59. Syphilitic paranoia?                                           225

  60. Paretic neurosyphilis _versus_ alcoholic pseudoparesis         227

  61. Alcoholic pseudoparesis _versus_ paretic neurosyphilis         231

  62. Alcoholic neuritis and paretic neurosyphilis                   234

  63. Chronic alcoholism _versus_ paretic neurosyphilis              236

  64. Neurosyphilis, diabetic pseudoparesis, or brain tumor          238

  65. Neurosyphilis and diabetes                                     240

  66. Neurosyphilis: hemianopsia                                     242

  67. Paretic neurosyphilis _versus_ syphilis and cerebral malaria   245

  68. Paretic neurosyphilis: gold sol test “syphilitic.” Autopsy     247

  69. Lues maligna                                                   250

  70. Neurosyphilis _versus_ multiple sclerosis                      253

  71. Atypical neurosyphilis                                         256

  72. Huntington’s chorea _versus_ neurosyphilis                     258

  73. Senile arteriosclerotic psychosis _versus_ neurosyphilis       262

  74. Hysterical fugue _versus_ neurosyphilis                        264

  75. Tabetic neurosyphilis _versus_ pernicious anemia               267

  76. Congenital neurosyphilis                                       270

  77. Congenital _versus_ paretic neurosyphilis                      272

  78. Juvenile paretic neurosyphilis                                 275

  79. Epilepsy _versus_ juvenile neurosyphilis                       277

  80. Addison’s disease and juvenile paretic neurosyphilis. Autopsy  279

  81. Neurosyphilis of the secondary period                          283

  82. Taboparetic neurosyphilis and typhoid meningitis. Autopsy      284


 SECTION IV. NEUROSYPHILIS, MEDICOLEGAL AND SOCIAL. CASES 83–98      289

 CASE

  83. A public character, neurosyphilitic. Autopsy                   289

  84. Debts, neurosyphilitic                                         295

  85. Suicidal attempt by a neurosyphilitic                          296

  86. Neurosyphilis and juvenile delinquency                         298

  87. Neurosyphilis in a defective delinquent                        300

  88. _Paresis sine paresi_ in a forger                              303

  89. Trauma: juvenile paretic neurosyphilis                         306

  90. Trauma: paretic neurosyphilis                                  308

  91. False claim for trauma: neurosyphilis                          309

  92. Traumatic exacerbation? in neurosyphilis                       310

  93. Trauma: cranial gumma at the site of injury                    311

  94. Occupation-neurosis _versus_ syphilitic neuritis               312

  95. Character change: neurosyphilis                                314

  96. A neurosyphilitic family                                       316

  97. A neurosyphilitic’s normal-looking family                      318

  98. The neurosyphilitic’s marriage                                 319


 SECTION V. THE TREATMENT OF NEUROSYPHILIS. CASES 99–123.

      (CASES 99–103 SHOW THE VARIETY OF STRUCTURAL LESIONS THAT
        TREATMENT HAS TO FACE)                                       323

 CASE

  99. An incurable spastic paresis in paretic neurosyphilis.
        Autopsy                                                      323

 100. A theoretically curable case. Autopsy                          328

 101. A highly meningitic case, theoretically amenable to
        treatment. Autopsy                                           332

 102. A highly atrophic case, theoretically not amenable to
        treatment. Autopsy                                           335

 103. Paretic neurosyphilis with markedly focal lesions. Autopsy     338

      (CASES 104 TO 123 ARE EXAMPLES OF TREATMENT INCLUDING
        SUCCESSES AND FAILURES.)

 104. Diffuse neurosyphilis: treatment successful after nine months  342

 105. Atypical neurosyphilis: treatment successful                   346

 106. Argyll-Robertson pupil not necessarily of bad prognosis:
        treated case an insurance risk                               350

 107. Spinal fluid cleared: symptoms persistent                      355

 108. Arteriosclerosis does not contraindicate treatment             359

 109. Symptoms of intracranial pressure relieved by treatment        362

 110. Therapeutic improvement in tabetic neurosyphilis               366

 111. W. R. rendered negative in tabetic neurosyphilis               367

 112. Example of successful treatment of paretic neurosyphilis       370

 113. Another example                                                372

 114. Clinical recovery but tests persistently positive in treated
        paretic neurosyphilis                                        375

 115. Improvement delayed in treated paretic neurosyphilis           377

 116. Non-neural syphilis in treated paretic neurosyphilis           380

 117. Partial recovery in treated paretic neurosyphilis              382

 118. Laboratory signs improved: clinical situation stationary:
        treated paretic neurosyphilis                                384

 119. Another example                                                386

 120. Failure of treatment                                           388

 121. Treatment, at first mild, later intensive                      390

 122. Intensive treatment                                            392

 123. Syphilitic feeblemindedness improved by treatment              395


 SECTION VI. NEUROSYPHILIS AND THE WAR.

      CASES A TO N FROM BRITISH, FRENCH, AND GERMAN WRITERS
        (1914–1916)                                                  399

 CASE

   A. Tabes “shell-shocked” into paresis? (Donath)                   401

   B. Latent syphilis “shell-shocked” into tabes? (Duco and Blum)    403

   C. Aggravation of neurosyphilis by service? (Weygandt)            404

   D. Aggravation of neurosyphilis _by_ service? (Todd)              406

   E. Aggravation of neurosyphilis _on_ service? (Todd)              409

   F. Duration of neurosyphilitic process important. (Farrar)        411

   G. Latent syphilis lighted up to paresis by war stress without
        shell-shock. (Marie)                                         412

   H. Paresis lighted up by “gassing”? (de Massary)                  414

   I. Epilepsy in a neuropath lighted up by syphilis acquired at
        war. (Bonhoeffer)                                            415

   J. Syphilitic—after Dixmude epileptic. (Bonhoeffer)               417

   K. Syphilitic root-sciatica in a fireworks man. (Dejerine, Long)  418

   L. Paresis lighted up in civilian by domestic stress of the war.
        (Percy Smith)                                                420

   M. Shell-shock pseudoparesis. (Pitres and Marchand)               421

   N. Shell-shock pseudotabes. (Pitres and Marchand)                 424


 SECTION VII. SUMMARY AND KEY                                        427

 APPENDICES:

   A. The six tests                                                  471

   B. Common methods of treatment                                    486




                              INTRODUCTION


It is a privilege to be allowed to write a word of introduction to a
textbook which so richly fulfils its function as does this volume on the
manifold disorders classified under Neurosyphilis, a subject of which
the importance for the welfare of society is found to loom the larger
the more deeply its mysteries are probed.

The case histories with which its pages are so amply stocked are
carefully analyzed in accordance with a broadly chosen plan, and the
generalizations that precede and follow them are obviously based on a
wide and varied personal experience such as alone could render a
familiarity with the literature of the subjects treated adequate to its
best usefulness. Both writers were indeed well adapted for this task.
Dr. Southard, as everyone is aware, has long been a highly
conscientious, ardent and productive worker in the department of
pathological anatomy, and of late years a careful student of clinical
diagnosis and methods, both at the Danvers State Hospital and still
more, at the Psychopathic Hospital which he worked so hard to found;
while Dr. Solomon’s researches, in the special field of neurosyphilis,
have been of the highest order.

Undoubted as are the merits of the case-system of instruction that has
been so much in vogue in recent years, and excellent as is the modern
supplementation of this method by the use of published records, the
danger is still real that the student will have presented to him a
picture of nature in disease that is too diagrammatic, too concise, with
the result that while the task of memory is lightened through simplified
formulation, the training of the doubting and inquiring instincts is
often given too little stimulus and scope. In this book this danger is
deliberately met through the casting of emphasis rather on the
pluralistic aspects of the processes at stake than (primarily) on their
unitary aspects.

The student who utilizes this volume cannot but emerge from his study a
more thoughtful person than he was at the period of his entry. He will
have seen that clinical rules of thumb cannot be followed to advantage,
and that, on the contrary, surprises are to be expected and prepared
for. Let the recognition of this fact, if it seems to increase the
difficulties in the way of diagnosis, not lead to pessimism in that
respect, or to hopelessness in therapeutics. On the contrary the
writers’ bias is towards the worth-whileness of clinical efforts and an
increased respect for accuracy and thoroughness in the utilization of
modern methods of research. The chance is indeed held open that even the
gaunt spectre of “General Paresis” may prove to be less terrible than it
seems, and for this hope good grounds are given.

It is in this way made clear, on the strength of anatomical evidence of
much interest, that even if in the treatment of a given patient, the
time arrives when a fatal or unfavorable result seems manifestly
foreshadowed, it may be still worth while to renew the treatment with
fresh zeal, for the sake of combatting some symptom or exacerbation, for
which a locally fresh process furnishes the cause.

Another noteworthy principle here emphasized and illustrated is that the
relationship between “functional” (hysterical, neurasthenic, migrainoid)
symptoms and the signs (or symptoms) of organic processes is clinically
important and worthy of much further study. This is a matter which, in a
general sense, has interested me for many years. Above and over the
“organic” hovers always the “functional,” as representing the first
indication of the marvelous tendency to repair, or substitution, for
which the resources of nature are so vast. Yet this functional tendency
also has its laws, of which, in their turn, the organic processes
display the action in quasi diagrammatic form. Hysteria, neurasthenia,
migraine, etc., do not arise _de novo_ in each case, but conform to
typical, though not rigid, formulas, susceptible of description. I have
recently had the opportunity to study in detail an analogous series of
transitions between the movements (and emotions) indicative of
apparently purposeless myoclonic movements (on an epileptoid basis) and
the movements of surprise, engrossment, purposeful effort, the
excitement and joy by which the former were excited and into which they
shaded over.

Taken altogether, this book represents work and thought in which, for
amount and kind, the neurologists of Boston may take just pride.

                                                        JAMES J. PUTNAM.

  ST. HUBERT’S, KEENE VALLEY, NEW YORK.
                  _August, 1917._




        Me miserable! which way shall I fly
        Infinite wrath and infinite despair?
        Which way I fly is Hell; myself am Hell;
        And, in the lowest deep, a lower deep
        Still threatening to devour me opens wide,
        To which the Hell I suffer seems a Heaven.

                            Paradise Lost, Book IV, lines 73–78.




       I. THE NATURE AND FORMS OF SYPHILIS OF THE NERVOUS SYSTEM
                            (NEUROSYPHILIS)


  PARADIGM to show possible abundance and variety of symptoms and
  lesions in DIFFUSE NEUROSYPHILIS (“cerebrospinal syphilis”).
  Autopsy.


=Case 1.= Mrs. Alice Morton[1] was in the hands of at least five
well-known specialists in different branches of medicine and surgery
during the nineteen years of her disease. It appears that she acquired
syphilis upon marriage at the age of 23 to a man who later became
tabetic and acknowledged syphilitic infection previous to marriage. Mrs.
Morton remained without children and there were no miscarriages.

At the age of 27, she developed iritis, paresis of the left eye muscles,
and ulceration of the throat, with destruction of the uvula. The
syphilitic nature of her disease was at once recognized and the
classical treatment was given, although, through numerous shifts in
consultants, this treatment was never pushed to the limit. At 28 Mrs. M.
began to suffer from severe headaches resembling migraine and
accompanied by attacks of paræsthesia; at 35, came severe pains in the
back and difficulty in walking.

At 36, the migraine attacks began to be accompanied by blurring of
vision and dizziness. The difficulty in walking became extreme,
affecting particularly the right foot. The legs became spastic, there
were pains and hyperæsthesia of the chest, and severe cramps of the
legs. Antisyphilitic treatment at this time yielded marked improvement.

During her thirty-sixth year, Mrs. M. sustained curious transient losses
of vision and of hearing. She was also irritable, and at this time
developed her first pronounced mental symptoms, namely, delusions
concerning her relatives. There were also a few seizures of an
epileptiform nature.

At 38 there was a spell of total deafness, followed by improvement. The
eye muscles were also subject to a variable involvement with intervening
spells of improvement. The _knee-jerks were lost, but after a time
returned_ in less pronounced form. Shortly, an absolute paralysis and
extensive decubitus developed, and death occurred at 39.

The autopsy is briefly summarized below, but it is important in the
understanding of Mrs. M.’s case (particularly some of the sensory
symptoms and the transiency of certain symptoms) to consider the
pre-infective history. Although there seems to be no doubt that the
patient acquired syphilis at about 23 years of age from a syphilitic
husband, who himself later became tabetic, yet it is of note that the
patient was the only child of parents, both of whom also suffered from
mental disease. Mrs. M.’s father died of what was called softening of
the brain (one should avoid terming _all_ old cases of _so-called_
“softening of the brain” syphilitic, since the older diagnosticians did
not always distinguish between non-syphilitic arteriosclerotic effects
and syphilitic disease). Mrs. M.’s mother also died insane (confusion
and emotional depression). It is clear, then, that we do not need to
suppose that every symptom shown by Mrs. M. is directly due to
destructive or irritative lesions immediately due to the spirocheta
pallida. The case is, in fact, an excellent lesson as to the association
of structural and functional effects in neuropathological cases.

Mrs. M. as a child had shown talent, but was somewhat nervous and
eccentric. At one time, she had an attack of hysterical dysphasia; at
another time, an attack of hysterical dyspnea; during another period, an
apparent obsession (kicking the mopboard at regular intervals).
Moreover, she had for years suffered from migraines of a severe and
unusual type. Both the hysterical tendency and the migrainous tendency
became mingled with the results of the neurosyphilis in later stages of
the disease in such wise that it was hard to tell exactly where the
structural phenomena left off and the functional phenomena began.

For example, at the age of 32, nine years after infection and four years
after the earliest nerve symptoms traceable to syphilis, and at about
the time of the onset of spinal cord symptoms, an attack was described
as follows:

    The patient had a very severe attack of migraine (?) yesterday,
    preceded and accompanied by paraphasia, so severe that for three
    hours she was unable to make herself understood, and indeed felt “as
    if her ideas were getting away from her.” This attack was ushered in
    by a numbness of the forefinger and thumb of the right hand, which
    lasted for about three hours, though the earlier attacks had lasted
    for only about ten minutes. During this period the hand felt as if
    it had been frozen and the loss of muscular power was so great that
    she was unable to hold objects in the hand. In some of the attacks
    this paræsthesia has affected the entire left half of the body, and
    occasionally the right half. Sometimes the seizures come on with
    great suddenness, so that once, when she was attacked while in the
    middle of the street, she had considerable difficulty in reaching
    the sidewalk. After the worst part of the attack is over a certain
    amount of paraphasia may persist for some days, together with
    awkwardness in the use of the right hand and numbness. She has had a
    great deal of nausea and vomiting, without reference to the taking
    of food.[2]

Bearing in mind the mingling of structural with functional symptoms in
this case, let us consider the autopsy findings.


                              =ANATOMICAL=

                        =FORMS OF NEUROSYPHILIS=

              AUTONOMIC (SYMPATHETIC) NEUROSYPHILIS?

              PERIPHERAL NEUROSYPHILIS

              CENTRAL NEUROSYPHILIS
                MENINGEAL
                VASCULAR
                PARENCHYMATOUS
                MENINGOVASCULAR
                VASCULOPARENCHYMATOUS
                DIFFUSE ( = MENINGOVASCULOPARENCHYMATOUS)

              GUMMA

                                                               CHART 1


                   =CLINICAL FORMS OF NEUROSYPHILIS=

                      =HEAD AND FEARNSIDES, 1914=

                  SYPHILIS MENINGOVASCULARIS
                    CEREBRAL FORMS
                    HEMIPLEGIA
                    AFFECTION OF THE CRANIAL NERVES
                    MUSCULAR ATROPHY
                    LATERAL AND COMBINED DEGENERATIONS
                    EPILEPSY

                  SYPHILIS CENTRALIS
                    DEMENTIA PARALYTICA
                    TABES DORSALIS
                    MUSCULAR ATROPHY
                    OPTIC ATROPHY
                    GASTRIC CRISES
                    EPILEPTIC MANIFESTATIONS

                                                               CHART 2


=Peripheral neurosyphilis=: The lesions of the cranial nerves were
characteristically asymmetrical. Whereas the left third nerve looked
entirely normal, the =right third nerve= had its diameter reduced
two-thirds. On the other hand, the fourth nerves were equal and
apparently normal. The sensory portion of the left fifth nerve was
normal; the right fifth nerve was normal. The =right sixth nerve= agreed
with the right third nerve in being atrophic, and was in fact reduced to
a mere thread without contained nerve fibres at a point 2 mm. from its
superficial origin. Although the right third nerve was atrophic, it was
the =left seventh and eighth nerves= which had become atrophic; the
process had spared the right seventh and eighth nerves. The remainder of
the cranial nerves were grossly normal, except that the =optic nerves=
had an outer zone of a translucent nature. So far, no spirochetes have
been demonstrated in any portion of the nervous system of this case, but
such asymmetrical and focal cranial nerve lesions are perhaps due to
local spirochetal infection, punctuating (as it were) the diffuse
process.

How much of the transient blindness, deafness, and ocular paralysis can
be explained on the anatomical findings in these nerves? Possibly a
portion of the phenomena can be so explained. Thus, the mechanical
conditions of pressure inside and outside these nerves, both in their
peripheral course and in their passage through the membranes, can be
readily understood to differ during the acute and subacute inflammation,
during the process of repair in the pial tissues, and during the process
of overgrowth of neuroglia tissue about the superficial origins of the
nerves. Of course, the majority of lesions of these nerves were entirely
extinct at the time of the autopsy, and their history could be surmised
only from the appearances in the _left eighth nerve_. Here occurred a
sharply marked focal area of gliosis with apparently total destruction
of nerve fibres and related with a _lymphocytosis_ of the investing
membrane (one of the few areas of lymphocytosis found anywhere in this
case).

If it were not for the pre-infective history, the hysterical dysphasia
and dypsnea, the youthful obsessions, the migrainous tendency, and the
psychopathic inheritance, we might be tempted to try to explain the
transient blindness, the deafness, and ocular palsies on the basis of
mechanical and toxic variations in the conditions of the peripheral
cranial nerves. The existence of a trace of lymphocytosis in the left
eighth nerve leads to the hypothesis that treatment might still be
effective in this particular region (see below in discussion of spinal
symptoms).

=Spinal neurosyphilis:= Not only the spinal cord but also the posterior
and anterior nerve roots exhibited severe lesions. These lesions were
both meningeal and parenchymatous. The meningeal process differed in its
intensity in different parts of the spinal cord, being severest in the
thoracic region. At one point in this region, the dura mater was so
firmly attached to the pia mater that the line of demarcation between
the two membranes was hard to make out. In fact, it seems clear that
there could have been no free intercommunication between the spinal
fluid above these adhesions of dura to pia mater and the spinal fluid
below the adhesions. Accordingly, it seems that _lumbar puncture_, had
it been practised in this case, _would have failed to show features
representative of the whole cerebrospinal fluid system_. Moreover, since
at no point in this region of adhesions or in the pia mater of the
spinal cord below this point, were found any lymphocytes, it seems clear
that the ordinary lumbar puncture would have failed to reveal a
pleocytosis. Whether this fluid would have yielded a positive globulin
and excess albumin test, it is now impossible to say; but it appears
that the process in the lower part of the spinal cord was to all intents
and purposes extinct.

However, there was one region of more severe inflammatory involvement.
The _spinal cord in the cervical region showed a lymphocyte
infiltration_ of its vessels amounting to a mild myelitis (meaning,
thereby, an inflammatory process of the spinal cord remote from the pia
mater). Moreover, in this region, there was, besides the perivascular
infiltration of the substance, also an infiltration of the overlying
membranes themselves, especially in and near the posterior root zones.

The lessons of this finding are several: The inflammatory process in
this case does not appear to have been entirely extinct! Can we not
suppose that treatment might still have benefited this local
inflammation (perivascular infiltration of the cervical spinal cord
substance and overlying lymphocytic meningitis)? Can we not also picture
the gradual ascent of the inflammatory lesions from lower segments to
higher segments and possibly conceive of the gradual elevation of the
zone of hyperæsthesia manifested in this case as following the gradual
displacement upward of the lymphocytic process? Are there spirochetes in
this tissue? So far none have been discovered, possibly through
inaccuracies of available technique. To the neuropathologist, however,
the lesion looks like a local reaction to organisms.

In addition to the spinal meningitis, chronic and acute, as above
described, there were extensive parenchymatous spinal lesions.

In the first place, the meningitis had affected practically all the
posterior roots so that the explanation of the posterior column
sclerosis of this case is clear. The meningitis had apparently been so
marked, also, that all the fibres anywhere near the periphery of the
spinal cord had been likewise destroyed. The posterior columns and the
posterior root zones were markedly sclerotic; or as we say (having
reference to the overgrowth of neuroglia tissue) gliotic. But there was
as much sclerosis (gliosis) of the lateral columns (particularly in the
posterior two-thirds) as there was in the posterior columns and root
zones. In fact, the entire posterior half or two-thirds of the spinal
cord markedly outstripped the anterior portions of the cord in the
severity of the gliosis (sclerosis) shown.

But although we can explain the posterior column sclerosis, the
sclerosis of the posterior root zones and the marginal sclerosis
(_Randsklerose_) round the entire periphery of the cord, on the basis of
long-standing effects of old meningitis, we cannot thus explain another
finding, namely, the destruction of the fibres in the lateral columns.
This, in fact, is explained through lesions (mentioned below) that
affected the encephalon. The net result of all these lesions of the
spinal cord was to leave only the gray matter and a small amount of
surrounding fibres (belonging to short tracts uniting nearby segments)
intact. Briefly stated, =every long tract in the spinal cord appeared
upon examination to be extensively degenerated=. The genesis of this
parenchymatous loss was, however, double, being in part due to a local
meningeal process (sometimes known as “perimeningitis”) and in part due
to a cutting off of the pyramidal tract fibres on both sides by lesions
higher up in the nervous system.

[Illustration:

  CASE I. SPINAL CORD (THREE LEVELS) SHOWING:

  A. Marginal sclerosis—effect of old meningitis now extinct.
  B. Posterior column sclerosis—effect of meningitis about posterior
    roots also now extinct.
  C. Bilateral pyramidal tract sclerosis—effect of cerebral thrombotic
    lesions.

  Note distortion of tissues in B and C, partly artificial (tissues in
    places diffluent).
]


                          =ANATOMICAL FORMULAE=

               =MENINGOVASCULOPARENCHYMATOUS INVOLVEMENT=

   M, V, P, or Combinations Applied to the Classification of Head and
                               Fearnsides

  I. SYPHILIS MENINGOVASCULARIS
     CEREBRAL FORMS                                     M or V or MV[3]
     HEMIPLEGIA                                         V
     AFFECTION OF THE CRANIAL NERVES                    M
     MUSCULAR ATROPHY                                   M
     LATERAL AND COMBINED DEGENERATIONS                 M
     EPILEPSY                                           M or V

 II. SYPHILIS CENTRALIS
     DEMENTIA PARALYTICA                                MVP or VP
     TABES DORSALIS                                     MP
     MUSCULAR ATROPHY                                   P
     OPTIC ATROPHY                                      P
     GASTRIC CRISES                                     (M? or) P?

 EPILEPTIC MANIFESTATIONS                               P?

                                                                 CHART 3


Can we offer any explanation of the =partial return of knee-jerks= after
their temporary total loss at a certain period of the disease? We may
assume that the knee-jerks were functionally lost about a year before
the death of the patient through the partial or even almost complete
destruction of the entering posterior root fibres at that level of the
spinal cord which is directly related with the knee-jerk. The later
partial return of the knee-jerks apparently requires us to suppose the
maintenance of some fibres and collaterals by which a functional
connection can be effected between the fibres of the posterior roots and
the anterior horn cells which innervate the quadriceps femoris. Let us
now suppose that _pari passu_ with the actual return of the knee-jerks,
the destructive processes that are affecting both pyramidal tracts high
up in the nervous system are now advancing. It is clear that, whatever
inhibitory influence these pyramidal tracts have been exerting up to
this time upon the knee-jerk reflex arc, that influence is now to be
decidedly reduced in amount and possibly absolutely lost. Upon the loss
of such inhibitory influences exerted from above, the few persisting
connections of the posterior roots and anterior horn cells are now
permitted to resume their functions.

=Encephalic neurosyphilis=: The lesions mentioned above as causing
destruction of the pyramidal tracts of the spinal cord were
symmetrically destructive and atrophic lesions of the gray matter of
both corpora striata with atrophy of the anterior segments of the
internal capsules. There was a degenerative process of the corpus
callosum especially affecting the forceps minor of the tapetum. The
ventricles were largely dilated, indicating a considerable destruction
and atrophy of the white matter in general.

After the above discussion of the possible effects of pyramidal tract
lesion in this case, it is unnecessary further to discuss the paraplegia
produced by the cystic lesions of the corpora striata. The theorist
might inquire how these cystic lesions are produced: whether by vascular
blocking or by toxic effects of the accumulations of spirochetes.
Evidence is lacking which would completely sustain either hypothesis.
Still, we do know that lesions almost identical in appearance may be
produced by the necrosis consequent to the plugging of nutritive vessels
in an organ like the brain supplied with end arteries. Therefore, it is
probable that most pathologists would believe these lesions of the
corpora striata to be produced by vascular plugging of the nature of
thrombosis.

It is worth while to note that there was a suggestion of foci of
encephalitis made out upon the gross examination. The cortex in general
showed strikingly few lesions. However, the convolutions did show in
places numerous ill-defined areas of hyperemia and slight swelling.
These areas were of irregular distribution and only a few mm. or cm. in
diameter. No gross vascular lesions were demonstrable in connection with
these focal areas. Microscopically, however, venous plugs of
polymorphonuclear leucocytes were found, and the local hyperemias were
found to be largely due to venous congestion. However, very few
polymorphonuclear leucocytes were found outside the blood vessels.

The white matter of numerous convolutions showed microscopically certain
pale spots suggestive of an early atrophic process. Very possibly these
represent a general tendency in the cerebrum to the same process of
parenchymatous loss which had proceeded to such a marked degree in the
spinal cord.

There was a single large so-called cyst of softening in the cerebellum
(1.5 mm. across by 0.5–7.5 cm. in depth).

How far can we explain the symptoms of this case on the basis of these
encephalic lesions? We can offer no correlation with the cerebellar
lesion; and possibly this lack of correlation is to be expected on
account of its failure to affect the vermis. As to the cystic lesions of
the corpora striata, their effect in producing paraplegia at the close
of life is obvious, and their possible relation to the partial return of
knee-jerks has been discussed. Literally amazing was the comparative
integrity of the cortical gray matter of this case when the spinal cord
and the interior structures of the encephalon had been subjected to such
severe and numerous lesions. The only mental symptoms noted in the case
were sundry delusions directed against the patient’s relatives and a
certain optimism which led the patient to cling as if with an obsession
to the belief that in the end she would get well.


     =VARIOUS FORMS OF NEUROSYPHILIS COLLECTED FROM SEVERAL SOURCES=

                      =MENINGEAL NEUROSYPHILIS (M)=

 GUMMA OF DURA MATER                                                 M
 GUMMATOUS MENINGITIS (PIAL)                                         M
 SYPHILITIC MENINGITIS (PIAL)                                        M
 SYPHILITIC CRANIAL NERVE PALSIES (PRIMARILY PIAL)                   M
 SYPHILITIC BULBAR PALSY                                             M
 SYPHILITIC ROOT NEURITIS                                            M
 SYPHILITIC TRANSVERSE MYELITIS                                      M
 SYPHILITIC NEURITIS (SOME CASES BY EXTENSION)                       M
 SYPHILITIC EPILEPSY (SOME CASES)                                    M
 SYPHILITIC MUSCULAR ATROPHY (SOME CASES)                            M

                      =VASCULAR NEUROSYPHILIS (V)=

 SYPHILITIC ARTERIOSCLEROSIS                                         V
 SYPHILITIC CEREBRAL THROMBOSIS                                      V
 SYPHILITIC APOPLEXY                                                 V
 ANEURYSM                                                            V
 SYPHILITIC EPILEPSY                                                 V

                   =PARENCHYMATOUS NEUROSYPHILIS (P)=

 GUMMA                                                               P
 CEREBROSPINAL SCLEROSIS                                             P
 SYPHILITIC PARANOIA                                                 P?
 SYPHILITIC CHOREA                                                   P
 SYPHILITIC EPILEPSY                                                 P
 TABETIC PSYCHOSIS                                                   P?
 SYPHILITIC MUSCULAR ATROPHY                                         P
 SYPHILITIC NEURITIS                                                 P

                                                                CHART 4A


                  =MENINGOVASCULAR NEUROSYPHILIS (MV)=

 CEREBRAL SYPHILIS                                                   MV
 CEREBROSPINAL SYPHILIS                                              MV
 SYPHILITIC EPILEPSY                                                 MV

               =MENINGOPARENCHYMATOUS NEUROSYPHILIS (MP)=

 CEREBRAL SYPHILIS                                                   MP
 CEREBROSPINAL SYPHILIS                                              MP
 TABES DORSALIS                                                      MP
 ERB’S SYPHILITIC SPASTIC SPINAL PALSY                               MP

               =VASCULOPARENCHYMATOUS NEUROSYPHILIS (VP)=

 CEREBRAL SYPHILIS                                                   VP
 CEREBROSPINAL SYPHILIS                                              VP
 PARETIC NEUROSYPHILIS (GENERAL PARESIS)                             VP
 LISSAUER’S GENERAL PARESIS                                          VP

           =MENINGOVASCULOPARENCHYMATOUS NEUROSYPHILIS (MVP)=

 CEREBRAL SYPHILIS                                                   MVP
 CEREBROSPINAL SYPHILIS                                              MVP
 PARETIC NEUROSYPHILIS                                               MVP
 TABOPARESIS                                                         MVP

           =DOUBTFUL (TOXIC?, IRRITATIVE?) NEUROSYPHILIS (?)=

 “PARESIS SINE PARESI”
 SYPHILITIC NEURASTHENIA
 TABETIC PSYCHOSIS
 SYPHILITIC PARANOIA
 SYPHILITIC POLYURIA, POLYDIPSIA
 SYPHILITIC NEURALGIA

                                                                CHART 4B


=Summary:= We have here dealt at length with a long-standing DIFFUSE
NEUROSYPHILIS affecting to some extent the entire =meninges= and
producing a destruction of posterior column fibres and numerous other
fibres of the spinal cord (=tabetiform= portion of the neurosyphilis
=picture=). We have also found central lesions of the corpora striata
affecting the destruction of both pyramidal tracts (=paraplegic= portion
of the neurosyphilis =picture=). We have found evidences of acute
inflammation (=lymphocytosis=) in the cervical region of the spinal cord
and in the left eighth nerve (=progressive inflammatory= neurosyphilis
=picture=). In short, we have presented a case of =diffuse=
(meningovasculoparenchymatous) =neurosyphilis= characterized by an
ascending character in a course of at least 16 years; we have indicated
a number of possible clinical correlations, not only with the major
portion of the clinical course (symptoms of myelitis and pyramidal tract
destruction), but we have also mentioned, merely for their suggestive
value, a number of finer correlations between histological findings and
certain clinical features (notably transient losses of vision and
hearing, and a partial return of the lost knee-jerks). Bearing in mind
the clinical and anatomical findings of this case, we shall be able to
discuss the cases that follow in a briefer and more condensed fashion.


  =TABETIC NEUROSYPHILIS (“tabes dorsalis,” “locomotor ataxia”)
  complicated by vascular neurosyphilis (hemiplegia). Autopsy.=


=Case 2.= Francis Garfield had been a successful lumberman and had
enjoyed good health until his forty-fifth year. Suddenly one day, while
walking on the street, Garfield lost the use of his legs and for a time
was quite unable to walk. However, he recovered locomotion and after a
time there was nothing wrong with his leg movements except a slight
ataxia.

At the age of 52 Garfield had to give up work. It appears that he had
been becoming cranky, sometimes, for example, shouting, whistling and
slamming doors, apparently to annoy the family. His intellectual
capacity seemed to be maintained, although his memory was slightly
impaired.

At 67 years there was an ill-defined seizure, followed a few days later
by another seizure with aphasia (wrong words used and lack of
understanding of things said).

For years Garfield had been totally deaf in the right ear (following
explosion of a gun?). Now, however, the left ear also showed a sensory
impairment. Slight slurring of speech had been noticed first in the
sixty-sixth year.

=Physically= there was a slightly enlarged heart with accentuated second
aortic sound and irregular rhythm. =Neurologically=, inability to stand
or walk; marked ataxia in his leg movements; upper extremities quite
well controlled; the pupils were small and unequal, the left being
larger than the right; although the reactions were difficult to test,
the pupils seemed to react slightly to direct light stimuli; the
knee-jerks were absent; tests for sensibility so far as could be
determined did not show any abnormalities; there was much complaint of
sharp pains in the legs.

There is no doubt that we are here dealing with a case of TABES DORSALIS
plus certain complications due to VASCULAR LESIONS. The case went on to
death from rupture of =aortic aneurysm= (also doubtless a syphilitic
complication). The death occurred at 71, four years after admission to
Danvers Hospital.


                     =MAIN FORMS OF NEUROSYPHILIS=

                    =(CLASSIFICATION OF THIS BOOK)=

  DIFFUSE NEUROSYPHILIS
    (non-vascular forms of “cerebral,” “spinal” and “cerebrospinal
       syphilis”)

  VASCULAR NEUROSYPHILIS
    (“cerebral arteriosclerosis,” “cerebral thrombosis”)

  PARETIC NEUROSYPHILIS
    (“general paresis”)

  TABETIC NEUROSYPHILIS
    (“tabes dorsalis”)

  GUMMATOUS NEUROSYPHILIS
    (“gumma of membranes, of brain”)

  JUVENILE NEUROSYPHILIS
    (paretic, tabetic, diffuse)

                                                               CHART 5


                         =POSSIBLE INVOLVEMENT=

                        =BRAIN AND CORD SYPHILIS=

                  [M]embranes, [V]essels, [P]arenchyma

 [MVP]   EARLY, LATENT?, SYMBIOSIS?, ATTENUATION?....
 MVP     CEREBRAL, CEREBROSPINAL SYPHILIS, PARESIS                   MVP
 [M]VP   PARESIS; SYPHILITIC ARTERIOSCLEROSIS                        VP
 M[V]P   ?SYPHILOTOXIN FROM MENINGITIS                               MP
 MV[P]   SYPHILITIC MENINGITIS; CEREBRAL OR CEREBROSPINAL SYPHILIS   MV
 [MV]P   SYPHILOTOXIC ATROPHY OR SCLEROSIS                           P
 M[VP]   SYPHILITIC MENINGITIS                                       M
 [M]V[P] SYPHILITIC ARTERIOSCLEROSIS                                 V

              M, V or P in brackets [] means not involved.

                                                                 CHART 6


                            =NEUROSYPHILIS=

                              =SIX TESTS=

                        BLOOD WASSERMANN
                        SPINAL FLUID WASSERMANN
                        SPINAL FLUID CYTOLOGY
                        SPINAL FLUID GLOBULIN
                        SPINAL FLUID ALBUMIN
                        SPINAL FLUID GOLD SOL

                                                               CHART 7


This case has been especially worked up and published by Dr. A. M.
Barrett on account of the fact that the vascular lesions of the brain
had produced a condition of pure word-deafness. Reference is made to the
Journal of Nervous and Mental Disease, Vol. 37, 1910, for a complete
description of the brain findings and an analysis of the word-deafness,
a summary of which is as follows:

    “Reaction to Words and Sounds.—Total deafness to words spoken, but
    gives attention to sounds; no ability to recognize meaning of sounds
    heard; no ability to repeat words heard. Spontaneous
    Speech.—Retained ability to speak spontaneously, with rare
    paraphasic utterances; occasional inability to speak readily the
    word desired, but later always giving the correct reaction;
    calculation fair; spelling good except for occasional paraphasia;
    spelling good for words pronounced. Reaction to Things Seen.—Objects
    correctly recognized and named except for an occasional paraphasic
    reply; mistakes in pronunciation not recognized; correct color
    recognition. Reaction to Things Felt.—Good for familiar objects; an
    occasional paraphasic reply. Reaction to Words Seen.—Reads printing
    and writing understandingly; unimpaired reading except for an
    occasional paraphasic reply; meaning of familiar signs recognized;
    slight difficulty in readily understanding meaning of arithmetical
    signs. Writing.—Spontaneous writing and drawing ability retained;
    ataxia (tabetic) in writing movements; no ability to write from
    dictation. Internal language.—No evidence of impairment.”

The brain post mortem showed severe atheromatous degeneration of the
arteries at the base of the brain. Both middle cerebral arteries showed
scattered atheromatous patches. The pia mater was transparent and
delicate, except in the regions of both Sylvian fissures. There were
residuals of old softening in both temporal lobes. In the fresh brain
the regions of the right and left first temporal convolutions were
sunken inward, and the pia intimately adherent to the softened areas.
The limits and more exact localizing of these softenings were worked out
from serial sections.

Barrett found in his serial sections that, although the transverse
temporal convolutions of the left hemispheres were intact, these
convolutions were undermined throughout their entire extent by
degenerations in the fibres of the center of the first temporal
convolution. Barrett, accordingly, regarded his case as essentially a
case of subcortical tissue destruction. He agrees with various authors
that the pure word-deafness of his case is the result of an isolation of
the receiving station in the transverse convolutions of the left
hemisphere. The tissue destruction produced by the vascular lesion had
cut off the transverse convolutions from the internal geniculate body.

We are here, however, not considering the origin and relations of pure
word-deafness but present the case as one of =tabes dorsalis= of 20
years standing, terminated by two characteristic syphilitic
complications, first, an extensive destruction of brain tissue through
=cerebral thrombosis= and secondly, =fatal aortic aneurysm=.

=Summary=: We have here dealt briefly with a long-standing case of
NEUROSYPHILIS of the TABETIC type: A characteristic but not necessary
complication of the case is the LATE CEREBRAL VASCULAR INVOLVEMENT. The
=posterior column sclerosis= is virtually the only spinal change. Spinal
meningeal changes are absent (although it is to be assumed that chronic
inflammatory changes in the posterior roots were at one time present in
some quantity and although the spinal fluid characteristically shows
lymphocytosis in tabetic neurosyphilis).

Whether the spirochetes produce special toxic components able to cause
tabes or whether special kinds of spirochete are the tabes-making kinds
is hard to say. Special qualities of individual tissue may be involved.

The =cerebral lesions= of a =cystic= nature are of vascular origin, like
the differently localized encephalic lesions of Case 1 (Alice Morton).
Vascular syphilis is not a special property of the vessels of the
nervous system. In fact this very case died of =aortic aneurysm=.


  =PARETIC NEUROSYPHILIS (“general paresis,” “dementia paralytica,”
  “softening of the brain”). Autopsy.=


=Case 3.= James Dixon, 44, was first seen at the Danvers Hospital,
reciting verses in a dramatic and noisy way. He remained good-natured
and jolly; nor was there any change in his euphoria until he had become
physically weaker and more generally demented. In fact, Dixon appeared
to become more and more expansive as he became physically weaker. He was
in the habit of describing himself as “O. K., No. 1, Superfine.”

=Physically= the patient was gray and bald on vertex, had a dusky
complexion, was very thin (6 ft. in height, weight 155 lbs.); the mucous
membranes were pallid; the teeth rather poorly preserved; the heart was
somewhat enlarged; the pulse irregular in rhythm, of poor volume and
tension.

=Neurologically=, the patient showed a characteristic Romberg sign and
ataxia in walking a straight line. The tremulous tongue was protruded to
the left, and there was a coarse tremor of the extended fingers. The
knee-jerks were absent, and the Achilles jerks could not be obtained;
the plantar reactions were slight; the arm reflexes were present. The
pupils were stiff to light. There was a marked vocal tremor. The
sensations could not be tested on account of the patient’s mental state.

It appears that Dixon had left school at about 16, at about 22 had gone
into the provision business, and later had become a hotel clerk. He had
married at 28; there had been two miscarriages, at three months and six
weeks respectively; one child was stillborn; four children were living.

The patient was not very alcoholic. The patient’s wife thought the
symptoms had been coming on since his forty-first year when irritability
set in, but he was not discharged from work until about a year since. He
was taken back again after his wife’s pleas, and remained at work about
three months; but for ten months before admission to the hospital, Dixon
had done practically nothing, had shown a marked memory failure and
speech defect, at the same time claiming to be a person capable of doing
and accomplishing everything. He had become careless of his personal
appearance, collected a drawer-full of stumps of cigars, carried lumps
of coal in his pocket, laughed causelessly, and spat on the carpet.

We here deal with a case of unknown duration from the initial infection,
but with symptoms lasting about three years and three months. Aside from
the cause of death (empyema of left pleural cavity associated with acute
hemorrhagic splenitis, acute ileitis, and bronchial lymphnoditis), the
body showed a number of other lesions outside the nervous system. There
was the usual sclerosis of the aorta, though perhaps less marked than
usual. There was a curious acute arteritis with fusiform dilatation of
the arteria profunda femoris, with an edema of the thigh muscles and
blebs of the overlying skin. There were also multiple chronic caseating
lesions of the liver, without evidence of fibrosis. The explanation of
these liver lesions is not yet clear. There was a cloudy swelling of the
kidney.

The calvarium was dense and the dura mater thick and adherent. There was
a chronic leptomeningitis, which, however, was rather unusual in being
most marked in the posterior cisterna and along the sulci of the
cerebellar hemispheres. There was a general cerebral sclerosis, with a
question of atrophy of the superior temporal gyri (suggesting the
so-called Lissauer’s paresis). There was a marked cerebellar sclerosis
with a consequent sclerosis (grossly palpable) of the commissural fibres
of the pons. There was a generalized slight spinal sclerosis. As a fair
sample of the variety of head findings in paretic neurosyphilis, the
details of the =head examination= are presented.

[Illustration:

  A. Normal postcentral cortex. (Compare B.)
]

[Illustration:

  B. Nerve cell losses. Perivascular deposits of mononuclear cells,
    amongst which are numerous plasma cells. Note decrease in number of
    nerve cells. Note irregular disposition of nerve cells. From paretic
    neurosyphilis.
]

    Crown bald, with a slight fuzzy growth of short hairs. Scalp
    slightly adherent to calvarium; latter of usual thickness but denser
    than normal. Dura adherent to calvarium in region of vertex; dura
    not remarkable. Sinuses normal. Arachnoid villi moderately
    developed. Pia mater a trifle thickened and rather evenly throughout
    the cerebral portion. Linear sulcal markings are remarkable for
    their absence. The wall of the cerebellomedullary cisterna is thick
    and opaque. The most prominent pial thickenings are over the
    cerebellum. These are linear or may show feathery out-growths and
    are seated over the sulci, particularly in the neighborhood of the
    fissure and about the great cerebellar notch. They correspond fairly
    well with the focal variation in consistence of underlying tissues
    noted below.

    =Brain= weight, 1265 grams. Consistence somewhat increased
    throughout and somewhat evenly increased. The prefrontal region
    shows the maximal increase of consistence but the remainder of the
    frontal region and corresponding occipital region are much firmer
    than normal. The two superior temporal gyri appear to be firmer than
    adjacent gyri and are possibly slightly diminished in superficial
    diameter. The hippocampal gyri are fairly firm. The substance on
    section is a trifle more moist than normal. The gray and white
    matter cut quite evenly. Diminution in depth of gray matter, if
    existent, could not be demonstrated. The ventricles show a moderate
    sanding throughout, best marked in the fourth ventricle. The basal
    ganglia are not remarkable except for the development of numerous
    dilated perivascular spaces about the lenticulostriate vessels. The
    =pons= is atrophic, but more so on the right side. The pons, like
    the prefrontal cortex, shows on section a distinct increase of
    consistence immediately beneath the pia mater. The white bands of
    the pons on section are distinctly firmer than the intervening
    substance. The olives are of equal consistence. Weight of
    cerebellum, pons, and medulla, 155 grams. The =cerebellum= shows an
    obvious atrophic and gliotic process of a symmetrical character. The
    superior surface, including both vermis and hemispheres, shows a
    consistence above normal and general reduction of the depth measured
    from the white matter. The reduction in depth gives rise to a
    visible depression as compared with tissue posterior to the
    postclival sulci. The lobus cacuminis, though slightly raised from
    the surrounding lobes, is equally firm, if not firmer. The superior
    and inferior surfaces show practically an equal increase of
    consistence. The dentate nuclei are not especially increased in
    consistence. The flocculi are reduced in size about one-third.

    There was slight universal increase in consistence of =spinal cord=,
    best marked in lumbar region.

    =Microscopic findings= are here presented merely in sufficient
    detail to establish the diagnosis. The left superior frontal gyrus
    shows extensive and somewhat irregular cellular and fibrillar
    gliosis of the plexiform layer, together with an increase of
    thickened vessels having lymphocytes and plasma cells in their
    sheaths.

    The perivascular infiltrations are most extensive in the lower
    layers of the cortex. The lamination is in places thoroughly
    obscured, except that representatives of the layer of large external
    pyramids are almost always demonstrable.

    The layer of medium-sized pyramids has undergone more numerical loss
    of elements than have the other layers.

    Gliosis of white matter.

    Specimens from the cerebellum show a destructive process of great
    severity, but a little irregular in extent, affecting chiefly the
    Purkinje cell belt. The Purkinje cells are often absent throughout
    one side of a given lamina, and there has ensued a dense
    accumulation of neuroglia cells along a former Purkinje cell belt,
    together with a considerable gliosis of the molecular layer.
    Considerable gliosis of the white matter, both diffuse and
    perivascular in distribution.

    Perivascular plasma cell infiltrations as in cerebrum, but largely
    meningeal or in the white matter.

    Sections from the corpora striata demonstrate a mild and early
    granular ependymitis, considerable subependymal gliosis of cellular
    type, considerable perivascular gliosis in the white portions of the
    tissue, and a moderate infiltration of perivascular sheaths with
    pigmented cells, lymphocytes, and plasma cells. There is little
    evidence of alteration in the nerve cells. Some are unevenly
    pigmented.

=Summary=: We here present a case with numerous and widespread
neurosyphilitic lesions. However, the gross cerebral vascular
complications of Case 1 (Alice Morton) and of Case 2 (Francis Garfield)
are notably absent in James Dixon. Rather atypical (there seems to be
_always something atypical in cases of neurosyphilis!_) are the liver
lesions and arteritis of the leg, atypical, that is to say, for PARETIC
NEUROSYPHILIS. Highly typical of paretic neurosyphilis and almost
constant therein is the aortic sclerosis.

[Illustration:

  Apparent new formation of small blood vessel. Photographed by Dr. A.
    M. Barrett.
]

[Illustration:

  Rod cells (Stäbchenzellen) in paretic neurosyphilis. Photographed by
    Dr. A. M. Barrett.
]

[Illustration:

  Granular ependymitis—microscopic appearance of a marked example of
    “sanding” of ventricle.
]

Characteristic and constant in paretic neurosyphilis is the
=Plasmocytosis and Lymphocytosis, Perivascular= in distribution about
small cortical vessels. There is also a characteristic (though
characteristically less prominent) =Plasmocytosis and Lymphocytosis,
Meningeal= in distribution. The pleocytosis of the spinal fluid, almost
constant though variable in amount in life, is an indicator of the
meningeal picture and less directly of the parenchymatous picture.

=Granular Ependymitis= (“sanding” of ventricle floors) is characteristic
and may be regarded as part of the parenchymatous picture. This
ependymitis is an indicator how chemical changes could be readily
produced at least in the ventricular fluids, since the limiting
membranes of the nerve tissue are here subject to multiple breaks. The
“sanding” is a neuroglia reaction to these multiple small breaks
(Weigert’s explanation).

Parenchymatous losses have led to =Atrophy and Sclerosis=, of very
varying extent in different parts of the encephalon. The atrophy is
characteristic in paretic neurosyphilis, but by no means constant.
Numerous cases have come to autopsy without clearly defined gross
atrophy. Sclerosis is also characteristic and even more frequent than
atrophy, doubtless because sclerosis represents an earlier phase of a
process eventuating in gross atrophy.

A =Tabetiform Picture= characterizes the spinal cord, but in this case
the tabetic clinical picture did _not_ precede the paretic clinical
picture. We are consequently to regard the tabetic spinal process as
incidental and on all fours with the =Cerebellar and Pontine Atrophy=.


  =VASCULAR NEUROSYPHILIS (“syphilitic cerebral thrombosis”).
  Autopsy.=


=Case 4.= James Pierce was an almshouse transfer to the Danvers Hospital
in his fiftieth year. He died three years later. The accompanying brain
pictures demonstrate so extensive a lesion of the left hemisphere that
it is of great interest to determine if possible the genesis and course
of his disease. It appears that syphilis had been acquired somewhere
about the age of 38 or 40, so that the total duration of the process was
between 13 and 15 years. In Pierce’s forty-third or forty-fourth year,
he had a shock while walking in the streets of his native city,
whereupon he was subsequently transferred to the Danvers Hospital, whose
data have been summed up as follows (we are obliged to Dr. Charles T.
Ryder for these data):

    =Neurological examination:= Neuromuscular condition: Barely able to
    walk or stand without assistance; hemiplegia of right side; swings
    foot out and drags toe out and around in attempting to walk. Right
    hand held by side, flexed at right angle; fingers contracted and
    thumb thrown across palm. Can lift arm from side; practically no
    movements of forearms or fingers; atrophy of deltoid, arm, forearm,
    and hand. Muscular movements of left upper extremities fairly well
    performed; good strength.

    =Cranial nerves:= Refuses to respond to any tests to determine
    hearing or vision, but evidently hears what is said to him, and in
    his movements gives no evidence of deafness. Right corner of mouth
    droops; tongue protrudes straight.

    =Reflexes:= Pupils dilated; margins irregular; left pupil larger;
    they vary in size but it is impossible to determine whether the
    variation is due to light or accommodation reflex. Reflexes of right
    side extremely exaggerated throughout; there is little ankle clonus;
    Babinski is not obtained, patient holding his toes in flexed
    position in resisting attempts to elicit reflexes.

    =Sensations:= Reaction to pain stimuli on either side. Evidently
    some anesthesia on right side, but pressure is apparently very
    painful. There is considerable spasticity of limbs on right side on
    passive motion. Too demented to make accurate tests.

    The above examination was made on May 6, 1904. On May 20th the
    record states:

    There is almost complete sensory aphasia with word-deafness; some
    paraphasic circumlocution. Many of his words are very well
    enunciated but have no meaning. Is apparently unable to recognize
    objects or their uses.

    Brother stated that he was always supposed not to be over bright.
    Physician’s certificate states that he is epileptic, averaging two
    attacks per week. On the 15th of May he had a general convulsion;
    was unconscious for half an hour, and dull and drowsy for two hours
    afterwards. On the 19th, he had a similar attack in the afternoon,
    the convulsion lasting a minute, and he was stuporous for an hour.

    On November 8th he had a severe epileptic convulsion. His body was
    curled up to the right. The convulsive seizure lasted for two
    minutes and was followed by complete unconsciousness for an hour,
    when the patient roused and appeared as usual in a few minutes. From
    that time to December 15th he had five epileptic convulsions; he was
    much more feeble, and unable to help himself as much as formerly.

    Nov. 7, 1905: Patient has had occasional convulsions since last
    note, but none during the last three months. He is confined to bed,
    has become very much demented, and shows very marked speech defect,
    so that he is almost unintelligible. He understands only the
    simplest directions. Legs are considerably contracted and knees are
    flexed. Arm and hand on the right are paralyzed and show some
    atrophic changes; partially flexed. Left elbow jerk is very lively.
    On May 23, 1906 he was reported as having Achilles on right side
    only, and Babinski on right side. He died January 5, 1907.

    =The autopsy findings= were as follows:

    =Head:= Calvarium of moderate thickness; diploë present; dura
    slightly adherent over bregmatic region. Longitudinal sinus contains
    cruor clot. Dura is somewhat thickened and slightly more opaque than
    normal. Pacchionian granulations, small but fairly numerous. Pia
    contains throughout a considerable excess of clear serous fluid. The
    convolutions in general are of good breadth and proportion. There is
    an atrophic area roughly circular in outline and about 2 cm. in
    diameter in the posterior part of the right third frontal
    convolution corresponding to Broca’s area on the opposite
    hemisphere. The space thus formed is filled with edema held by the
    pia. On the left side is a similar subpial collection which covers
    the site of the posterior portions of all of the third frontal
    convolutions, parts of the lower end of the precentral convolution,
    and the whole of the first temporal convolution, which have
    disappeared entirely. The basal vessels show slight changes.

    Cerebellum and basal ganglia are grossly normal.

    The spinal membranes are negative. The regions of the pyramidal
    tracts in the cord are firm, project slightly from surface of
    section, and are china white.

=Summary:= Here is a picture made up almost purely of VASCULAR
NEUROSYPHILIS, with SECONDARY SPINAL (PYRAMIDAL TRACT) CHANGES.
Doubtless the genesis of this picture is allied to that of Case 1 (Alice
Morton) and to that of the terminal vascular complications in a tabetic,
Case 2 (Francis Garfield).

The absence of meningeal and parenchymatous (i.e., outside the region of
necrosis produced by the vascular disease) lesions is characteristic of
an important group of neurosyphilitic diseases. It is clear that the
case, although one of _extensive_ lesions, is _not_ one of _diffuse_
lesions in the sense of Case 1 (Alice Morton).

The spinal fluid picture in life may nevertheless show (as other cases
amply demonstrate) a certain amount of lymphocytosis and possibly
plasmocytosis, together with a variety of other changes. Treatment might
be expected to keep down these associated changes, although obviously
the effects of the necrosis are final and definite. Franz in Washington
has succeeded in “reeducating” some of these hemiplegics, employing
lower mechanisms of the nervous system.

[Illustration:

  Vascular neurosyphilis—effects of syphilitic thrombosis of Sylvian
    artery 10 years before death. (Case 4.)
]

[Illustration:

  =Case 4.= (See previous figure for brain lesion.) Three levels of the
    spinal cord showing unilateral pyramidal tract sclerosis, 10 years
    after cerebral thrombosis.
]


  =JUVENILE PARETIC NEUROSYPHILIS (“juvenile paresis”). Autopsy.=


=Case 5.= John Lawrence was an under-sized negro, who came under
hospital observation when he was 23 years of age. There was some
evidence that the patient’s father was a neurosyphilitic although
accurate data were out of the question. At all events, John had
Hutchinsonian teeth, a forward bowing of the tibiae, and
Argyll-Robertson pupils. These findings together with a history of
backwardness at school seem to stamp the diagnosis. It seems that there
had been a change for the worse from the age of 18, though the boy had
been able to sell newspapers and black shoes up to within a year of his
arrival at the hospital. During the last months of his life, he showed a
general incoördination, with false movements suggesting those of a
drunken person. There were numerous tremors, the glance was shifting,
and there was a tendency to nystagmus. Some of these phenomena (taking
into account that the Hutchinsonian teeth were not entirely typical and
there was even at times some doubt as to whether the pupils were
actually stiff) led to a question of the diagnosis multiple sclerosis.

There was, however, little doubt that the case was one of juvenile
paresis. Among the symptoms found at various times in this case are the
following: disorientation for time, place and persons, confusion, with
coarsely irrelevant replies to questions, ill-defined and transitory
delusions of persecution, auditory, tactile, and visual hallucinations,
and defective memory.

Early in life, the patient had had a habit of falling asleep in school
hours, and had experienced a number of falls at various times. During an
attack of measles he had had a number of spasms, each of which lasted
ten minutes or more.

The =autopsy= showed death to be due to an early bronchial pneumonia.
The thymus was persistent, measuring 3 × 2 × .5 cm. The marrow of the
femur was red.

There was a moderate degree of =sclerosis of the aorta= confined to a
few plaques in the arch (not a characteristic syphilitic scarring of the
aorta). The spleen was small and had a thickened capsule.

The majority of the lesions, however, were in the =nervous system=, and
the following description is taken from the routine hospital records to
exemplify the findings in a fairly characteristic case of JUVENILE
PARESIS.

    =Head:= Scalp closely adherent to =calvarium=. Calvarium heavy
    without diploë. =Dura= adherent to calvarium in bregmatic region.
    Sinuses contain liquid blood. Arachnoidal villi in considerable
    quantity. =Pia mater= contains considerable clear fluid and shows
    diffuse haziness and focal thickenings. The diffuse haziness is
    almost universal and is best marked over the superior surface of the
    cerebellum. The focal thickenings are of general distribution over
    the veins of the sulci on the superior surface of the brim and are
    heaped up to form considerable linear mounds near the region of the
    arachnoidal villi. The superior surface of the cerebellum is
    traversed by similar linear mounds of fibrous tissue running at an
    angle to the laminæ. There is no notable increase of fibrous tissue
    at the base.

    =Brain:= Weight 965 grams. The sulcation is roughly symmetrical
    except in the occipital poles where there is unusually rich and
    complex but shallow sulcation. The cortical substance is everywhere
    firmer than normal, but the sulci fail to flare notably. In a few
    places there is a focal increase of consistence of still greater
    degree with apparent local hypertrophy (or gliosis with increase of
    substance). These foci are in the right second temporal gyrus (3 cm.
    in diameter) and in the left first temporal gyrus (of same size but
    somewhat less firm) and are of a whitish, waxen appearance, being
    visible several feet away by reason of their color and apparent
    encroachment upon the adjacent sulci. The foci are sharply limited
    by the sulci laterally, but pale out gradually before and behind.

    The convolutions of the vertex show another type of lesion. The
    tissue of the greater part of the vertex resembles that of the
    flanks and base in being firmer than normal and of a grayish pink
    color. Behind the fissure of Rolando on the right side and behind
    the anterior limits of the ascending frontal region on the left side
    the brain tissue of the vertex becomes suddenly still firmer and of
    a yellowish gray color. This lesion disappears gradually into the
    occipital microgyria behind and the gyri gradually lose their
    yellowish tint. The lesion fades away gradually so that it fails to
    involve the temporal convolutions.

    The cerebral tissue cuts firmly and smoothly. The tissue of the
    frontal region is a little edematous. The white matter is of a
    normal appearance. The ependyma of all the ventricles is somewhat
    sanded. The fourth ventricle is most affected.

    The =cerebellum= is not edematous and is as firm as the normal
    olivary bodies. The cerebellar hemispheres are symmetrical and of a
    normal appearance, save that the laminæ are slightly narrower than
    usual and very compactly set. The color, where not obscured by the
    haziness of the pia mater, is of a grayish pink somewhat suggestive
    of freshly tanned shoe leather. The substance cuts smoothly and
    firmly. The dentate nuclei are unusually firm. The =pons= is small,
    but of the usual color. Lower structures normal except the =cord=
    which is small and shows curious deviations from the normal
    markings. The posterior horns and gray commissure are at many levels
    the only structures to preserve the normal gray appearance, so that
    the H or butterfly appearance is replaced by a crescent. At these
    levels, traces of gray matter often stand out in the loci of the
    anterior horns.

The important =anatomical diagnoses= in the nervous system are as
follows:

Atrophy of cerebrum, 965 grams (there is of course a question whether we
are not dealing with a degree of cerebral hypoplasia).

Focal scleroses of cerebrum, suggesting the tuberous scleroses of
Bourneville.

Occipital microgyria.

Cerebral and cerebellar gliosis.

Chronic ependymitis.

Gliosis of the gray matter of the spinal cord.

Chronic diffuse and focal leptomeningitis.

The =microscopic examination= confirmed the diagnosis of paresis. The
hypertrophic nodules were of special interest. They were found to be
overlain by a characteristic though thin exudate of lymphocytes and
plasma cells, together with pigmented cells. The nodules appeared to be
supplied with an unusual number of vessels of small calibre, about which
were a few lymphocytes. The large vessels and those with well developed
adventitiæ were surrounded by more numerous lymphocytes and by more
focal accumulations of pigmented cells. The cortex in the middle of a
nodule had almost lost its characteristic cortical layering. The cortex
was here reduced (specimen from temporal lobe) to about one-quarter of
its normal thickness, and was found to be composed largely of expanded
neuroglia cells and vascular tissue, with a few nerve elements, small,
shrunken, and dark-staining. The destructive process appeared to have
borne hardest on the layer of internal large pyramids and the fusiform
layer. There was, however, nowhere any evidence of focal necrosis such
as ought to characterize a true gumma. The sections stained by the
Marchi method failed to show evidence of fatty degeneration within the
focus, although there was a marked diffuse accumulation of fatty
granulations along the nerve fibres in the underlying white matter. A
special study of the cerebellar material was made by one of the
authors.[4] Occasional Purkinje cells showed the characteristic
binucleate condition, which has frequently been noted in recent
literature.

The cerebellum of this case was perhaps the most markedly diseased of
all portions of the nervous system. As noted, the cerebellar tissue was
exceedingly firm. How far the notable incoördination of the case (he was
observed on staff rounds characteristically curled up in a heap, showing
quite an unusual degree of general incoördination) was due to the
cerebellar lesions, it is perhaps not possible to say.

=Summary=: John Lawrence, JUVENILE PARETIC NEUROSYPHILIS, is a foil to
Case 3 (James Dixon), paretic neurosyphilis due to acquired syphilis.

Both showed =Cerebral Atrophy=, but Lawrence the more markedly because
of hypoplasia incidental to the congenital origin of his condition.

Whereas Dixon gave little or no sign of =stigmata=, Lawrence (besides
being under-sized, having suspicious teeth, and showing at autopsy a
persistent thymus) showed a =Hydromyelia= and curious trefoil shape to
the spinal cord. Dixon on the other hand had liver lesions and arterial
lesions of the leg.

The suggestion of =Tuberous Sclerosis= in Lawrence is not found in
Dixon; but we have not found it elsewhere. Bourneville did not describe
tuberous sclerosis as syphilitic.

Binucleate Purkinje cells emphasize the congenital source of the lesions
in Lawrence.

=Plasmocytosis and Lymphocytosis=, =Perivascular=, and (less marked)
=Meningeal=, are found in both the congenital and the acquired cases, as
also parenchymatous changes, both =nerve cell losses= and =gliosis=.
Both also show granular =ependymitis=.

It is clear that, over and above the factors of destruction evident in
both Lawrence and Dixon, the congenital case, Lawrence exhibits also the
effects of arrest (in brief not merely atrophy but also hypoplasia).
Early treatment is, therefore, theoretically indicated in the juvenile
group, which means early diagnosis. Early diagnosis and treatment are
still more to be recommended because these juvenile cases progress often
very slowly at first.


  =FOCAL BASILAR MENINGEAL NEUROSYPHILIS (“syphilitic extraocular
  palsy,” plus other symptoms). Autopsy.=


=Case 6.= Flora Black, a housewife of 43 years, had been tired out for a
year but had been apparently in fair health. She awoke one day with
double vision due to a left internal strabismus. The visual difficulty
gradually passed away so that five months after the sudden seizure she
was apparently quite well again. There was one exception: about three or
four months after the attack of diplopia, Mrs. Black had begun to feel a
kind of weakness in various parts of the face and there were also fairly
definite paresthesiæ. In the sixth month after the initial attack, the
patient began to be unable to chew and was fain to support the lower jaw
with a bandage to aid in mastication. Deglutition was, however, quite
unaffected and there was never any regurgitation of food. There were
pains in the face, the forehead and the back of the neck.

Upon =physical examination= at entrance to a general hospital, no
changes in the body at large were discoverable. There was a slight edema
of the ankles, otherwise no sign of bodily disease.

Conditions in the =head= were as follows: The facial lines were (notes
by courtesy of Dr. E. W. Taylor) smoothed out; both upper and lower
eyelids and the corners of the mouth drooped slightly and more markedly
on the left side. There was slight photophobia and considerable
lachrymation. The patient was unable to pucker forehead, nose or mouth.
The unsupported lower jaw fell and the patient was unable to open the
mouth widely. The movements of the tongue were normally performed.
Speech was mumbling. Sensations of touch, heat and cold were preserved
all over the face except that the left cheek below the level of the
mouth yielded a less accurate registration of tactile sensations. A hot
test tube did not feel as hot in the lower left cheek as elsewhere.
Quinine and sugar could not be tasted over the left half of the tongue
in front. Smell and hearing were also diminished on the left side. It
appeared that there was a complete paralysis of the 5th and 7th nerves
and a partial paralysis of the 8th, 11th and 12th, as well as a defect
in smell.

The patient died suddenly, three weeks after admission, running a slight
temperature during her stay. The autopsy showed (rather surprisingly) a
double ovarian carcinoma with metastases into the retroperitoneal
glands. Both kidneys were found to be riddled with nodules of carcinoma.
The pelvic veins were thrombosed and there was a complete occlusion of
the pulmonary artery. There was a riding embolus in the foramen ovale
and there was coronary embolism.

The striking nature of these complications and the interest of the case
neurologically would warrant its publication in complete detail. We here
present the case with utmost brevity as an example of a SYPHILITIC
CRANIAL NEURITIS by extension from the meninges.

The =brain= was in general without change but there was a considerable
exudate over the entire =pontine region= which had involved several
cranial nerves. The 5th nerves, especially the left, showed gross
effects of the inflammatory lesion. There seems to be little or no doubt
that this neuritis was of syphilitic origin despite the complication of
the case with carcinoma of the ovary and despite the fact that the case
was observed and came to autopsy before the modern methods of systematic
diagnosis could be applied. It is the best case available to us for the
demonstration of a focal cranial nerve lesion of the type characteristic
of neurosyphilis. We may well suppose that similar conditions would have
been found at various stages in the development of Case 1 (Alice
Morton). The pontine region of Case 1 was entirely free from lymphocytic
exudate at the time of the autopsy. Possibly the clearing up of the
pontine pia mater in Case 1 was a therapeutic effect of the thorough
treatment therein used. Whether a case like Mrs. Black’s could be cured
(aside from the ovarian carcinoma and its complications) by the
institution of vigorous systematic treatment is a matter of doubt.
Still, in a general way, these cases of focal syphilitic neuritis are
among the most favorable cases for treatment.

=Summary=: We present the case of Flora Black to emphasize how slight in
extent and theoretically curable neurosyphilis may be. We fear that Case
1 (Alice Morton) may present too unrelieved and pessimistic a picture.
The extensive vascular lesions and complications of Alice Morton, of
Case 2 (Francis Garfield), of Case 4 (James Pierce) arrest attention by
the incurability of their residual effects (if we omit modern attempts
at reeducation of lower arcs). On the other hand the unrelenting
progress to destruction of important parenchymatous structures, as shown
in the paretic James Dixon (Case 3) and his juvenile replica John
Lawrence (Case 5), as well as in Alice Morton (Case 1) and the tabetic
Francis Garfield (Case 2), lead to a certain justifiable pessimism. For
it is only the meningeal and fine vascular infiltrations of these cases
that we can theoretically hope to combat, probably by destroying the
spirochetes in these meningeal and perivascular loci. We seem
theoretically less able to stop the progress of the often highly
systemic and symmetrical, parenchymatous lesions of the tabetic and
paretic group.

The condition in Flora Black is clearly much more hopeful, both being
more focal and being almost purely meningeal and therefore accessible to
therapy.

The two cases which conclude our general survey of neurosyphilis are
also focal cases, one of gumma (Lecompte) and one of focal dural lesion
(Wyman).

[Illustration:

  1. Pons, normal except for focal infiltration of left fifth nerve.
]

[Illustration:

  2. Higher power view of infiltrated left fifth nerve.
]

[Illustration:

  3. Detail of infiltrated left fifth nerve, showing: 1, diffuse
    infiltration with mononuclear cells; 2, perivascular infiltration;
    3, strands of relatively unaffected nerve fibers.
]

Microscopic appearances in Case 6. Extraocular palsy (focal meningeal
syphilis, especially of left fifth nerve). Illustrates exquisite
focality of the syphilitic process sometimes found, as well as its
unilaterality (giving rise to asymmetrical symptoms and signs). Process
in itself probably curable.


  =GUMMATOUS NEUROSYPHILIS (“gumma of brain”). Autopsy.=


=Case 7.= Mrs. Lecompte was a woman of middle age, who, according to the
history given by her son, had been entirely well until her final
illness, which began approximately two years before admission to Danvers
Hospital. The beginning of her trouble seemed to be chiefly headaches,
which would last continuously for several days, or more than a week at a
time. These headaches lasted throughout the course of the disease. In
the morning, on arising, she would feel very dizzy, but this would pass
away during the day. She had had a number of spells of unconsciousness,
lasting about fifteen minutes. In these attacks she would breathe
heavily, there was frothing at the mouth, twitching of the hands, and
the eyes would roll about. Her memory failed gradually, her disposition
changed and she became very irritable. Vomiting occurred almost every
day, and at times was of a projectile character. She became
hallucinated; the hallucinations were chiefly of a visual nature.

About four months before admission to the hospital, after one of her
seizures, the entire right side was found to be completely paralyzed,
and she complained that it was numb. At this time, she had difficulty
with her speech. In a few days, however, she was able to talk correctly
again, and in a week she was back at work, although the right side was
weak and awkward. She continued to grow worse, and then began to have
spells lasting several days, so that it became necessary to have her
placed in a hospital.

On admission to the hospital, aside from obesity, the general viscera
showed no points of special interest, and there was no evidence of any
new growth outside of the nervous system. She was unsteady on her feet,
standing with them wide apart. The gait was quite ataxic; the whole
right side was weaker than the left and used more awkwardly. There was a
paralysis of the right side of the face; the right angle of the mouth
drooped; the right eyelid could not be closed but remained continuously
open; nor could the right side of the forehead be wrinkled. Vision and
hearing were not affected. She miscalled tastes and smells; whether this
was due to aphasic difficulties or to cranial nerve involvement could
not be divined. There seemed to be some difficulty in deglutition. The
knee-jerks were markedly exaggerated; slight clonus was obtained but was
not always present. Both pupils reacted well to light and distance and
consensually. Sensation could not be readily tested. There was marked
ataxia, especially with the eyes closed. The speech was thick and
mumbling. The patient was unable to write or copy. Mentally the patient
was quite dull; at times, stuporous; when aroused, was found to be
entirely disoriented. Memory almost entirely absent. In general she
showed herself to be very much confused.

She remained practically in this condition, even gaining in weight, for
the following two years, when suddenly one morning, she had an epileptic
seizure, vomited, coughed a great deal, with bleeding from the mouth and
ears, and died in a few hours.

The symptoms in this case pointed to brain tumor. The only inconsistent
thing was the long-continued life,—four years,—after the symptoms were
observed. As she lived before the W. R. and spinal fluid tests were
known, no light was gained in these ways. The post mortem examination
showed the patient had a GUMMA OF THE BRAIN.

The =summary of the anatomical diagnoses= at autopsy was:

Decubitus.

Lymphadenitis of the mesenteric nodes.

Chronic fibrous peritonitis.

Chronic fibrous myocarditis.

Pulmonary hypostasis.

Thrombosis of vein in right adrenal, with hemorrhage.

Syphilitic leptomeningitis.

Gumma of left hemisphere.

Focal softenings in the pons.

The =anatomical description of the head= (Dr. A. M. Barrett) is as
follows:

    The sutures in the =calvarium= are well outlined; diploë large in
    amount. The =dura= is diffusely but lightly adherent to the
    calvarium; it is very dense, especially over the left hemisphere.
    The meningeal arteries are thickened but not atheromatous. The
    sinuses contain a small amount of fluid blood and post mortem clot.
    The inner surface shows nothing abnormal. There is a great
    flattening of the convolutions of the left hemisphere, which is not
    the case on the right side. Over the convexity, the =pia= is thin
    and not abnormal except for some slight adhesions between the
    frontal lobes and the two lips of the Sylvian fissures. The pia at
    the base over the cisterna, pons, and medulla is thick, cloudy, and
    of a grayish gelatinous appearance. It is so thick that it is easily
    removable in a large piece.

    The surface of the left hemisphere is dry, and the whole brain is
    flabby and bulges as if from internal pressure. A section through
    the hemispheres at the region of the optic chiasm shows a hard, firm
    area in the left hemisphere deep down in the white substance. It is
    about 2½ cm. in diameter, with a wavy border. The central part is of
    a silver-gray gelatinous-like appearance, with red spots and whitish
    streaks radiating from the centre. In the pons on the right side, in
    a plane passing through the posterior corpora quadrigemina, are two
    pinhead size softenings among the pyramidal fibres. The ependyma of
    the fourth ventricle is granular.

    =Microscopic examination of the tumor=: The area evidently contains
    several central necrotic foci surrounded by zones of infiltration
    and proliferation, with bordering areas of nervous tissue showing
    secondary reactions. The necrotic area stains poorly. From the edge
    there are projections of reddish homogeneous bands, some intermixed
    with well-differentiated fibrillæ, probably glia fibrils. The
    bordering zone is densely infiltrated with lymphoid, plasma, and a
    few epithelial cells. The nerve tissue outside of this zone is
    spongy and infiltrated with lymphoid and plasma cells. There are a
    few scattered, shrunken nerve cells. In this zone and in the zone of
    infiltration near the necrotic area, there are scattered cells
    resembling giant cells. There are many obliterated vessels in the
    area, and other vessels show many infiltrating lymphoid and plasma
    cells in the walls. The examination of the specimen stained by the
    methods for bacilli of tuberculosis are negative. The growth is a
    classical gumma.


  =GUMMATOUS NEUROSYPHILIS (gumma of spinal meninges, “meningitis
  hypertrophica cervicalis of Charcot?”). Autopsy.=


=Case 8.= John Wyman was first seen in his thirty-sixth year by Dr.
James J. Putnam. He denied syphilitic infection and stated that the
first symptoms had come four months before. He had begun to notice a
numbness of the fingers, at first of the right hand and shortly
thereafter of the left hand. After a few weeks there had been difficulty
in walking, and a few weeks later headaches, especially on the right
side, developed. Two weeks before he was first seen medically, he had
begun to have a feeling of tightness or constriction in his arms.

It appears that micturition had been impaired early, that is to say, a
few weeks after the initial sensory disorder had begun. A catheter was
used for a time and improvement followed. Shortly before consultation
retention of urine developed again, this time associated with rectal
incontinence. The feet began to feel heavy and dead. Then the legs began
to be increasingly weak so that the patient was almost bedridden. Vision
appeared to be normal except that reading was followed by fatigue. The
speech was also slow but the slowness could be attributed to fatigue.

Notes of Dr. Putnam’s =physical examination= are as follows: The patient
lay in bed on the left side, without motion, and almost incapable of
motion. The tongue was protruded, and there was no paralysis of facial
muscles, or of the eye muscles (the right pupil had been reported to be
slightly larger than the left). There seemed to be a disinclination to
move the head, but with some effort it could be moved, and without pain.
The arms and hands were held rigidly in median positions; many movements
were possible, but all were imperfect and of slight amplitude. The
fingers were flexed to a moderate degree, and could not easily be
straightened, and there was, in fact, a general rigidity of most of the
muscles of the body below the neck, and even, in some degree, of the
neck. The immobility was so great that the general impression made was
almost that of a patient with fracture of the spine in the cervical
region. Even the breath, and especially the inspiration, was imperfect.
The legs were more freely movable than the arms, but still the motions
were very stiff and awkward, and of slight amplitude; with effort the
whole leg could be lifted from the bed, and flexed or extended with
moderate force. The right leg was rather stronger than the left, but the
left hand and arm were stronger than the right. The sensibility was
almost absent over the hands and lower part of the arms, and was
impaired over the entire head and neck, except the forehead, the middle
part of the face, and the nose. It is interesting to compare the
conditions of the sensibility here present with those seen in cervical
syringomyelia. The sensibility of the upper part of the forehead was
less good than of the lower part, and there was slight impairment even
over portions of the lower jaw. The sensibility of the left (stronger)
arm was rather more impaired than that of the right arm, while on the
contrary the sensibility of the left leg was better than that of the
right leg, though the difference between them was not great. These
statements apply to sensory tests by contact, heat, cold, and pricking.
Knee-jerks were highly exaggerated, and likewise the wrist-jerks. All
forced attempts at movements were attended by a high degree of muscular
tremor, especially when the patient was fatigued or under emotional
strain. The fingers especially were the seat of coarse tremor.

The remainder of this clinical description (courteously supplied us by
Dr. Putnam) may be quoted. A second examination which included also a
few facts not given in the first examination was made on the following
March 28, 1905. This report says “the ends of the fingers became numb
about June 1, 1904. Work was given up on July 3, and at that time the
patient was walking very badly. No treatment was used and no
satisfactory diagnosis made. In the course of July he improved somewhat,
and during August he was able to ride out a little (these spontaneous
improvements are of interest for the diagnosis). He went away from home
for a short time, but from the time of his return, about the last of
September, he grew worse rapidly, and fell into the condition above
described, in which he was wholly unable to help himself, even to
turning in bed. At times he had a great deal of pain in the neck and
forehead. Antisyphilitic treatment was recommended, and for a time
potassium iodid and other iodid preparations were given, but at first in
relatively small doses (grs. 75 daily). Under this treatment the
excretion of urine rose to four quarts daily as a maximum though
sometimes the quantity was not so great.”

Under this treatment the patient began soon to improve, and continued
doing somewhat better till about five months later. He became able to
walk downstairs and out of doors, and regained considerable use of his
hands. The quantity of urine passed became greatly increased by the use
of the iodid.

About the middle of March he became worse again. A careful examination
of the sensibility showed that in general the condition was much the
same as that previously reported. The iodid treatment, with perhaps some
mercurial, was resumed; the potassium iodid was given in doses which
were increased up to 850 grains daily, although this maximum dose was
taken only for about one week. This large quantity gradually impaired
the sense of taste for the time being, and blurred his vision, but
otherwise did him no harm. Under this he improved, so that he became
able to run more or less, and went about freely, and attended to his
business, though still retaining some stiffness in his movements.

This improvement continued until about two years later, when he again
had a relapse, and was seen medically once more. His condition at this
time was still a pretty good one, but the movements were stiff and
awkward. The bin-iodid of mercury was advised, which was taken in doses
of 9⁄25 grain daily. It will be remembered that this was long before the
days of salvarsan treatment.

This was toward the end of June, 1907. Contrary to expectation, there
was no material gain from this treatment, and the patient died early in
October, without being seen again.

The =autopsy= was limited to the =nervous system= and the findings were
as follows (Dr. A. R. Robertson):

    =Head=: Hair abundant, fair, of fine texture and rather curly. Scalp
    of medium thickness and strips readily from calvarium. The latter
    appears normal and upon removal is of about the normal thickness. It
    lifts readily from the dura mater, except for the numerous
    attachments of Pacchionian granulations.

    =Meninges=: The dura is smooth, moderately injected and shows no
    areas of thickening; it lifts readily from the pia-arachnoid. The
    pia-arachnoid shows discrete and in many places diffuse areas of
    opacity. There is a moderate amount of subpial clear fluid and the
    vessels are moderately injected. Over the anterior surface of the
    medulla and lower portion of the pons and largely confined to the
    right side there is a very marked thickening of the pia-arachnoid to
    which the dura is densely adherent. This thickening extends down
    anteriorly and laterally on the right side over the upper part of
    the cervical cord. The thickened meninges over the upper part of the
    medulla completely surround the right vertebral artery, shortly
    before it joins its fellow of the opposite side to form the basilar.
    Dissection of the arteries shows them to be patent and thin walled.
    Over the =cerebrum= and cerebellum the pia-arachnoid strips readily
    leaving a smooth surface. Section of the cerebral cortex, basal
    nuclei, pons and cerebellum show no gross lesions. The ventricles
    are moderately distended with fluid. The ependyma contains numerous
    small cysts. Section of the =pons= shows no lesions of the nervous
    tissue, but very marked thickening of the surrounding meninges as
    noted above.

    =Cord=: Throughout the cervical and dorsal region the dura is quite
    tensely distended with an abundance of clear, light, straw-colored
    fluid. Upon snipping the dura this fluid escapes with a small spurt,
    as if under considerable pressure. The cord within, for the most
    part, lies free, but over the upper three or four centimeters of the
    cervical portion it is densely adherent to the dura anteriorly and
    laterally on the right side. Cross sections were made through the
    upper three or four centimeters of the cord, and over this area the
    cord is constricted by very marked thickening of all the meninges.
    The meninges here average from one to three millimeters in
    thickness. On the right side and somewhat anteriorly opposite the
    junction of the atlas and axis there is a single nodular, firm mass
    which on section shows a yellowish, firm center surrounded by very
    dense, pearl-gray tissue. The demarcation between the homogeneous
    yellowish centre and its surrounding gray tissue is very sharp. This
    nodule measures about 0.75 to 1 cm. in diameter. The adjacent cord
    is deeply indented by it. Below this nodule there is a translucent,
    grayish appearance of both posterior sensory columns which extends
    downwards and diminishes in intensity until it finally disappears in
    the upper dorsal region. This same appearance is well marked on the
    right outer margin of the upper cervical cord corresponding to the
    crossed pyramidal tract, and extends downwards diminishing in
    intensity until it disappears about the mid-dorsal region. The left
    pyramidal tract appears to be similarly but very slightly involved;
    section of the lower dorsal cord entirely negative.
    =Microscopically=, characteristic GUMMA.

It is a question whether this case is one of the group described in 1871
by Charcot under the name of _pachymeningitis cervicalis hypertrophica_.
Charcot did not regard his new disease as syphilitic, and it is very
probable that syphilis is not responsible for all cases. Charcot,
however, noted that his new disease was not incurable: he noted that the
resulting paraplegia, although it might be very marked and accompanied
by flexion of the leg on the thigh and although the paraplegia might
have lasted a very long time, might end in recovery. Charcot thought
that surgical intervention was necessary. He described three periods in
the disease, the first or neuralgic (pseudo-neuralgic) was characterized
by sharp pains in the neck and by the sensation of constriction in the
upper part of the thorax. The second phase of the disease was, according
to Charcot, the paralytic phase, in which a cervical paraplegia
accompanied by muscular atrophy developed. Sometimes cases were found to
remain in this paralytic phase and even to end spontaneously in cure. If
the muscular atrophy was degenerative, then the atrophy was never
replaced; but, according to Charcot, some cases of atrophy were simple
and accordingly curable. If, however, the spinal cord itself became
involved in the meningeal inflammation, then phenomena of transverse
myelitis set in with a spastic paraplegia and involvement of the bladder
and rectum. Muscular atrophy never developed in the legs, at least in
typical cases.

Among the causes of this condition the following have been mentioned:
cold, overexertion, alcoholism, tuberculosis and syphilis. Syphilis
undoubtedly plays the major part. Even before the days of the W. R.,
observers, among whom may be mentioned Dejerine-Tinel and Pförringer,
discovered syphilis in nearly all sufferers from _pachymeningitis
cervicalis hypertrophica_.

It should be differentiated from caries of the spine and cord and
meningeal tumors. The spinal fluid examination makes this somewhat easy.

Antisyphilitic remedies are indicated, and should be tried even when the
etiology is obscure, if only as a therapeutic test.


    But what have been thy answers? What but dark,
    Ambiguous, and with double sense deluding,
    Which they who asked have seldom understood,
    And, not well understood, as well not known?

                            Paradise Regained, Book I, lines 434–437




    II. THE SYSTEMATIC DIAGNOSIS OF THE MAIN FORMS OF NEUROSYPHILIS


  =PARETIC NEUROSYPHILIS (“general paresis”) sometimes persistently
  receives the diagnosis NEURASTHENIA simply through omission to apply
  approved diagnostic methods.=


=Case 9.= Greeley Harrison, a man of 46, certainly looked like a
neurasthenic. He wanted aid for nervous indigestion of years’ standing,
headache, insomnia, nervousness, failing memory, and deafness. He
volunteered, in fact, that he had neurasthenia, and that he had been
treated for this by hypophosphites.

During the practically negative =physical examination=, Harrison
complained of headache and throbbing in the head, and during examination
of the abdomen felt much nauseated and proceeded to vomit rather
persistently. There were hemorrhoids.

=Neurological examination= showed that the left pupil was smaller than
the right, was irregular, failed to react consensually, and reacted very
slowly to direct light. For the rest, however, the neurological
examination was negative. On account of the nausea and vomiting, special
examination of the gastric contents was made, but nothing abnormal was
found.

=Mentally=, it was rather striking that the patient’s memory was quite
inaccurate both for remote and for recent events. His school knowledge
was very meagre. As for delusions, the only approximation thereto was
the patient’s continually dwelling upon his bodily symptoms.
Emotionally, he varied between depression and a sanguine attitude.

Although there was no symptom directly suggesting syphilis in the
Harrison case, the slightly abnormal pupillary reactions and the amnesia
warranted the suspicion of syphilis. The blood and spinal fluid both
proved positive to the W. R.; the gold sol reaction was of the “paretic”
type; there were 18 cells per cmm.; there was considerable globulin, and
an excess of albumin. On the whole, therefore, we felt entitled to make
the diagnosis GENERAL PARESIS. Why should not a careful observer have
considered syphilis seriously? Yet in our experience such cases are
frequently diagnosticated neurasthenia, thus entailing dangerous delay
in treatment (in this case, five years’ delay).

Going over the history of the case with still greater detail, we learned
that for a number of years past, there had been symptoms of a
neurological nature. For instance, five years before, at the age of 41,
the patient had been apparently overcome when working near a stove, and
went upstairs talking incoherently, but recovered shortly. Thereafter,
such spells occurred almost every month; later, more frequently; still
later, the attacks were associated with unconsciousness and amnesia.
Occasionally preceding the attack there would be twitching of the mouth,
jerking of the arms, and incoherent talk. Throughout these last five
years, in point of fact, the patient had been unable to do regular work,
had been given to much complaining, and had been far less efficient than
formerly. In short, it would seem that, with the improved technique now
in the possession of medical science for the diagnosis of general
paresis, cases like that of Harrison will be diagnosticated earlier and
earlier.

    1. How typical is the insidious onset of symptoms in the case of
      Harrison? The onset of symptoms in neurosyphilis is ordinarily
      considered to be sudden, and this statement is generally true
      despite the fact that after the diagnosis is established a number
      of mild prodromal symptoms can be remembered by the relatives.
      However, some cases, of which Harrison is an example, have an
      exceedingly insidious onset without sudden access of striking
      symptoms. Joffroy and Mignot remark that with the improvement of
      clinical methods, the course of paretic neurosyphilis must now be
      stated to take some six or seven years for completion. In point of
      fact, there were early episodic symptoms (seizures almost monthly)
      which should not have escaped medical attention. They did escape
      medical attention, however, and Harrison was wont to say “Why
      wasn’t I told that my disease was syphilis five years ago?”

    2. Is there such a disease as syphilitic neurasthenia? According to
      Kraepelin, syphilitic neurasthenia has been described as occurring
      shortly after infection and in the first stages of syphilis. There
      are milder and severer forms; the milder forms show discomfort,
      difficulty in thinking, irritability, insomnia, cephalic pressure,
      indefinite variable, uncomfortable sensations, and pains. The
      severer cases acquire anxiety, more pronounced emotional disorder,
      dizziness, disorder of consciousness, difficulty in finding the
      right word, transient palsies, pronounced sensory disorders,
      nausea, and increase of temperature. Kraepelin is in doubt whether
      there is any definite clinical picture of this sort, and whether
      there is any causal relation between the syphilitic infection and
      such symptoms as those described. If the effect of knowledge
      concerning infection is a merely psychic effect, then it is
      improper to term the neurasthenia in question a syphilitic
      neurasthenia. For the relation of hysteria to the acquisition of
      syphilis, see below the case of Alice Caperson (46). In point of
      fact, modern work has shown even in the primary and secondary
      stages of general syphilis more or less pronounced neurosyphilitic
      phenomena in the shape of the so-called meningitic irritation of
      French authors. (Besides the case of Caperson (46), see the case
      of Fitzgerald and the discussions under these cases.)

    3. What is the relation of the early symptoms of this case to the
      so-called preparesis of Dana? The case might well have been an
      example of Dana’s preparesis. For a discussion of this, see Case
      of William Twist (13).

    4. What is the classical differential diagnosis between paretic
      neurosyphilis and neurasthenia? The testing of the blood by the W.
      R. is unconditionally necessary. If the W. R. is negative, the
      diagnosis of paretic neurosyphilis is extremely improbable. (It
      must be borne in mind that a number of cases of paretic
      neurosyphilis have been shown to have a negative W. R. in the
      serum, and receive a proper diagnosis only after spinal fluid
      examination.) Next to the serum W. R. stand the pupillary and
      aphasic symptoms. In the presence of Argyll-Robertson pupil or
      even a slight speech defect, the diagnosis of neurasthenia must
      certainly be made with caution if at all. Kraepelin remarks: The
      sudden occurrence of neurasthenic disorders in a male of middle
      age without any evident cause therefor is always suspicious. Yet
      it must be emphasized that a complaint of occasional dizziness,
      slight speech defect, tremor of tongue, and a moderate increase of
      tendon reflexes do not possess any marked diagnostic significance.
      Clear insight and understanding of the nature of the disease
      phenomena, a persistent search for recovery, reasonableness in
      conversation, progressive improvement under appropriate treatment,
      speak for neurasthenia.

      Joffroy and Mignot differentiate what they call preparetic
      neurasthenia from other neurasthenic states, not only on the basis
      of its etiology but on the basis of its symptoms. They also call
      attention to the fact that neurasthenia, being a pure neurosis,
      develops either on a manifestly hereditary basis or upon some
      physical injury, weakening disease, or moral shock. The pure
      neurotic suffers a great deal more than the patient who is
      destined to become a victim of paresis. The character change in
      neurasthenia does not amount to that entire transformation of
      personality (even to the performance of criminal acts) that we
      find in paretic neurosyphilis; at the most, the neurasthenic shows
      minor emotional disturbances and a certain pathological egoism.
      The psychotherapeutic test also rather readily dissipates many of
      the neurotic, hypochondriacal fears and feelings. Although both
      pure neurasthenia and the paretic pseudoneurasthenia are
      characterized by sexual weakness, the sexual anæsthesia of the
      preparetic is practically always preceded by a stage of sexual
      over-excitement. These finer clinical indications, however, fade
      into insignificance beside the data that can and should be
      obtained from laboratory tests.

    5. How exceptional is such a case as that of Harrison? We have in
      our experience seen many patients with a similar course and
      configuration of symptoms, although the majority of these cases in
      a community advanced enough to provide easy access to a Wassermann
      laboratory are now diagnosticated far earlier than was the case of
      Harrison.

    6. What attitude shall we take toward so-called syphilophobia? It
      seems to us that resort to a serum W. R. is indicated, both from
      the standpoint of the community and still more importantly from
      the standpoint of the patient. We are even inclined to suggest for
      a case of persistent syphilophobia, when the serum W. R. has
      proved negative, a lumbar puncture. Syphilophobia must be
      considered, not as a syphilitic psychosis, but as a phobia to be
      classified among the psychoneuroses. It becomes a difficult
      question to decide at times whether a patient who has had
      syphilis, has had a considerable course of treatment and shows the
      symptoms of a syphilophobiac should be further treated for
      syphilis or merely for his phobia. We have seen recently such a
      patient who gave a certain history of syphilis and who was greatly
      disturbed lest he should be developing paresis. This fear bothered
      him greatly. Examination showed irregular pupils, but no other
      signs of syphilis. The W. R. in blood and spinal fluid was
      negative as were the other spinal fluid tests. It was considered
      wise to treat him only for his phobia and under this treatment he
      was given some relief.


  =PARETIC NEUROSYPHILIS (“general paresis”) may look precisely like
  MANIC-DEPRESSIVE PSYCHOSIS.=


=Case 10.= The mental picture in Lyman Agnew, an architect, 58 years of
age, was wholly characteristic of manic-depressive psychosis. In the
first place, there had been (at 55) a previous attack of depression,
lasting a few months, from which Agnew had completely recovered. He had
remained entirely well up to four months before consultation.
(Manic-depressive psychosis is, at least in a majority of cases,
hereditary. There had been mental disorder in one maternal cousin, and
mental impairment in the patient’s mother some time before her death
from cerebral hemorrhage. There was no other report of mental disease in
the family.)

It appears that in the interval between attacks, Agnew had been working
very hard and had been fairly successful in paying off a mortgage on his
house. A marked elation, somewhat natural, followed this success and
continued to an abnormal degree. Agnew labored under considerable
excitement, was over-fussy, and at times showed a flight of ideas. His
mania or hypomania gradually diminished and depression set in, in which
depression he arrived for consultation. He had marked ideas of
self-accusation, was emotionally unstable, wept much, and showed a
characteristic retardation of activities and unrest.

=Physically=, there was no neurological disorder. The patient appeared
rather under-nourished. The heart borders lay 2 cm. to the right and at
11½ cm. to the left of the mid-sternal line. The aortic second sound was
very loud. There was a moderate radial arteriosclerosis. Systolic blood
pressure was 210, diastolic 155.

The high blood pressure suggested nephritis, possibly of
arteriosclerotic origin, but urine examination and blood-nitrogen tests
yielded no evidence of kidney disease. Moreover, it is our experience
that a manic-depressive psychosis in persons past middle life is not
infrequently complicated by high blood pressure. In point of fact, some
authors insist upon a relation between manic-depressive psychosis and
the arteriosclerosis which rather frequently sets in in this disease.

Routine examination of the blood serum, however, yielded a positive W.
R. Following the approved rule of making an examination of the spinal
fluid in all mental cases having a positive serum W. R., we proceeded to
lumbar puncture. The fluid was clear and contained 35 cells per cmm.,
the albumin was in excess, and there was a positive globulin reaction.
The gold sol reaction was of the “paretic” type; the W. R. was strongly
positive.

On this basis, it seems worth while to consider the diagnosis of GENERAL
PARESIS or that of some form of non-paretic neurosyphilis. The former is
the diagnosis which we prefer.

    1. What is the classical differential diagnosis between
      manic-depressive psychosis and neurosyphilis? The laboratory tests
      have naturally supplanted the older purely clinical methods of
      differential diagnosis. The difficulties lodge, in the first
      instance, in depressive states. It would appear to be impossible
      on purely clinical grounds in certain cases to tell the depression
      of neurosyphilis from the depression of manic-depressive
      psychosis, since the slightly greater interest in the outer world
      taken by manic-depressive patients and their greater
      responsiveness to diagnostic threats (suggestion that patient is
      to be pinched or cut) are of no special value in the individual
      case. Identical considerations hold for the maniacal phases of
      manic-depressive psychosis, for these maniacal phases may even
      develop delusions (Kraepelin) of precisely the same nature as the
      characteristic expansive delusions of the excited paretic.

    2. If the clinical symptoms are insufficient in differential
      diagnosis, are not the pupillary signs and the speech defect of
      greater value? They are of value if present, but as in the case of
      Agnew, the victim of neurosyphilis may show no pupillary or speech
      disorder. Instances are familiar, also, in which the pupillary and
      speech signs are absent in very advanced cases of non-paretic or
      even of paretic neurosyphilis.

    3. Would not a circular course or recurrence of attacks be decisive
      for manic-depressive psychosis? Paretic neurosyphilis sometimes
      exhibits the same circular or recurrent course. We conclude that
      neither the clinical symptoms, the classical pupillary and speech
      signs, nor the ups and downs of a particular disease, are at all
      decisive as between manic-depressive psychosis and paretic
      neurosyphilis. Resort must be had to laboratory tests.

    4. What is the significance of the high blood pressure in paretic
      neurosyphilis? Work from our laboratory (Southard and Canavan) has
      shown plasma cells in the kidneys in 17 out of 30 paretics (56%),
      and in 16 of these 17 paretics with renal plasmocytosis, the
      plasma cells were found in the periglomerular region. What the
      relation of these findings may be to heightened blood pressure is
      as yet unknown. The severe syphilitic involvement of the aorta so
      characteristic in paretic neurosyphilis, as in other forms, may
      possibly have a bearing on blood pressure.


  =A POSITIVE SERUM WASSERMANN REACTION associated with mental
  symptoms (even with grandiosity) does NOT prove the EXISTENCE OF
  PARETIC NEUROSYPHILIS (“general paresis”).=


=Case 11.= Juliette Lachine came to a general hospital with pain in the
right upper quadrant of the abdomen, wherein was found an enlarged
liver. This liver was regarded as syphilitic on the ground that the
patient had a positive serum W. R. and that her two elder children were
clearly suffering from congenital syphilis. The liver mass was promptly
reduced by antisyphilitic treatment of the classical sort. When,
however, the patient was given an injection of salvarsan, she shortly
began to develop marked mental symptoms, whereupon she was removed to
the Psychopathic Hospital.

The =mental picture= at the Psychopathic Hospital was as follows: Lack
of orientation for time, marked distractibility of attention, with a
certain jumping from one subject to another, delusions of a religious
nature, claims of wonderful powers possessed by the patient, moods
variable, though as a rule of a euphoric and elated nature, with
laughing and singing. The activity seemed to be of a mental rather than
a peripheral nature. The patient did not regard herself as mentally
abnormal. The liver was still 4 cm. below the costal margin in the
nipple line. We found the W. R. to be positive in the serum but negative
in the spinal fluid. In fact, the spinal fluid was entirely negative.

So far as we are aware the picture presented by this case is one of
MANIC-DEPRESSIVE PSYCHOSIS. We regard the disease as merely complicating
the syphilis, although it is entirely possible that some visceral
condition incidental to the syphilis might be proved (in a higher stage
of psychiatric science) to have produced the mania.

In any event, the patient quite recovered from her mental symptoms in a
month. She was then able to tell us of a previous attack of depression
some 12 years previously, namely, at the age of 26. It appears that she
had at that time been committed to a hospital for the insane.

    1. In this case, in which the diagnosis of manic-depressive
      psychosis and not paretic neurosyphilis was made, are we sure that
      the symptoms that we term manic-depressive psychosis were not
      actually produced by syphilotoxins? In other words, in the absence
      of spinal fluid signs of inflammation or chemical change, might it
      not be possible for generalized syphilis outside the nervous
      system to produce manic-depressive symptoms? There is so far in
      the literature no experimental or other evidence of syphilotoxins.
      The existence of products and substances permitting the W. R. and
      the gold sol reaction is not of course evidence of syphilotoxins.
      Although there is no evidence of soluble syphilotoxins, it is
      thought that in the so-called Järisch-Herxheimer reaction (the
      intensification of clinical symptoms after salvarsan injection)
      effects may be due to the liberation of products from the killed
      bodies of spirochetes. Such endotoxins are not here in question.

    2. Is visceral syphilis, such as gumma of the liver, able to produce
      characteristic syphilitic reactions in the spinal fluid? We have
      had an autopsied case in which there was a “paretic” gold sol
      reaction of the fluid (though without other signs). The autopsy
      showed gummata of the liver. However, the finer anatomy of the
      nervous system showed a mild but definite meningo-encephalitic
      process, which was doubtless responsible for the gold sol
      reaction.

    3. What is the value of grandiose ideas? Ballet distinguishes two
      groups of grandiose ideas: (_a_) ideas of self-satisfaction,
      including ideas concerning extraordinary capacity, strength,
      power, and wealth on the part of the patient; and (_b_) ideas of
      ambition; the latter being of a more exact, constant, uniform and
      systematizing nature. The more vague and less systematized ideas
      of self-satisfaction rest in a phase of contentedness and
      optimism; the more definite ideas of pride and ambition are
      responsible for striking transformations of personality. General
      paresis shows, according to Ballet, these ideas of
      self-satisfaction in their most developed form. A certain
      variability, absurdity, incoherence, and contradictoriness
      characterize these ideas and the patient has little or no insight
      into their nature. When such ideas occur at the outset of the
      disease, they naturally may be of medicolegal interest. Cotard
      explains these ideas of megalomania on the part of paretics on the
      ground that they are essentially motor or will disorders and rest
      upon a sort of hyperbulia, exhibiting itself in exuberant
      activity. Régis has thought that the delusional generosity and
      liberality of the paretic, and his willingness to lend his wealth
      and talents to social progress, is helpful for diagnosis when
      contrasted with the more personal egoism of the victim of
      manic-depressive psychosis. The self-satisfaction of the
      manic-depressive patient often does not reach a delusional stage,
      but remains a mere feeling of pathological well-being or euphoria.
      The maniacal patient may compare himself with some great man but
      he does not identify himself with him. It must be remembered that
      these ideas of self-satisfaction occur also in alcoholism, but
      according to Ballet they occur only in the dementing phase of
      chronic alcoholism, and have no special diagnostic value. They may
      be a clinical stumbling-block for a time in the cases of alcoholic
      pseudoparesis. As for the ideas of ambition in which the patients
      believe themselves to be princes, emperors, divine messengers, and
      the like, these are less characteristic of paretic neurosyphilis
      than of delusional psychoses of a non-syphilitic nature. At all
      events, such ideas if definite, of long-standing, and systematized
      by the patient to form a thorough-going portion of his life, are
      not characteristic of neurosyphilis. The victim of paretic
      neurosyphilis can as a rule be persuaded out of his delusions, at
      least for the time being. These distinctions, it must be added,
      are hardly of value in the early cases of any of the psychoses in
      question, and cannot be made as a rule in either private or
      psychopathic hospital practice. Typical examples of grandiosity,
      although not so frequent as might be thought from textbooks, are
      always on display in institutions for the chronic insane.


  =PARETIC NEUROSYPHILIS (“general paresis”) may look precisely like
  DEMENTIA PRAECOX. Autopsy.=


=Case 12.= Henry Phillips remains a striking case in the memory of those
who knew him and his medical findings. Phillips came to the hospital
voluntarily at 42 years of age from the bank where he worked as a clerk;
he came at the suggestion of his employer. It seems that he had been
annoying his associates because he had fallen into a habit of
continually scratching himself. Phillips was entirely sure that he was
the victim of what he called the “Scotch itch,” and explained off-hand
that this itch had been put upon him by the Free Masons as a matter of
revenge because he would not join their order. He said once, for
example: “At times I feel like raising Hell; then I get a psychic
intimation; and then I get to using a foot-rule on my back and to
slapping my face.” He explained this psychic intimation as coming from
the order of Scottish Rites. Another example of talk is as follows: “My
father is a fighting man; that is part of it. They mean to throw me
down. I am through now trying for membership in the Free Masons. They
have good cause, they must fight. They do not want me for some personal
matters. I can go just so far in agreeing and seconding their advances,
but in the end it fails. I have no strength nor endurance.”

Aside from these delusions, there was little abnormality to be found,
though his recollection for minor events of the immediate present was
inaccurate. He was rather abnormally impulsive, gesticulating a good
deal while talking, and was of the appearance that the laity call
“nervous.” It appears that he had always been peculiar, subject to
violent fits of temper, in which fits he might throw things at other
members of the family. He always had pronounced likes and dislikes which
he never concealed. He had never had friends, had always been secretive;
and he was often termed a great student. For some five years he had been
studying Japanese from time to time, associating himself with a
Japanese.

It never does to jump at the diagnosis dementia praecox. However, the
picture seemed characteristic enough for the paranoid form of this
disease. Physically, Phillips had no particular abnormality; the
knee-jerks were a little lively, and the pupils reacted a little
sluggishly. However, the routine W. R. of the serum proved to be
positive. Examination of the spinal fluid was resorted to,—as in all
cases with a positive serum W. R.—and it also proved to be positive and
strongly so; the globulin and albumin were increased, and there was a
pleocytosis. A diagnosis of neurosyphilis was hardly avoidable. Phillips
later admitted a chancre, which he claimed was located on the mucous
membrane of the cheek and acquired by using the same utensils as his
Japanese friend, which friend, he stated, had active syphilis.

Antisyphilitic treatment of considerable intensiveness was begun, with
intravenous injections of salvarsan and intraspinous injections of
salvarsanized serum, but the patient grew steadily worse. His mental
symptoms became more marked, although not especially characteristic of
general paresis. =Neurologically,= he did develop signs more suggestive
of general paresis, and 18 months later died.

The =autopsy= showed features of GENERAL PARESIS. It is not necessary to
enter into the question of the details of histological correlation at
this time.

    1. What conclusion can be drawn from lively knee-jerks? Lively
      knee-jerks are of very little significance. Not only certain
      neurosyphilitics but also a variety of neurotic persons, victims
      of dementia praecox and hysteria, are very prone to have active
      tendon reflexes. Of course, extreme degrees of exaggeration are of
      importance, and especially an association of the hyperreflexia
      with the Babinski reaction, the Gordon, or Oppenheim reflexes,
      ankle clonus, and the like.

    2. Is there any special or differentiating factor in an extragenital
      chancre as against a genital chancre? Probably this question
      should be answered in the negative. Some have claimed that
      chancres draining by lymphatic channels of the head are more
      likely to lead to cerebral syphilis. This idea cannot be said to
      be established.

    3. Is there any significance in the story, if true, that Phillips
      acquired his syphilis from a Mongolian? It seems to be fairly well
      established that syphilis of the nervous system is extremely rare
      in China and Japan, whereas bone syphilis is very frequent there.
      It has been held that this has to do (_a_) with strains of
      spirochetes, (_b_) with the state of civilization, or (_c_) with
      the degree of “syphilization.” Apparently when a race is first
      infected with syphilis the lesions are chiefly of the cutaneous
      and osseous systems; only in later generations the vascular and
      nervous systems suffer. However, involvement of the nervous
      systems of Mongolians resident in this country is no rarity, a
      point possibly in favor of the theory of special strains affecting
      the nervous system as prevalent in western countries. Little or
      nothing is known as to the effect of transmission from one race to
      another, as from Mongolian to Caucasian in Phillips’ story.


  =NEUROSYPHILIS is NOT to be entirely ruled out by a negative serum
  Wassermann Reaction; for the fluid Wassermann Reaction may be
  positive.=


=Case 13.= William Twist is a case of note in the matter of the
so-called preparetic period (the idea of Charles L. Dana which was
scoffed at when first proposed by him in 1910). The patient, a very
successful traveling salesman, 35 years of age, was admitted to the
Psychopathic Hospital showing a typical picture of general paresis.

Thus, =mentally=, the patient showed elation, grandiosity (millions of
dollars to give away), intellectual weakness, disorder of memory, lack
of judgment, rambling talk, speech defect, omission of letters in
writing and spelling.

=Neurologically=, there was tremor of the lips, slight irregularity of
the pupils, which however reacted well, and lively knee-jerks.

Mr. Twist had sought advice at our out-patient department in his
thirty-third year. The records show that at that time he was somewhat
depressed, and his speech was even then, according to his own statement,
stammering. However, we found the W. R. at that time to be negative in
the blood serum. It appeared that his mother had died of consumption;
his father was said to have committed suicide. A brother had once
recovered from an attack of depression, presumably an attack of
manic-depressive psychosis. Accordingly, we thought at the time that the
case was probably one of manic-depressive psychosis. Moreover, our
routine serum W. R. failed to indicate any syphilitic process. As for
the so-called stammering of speech, this appeared to be a matter of the
patient’s own recollection rather than of our observation. In any event,
the patient had gone into the country and appears to have entirely
recovered; falling, again, however, into mental difficulties after a
short period, and finally arriving at the hospital in the
above-mentioned classical condition.

The W. R. in the blood serum proved again negative. The test was
repeated a number of times; also, after salvarsan had been given. The
salvarsan did not act provocatively, and the blood serum has remained
consistently negative.

In cases of syphilis the W. R. is at times negative. Swift claims that
in such cases an injection of salvarsan will often produce a positive W.
R. if the blood is tested on several days following the injection.

The spinal fluid, however, did show a positive W. R. as well as a gold
sol reaction of a “paretic” type. There were at the first examination
194 cells per cmm., there was a moderate excess of albumin, and a
positive globulin test. In short, there was no question of any other
diagnosis than GENERAL PARESIS.

    1. How can the negative W. R. of the blood serum be explained? It is
      difficult or impossible to explain this. Figures differ as to the
      percentage of cases of general paresis with negative blood serum;
      perhaps 3 to 5% of these cases yield a negative serum W. R.

    It is important to note the long preparetic period: at least a year
      and a half. Could our diagnostic methods be sharpened a trifle,
      such cases as these could be obtained early in this preparetic
      period and it might then be safe to promise good therapeutic
      results.

    2. What is the nature of the preparesis of Dana? When Dana’s brief
      paper on preparesis was written, there was of course hardly any
      idea that cases of paretic neurosyphilis could be cured or would
      recover, except possibly vanishingly few _curiosa_ about which
      there would always rage a diagnostic question. Accordingly, Dana,
      having found certain cases that seemed to him to have early signs
      of paresis but had apparently been cured by treatment, proposed to
      call them cases of preparesis. His idea was that he would thereby
      not offend those who held that general paresis was theoretically a
      fatal disease. With modern work and the display of more and more
      atypical cases of neurosyphilis, and the observation of relatively
      numerous cures or remissions under treatment, the designation of
      preparesis for a separate entity, or even for a sub-form of
      neurosyphilis, becomes superfluous.

    3. What is the percentage of cases of paretic neurosyphilis that
      show a negative serum W. R.? Among the best figures are those of
      Müller, who found that of 386 examples of paretic neurosyphilis,
      379 showed all reactions positive, or 98.5%.

    4. What is the meaning and value of the so-called provocative
      salvarsan injection? In practice, there may be a series of
      negative W. R.’s in the blood serum before a positive reaction is
      finally obtained, owing to technical difficulties or biological
      peculiarities. Where intensive work is being done upon the
      neurosyphilis problem, it is beyond question desirable to make the
      W. R. test upon at least three separate samples of blood drawn at
      intervals, for the second or third test may prove positive. This
      situation makes the interpretation of the so-called provocative
      salvarsan injection exceedingly doubtful; that is, the reaction
      might have been positive on repetition without the injection of
      salvarsan. The present case, as above stated, failed to yield a
      serum W. R. even after repeated tests and the “provocative.”

    5. What is the significance of the irregular pupils in this group?
      Paretic neurosyphilis shows inequality of the pupils in a high per
      cent of cases. Irregularity of outline of the pupils is commonly
      thought to be an important sign and to suggest neurosyphilis. It
      is true that many cases of pupillary irregularity are syphilitic,
      but the sign is of little or no differential value since
      congenital malformations and relics of old injuries and adhesions
      may produce effects identical with those of neurosyphilis.


  =DIFFUSE (that is, meningovasculoparenchymatous[5]) NEUROSYPHILIS is
  typically associated with six positive tests (serum Wassermann
  reaction, fluid Wassermann reaction, spinal fluid gold sol reaction,
  pleocytosis, positive globulin, excessive albumin); but one or more,
  and frequently several, of these tests are likely to run mild as
  compared with the tests in PARETIC NEUROSYPHILIS (“general
  paresis”). The clinical course of the diffuse (and especially the
  meningovascular) cases is likely to be protracted, with a good
  prognosis as to life (barring fatal vascular insults).=


=Case 14.= We shall present the case of John Jackson, a surveyor, 31
years of age, suffering from a left hemiplegia, with this in mind: To
exhibit difficulties in diagnosis in the presence of an embarrassment of
symptomatic riches.

The patient arrived at the hospital, in the first place, because he had
been threatening a woman who lived next door to him. He believed that
this neighbor had been talking about him and circulating reports against
him. Excited by these ideas, he had threatened to cut her throat.

Now the occurrence of hemiplegia in adult life before the approach of
senium is always suspicious of syphilis, and this suspicion we naturally
entertained from the beginning. However, there was upon the scalp a
crooked linear furrow about six inches long, running from the vertex to
the right parietal eminence. Another furrow about an inch long was
present upon the forehead. These furrows appeared to be of a bony nature
and were not tender. There was evidence of an old decompression
operation on the right side of the head; there were also large scars on
both sides of the neck, evidently the result of old operations; and
there were numerous palpable glands—the largest about the size of a lima
bean—all firm and not tender.

[Illustration:

  Station in syphilitic hemiplegia. Syphilitic pigmentation of skin.
]

It seems that at the age of eight, according to the patient’s mother,
Jackson had received a head injury and had remained unconscious for
three weeks. Upon recovery, he had to relearn both to walk and to talk;
however, he was able to begin school where he left off. He became more
nervous and irritable after the accident than previously. Nothing
further had developed until, at about 25 years of age, a tubercle was
discovered in his eye (the right pupil was smaller than the left,
reacting more slowly; right iris bound down by adhesions, with white
opacity of anterior chamber). For two years, 25 to 27, the patient was
under medical treatment for tuberculosis, and at the conclusion of this
period numerous glands were removed from the neck and diagnosticated
tuberculous. However, the neck did not heal and he carried bandages upon
it for two years.

At 28, the patient’s mother described the occurrence of a slight shock,
with head retraction, for a minute or two, and inability to speak.
Thereafter there had been five or six similar attacks, less severe, and
without loss of speech. The attacks were never accompanied by convulsive
movements. Then occurred a paralytic stroke, leaving the patient with a
left hemiplegia, which had somewhat improved. Mentally, the patient had
gone down hill, becoming less alert and more apathetic, and to some
extent amnestic. One had to consider, accordingly, the somewhat doubtful
possibility of post-traumatic and post-operative conditions, and the
question of tuberculosis (possibly errors in diagnosis; the lungs showed
no evidence of tuberculosis).

=Physically,= the signs of a left hemiplegia were appropriate.
Spasticity on the left side was found; there were Babinski, Gordon,
Oppenheim reflexes and ankle clonus on the left side (all absent on the
right). Speech defect was present. =Mentally,= aside from the delusions
noted at the beginning of our analysis, a striking feature was the
patient’s childishness. While reciting delusions, the patient was
overactive and evinced a somewhat childish interest. Arithmetically,
Jackson had preserved a fair ability but his apathy and lack of interest
interfered with tests, and possibly also with the exercise of memory. As
above noted, we were compelled to maintain the suspicion of syphilis
throughout despite the attractive hypotheses of traumatic and
post-decompressive effects and cerebral tuberculosis. A history of the
acquisition of syphilis an unknown number of years before admission
entered to strengthen the suspicion of the syphilitic nature of the
mental symptoms.


       TYPICAL LABORATORY FINDINGS IN NEUROSYPHILIS  (NONNE, 1915)
 ─────────────┬─────────┬─────────┬─────────┬───────────────┬───────────
   DIAGNOSIS  │ W. R.,  │  W. R.  │ SPINAL  │   PHASE I,    │PLEOCYTOSIS
              │  BLOOD  │0.22 CC. │ FLUID,  │   GLOBULIN    │
              │  SERUM  │  BLOOD  │ 1.0 CC. │               │
              │         │  SERUM  │         │               │
 ─────────────┼─────────┼─────────┼─────────┼───────────────┼───────────
 PARESIS OR   │POSITIVE │POSITIVE,│POSITIVE,│POSITIVE,      │POSITIVE,
   TABOPARESIS│  IN     │  85–90% │  100%   │  95–100%      │  ABOUT 95%
              │  ALMOST │         │         │               │
              │  100%   │         │         │               │
              │         │         │         │               │
 TABES (not   │POSITIVE,│POSITIVE,│POSITIVE,│POSITIVE,      │POSITIVE,
   combined   │  60–70% │  20%    │  100%   │  90–95%       │  90%
   with       │         │         │         │               │
   paresis)   │         │         │         │               │
              │         │         │         │               │
 CEREBROSPINAL│POSITIVE,│POSITIVE,│POSITIVE │POSITIVE almost│POSITIVE
   SYPHILIS   │  70–80% │  20–30% │  ALMOST │  always;      │  ALMOST
              │         │         │  ALWAYS │  NEGATIVE only│  ALWAYS
              │         │         │         │  EXCEPTIONALLY│
 ─────────────┴─────────┴─────────┴─────────┴───────────────┴───────────
                                                                 CHART 8


[Illustration:

  Syphilitic thrombosis. Contours of brain preserved.
]

The W. R. proved positive in blood and spinal fluid. The gold sol
reaction was of the syphilitic type; 37 cells were found per cmm.; there
was a slight amount of globulin and a slight excess of albumin.

We made a diagnosis of CEREBROSPINAL SYPHILIS rather than general
paresis on account of, first, the slow course of the disease; second,
the vascular type of the cerebral insult, hardly typical of paresis; and
third, the mild spinal fluid reaction. Treatment will hardly cure the
hemiplegia, at least so far as restoration of cerebral tissues lost in
the insult is concerned. We were perhaps entitled to consider that, as
in the cases of Petrofski (17), O’Neil (19), Robinson (45), the
meningitic process could be arrested. Unfortunately, our treatment of 20
injections of salvarsan over a period of 10 weeks, followed by a number
of months of bi-weekly injections of mercury salicylate, proved
incapable of making any change in the mental and physical picture or in
the laboratory findings.

    1. Can we explain the apparently poor reaction to treatment of the
      cerebrospinal syphilis in the case of Jackson by supposing a more
      deep-seated involvement than the meningovascular involvement
      indicated by the hemiplegia and the signs in the fluid? Autopsied
      cases in our experience show focal parenchymatous involvements
      that have not caused obvious clinical symptoms at any time during
      the course of the disease. These symptomatically silent lesions
      may have been present.

    2. What is the comparative prognostic value of seizures in paretic
      neurosyphilis and in such a meningovascular case as that of
      Jackson? Paretic seizures are often and indeed characteristically
      recovered from. Moreover, autopsies in paretic neurosyphilis
      characteristically show no gross focal destructive lesions to
      correspond with the seizures. The paretic seizures are apparently
      more irritative than paralytic. However, the seizures of the
      meningovascular group of neurosyphilis are also, though less
      commonly, recovered from, so that the differential diagnosis on
      the basis of the outcome of seizures is not safe. Rarely paretic
      neurosyphilis itself also develops seizures from which no recovery
      is made.

    3. What is the relation of neuropathic heredity to neurosyphilis?
      The family history of John Jackson is undoubtedly poor, since his
      father died of diabetes and a paternal uncle was insane; and on
      the mother’s side, the grandmother died of tuberculosis and an
      aunt died insane. This general question was more interesting in
      the days before the syphilitic nature of general paresis and of
      allied diseases was known. However, we may still hold perhaps that
      not only syphilis but also various intoxications, especially
      alcoholism, do flourish upon a neuropathic soil. This question,
      like that of Krafft-Ebing’s celebrated claim of the relation
      between syphilization and civilization, needs revision in the
      light of more extensive applications of the W. R. in larger and
      larger groups of persons under various community conditions.


  =The SIX TESTS (serum Wassermann reaction, fluid Wassermann
  reaction, pleocytosis, gold sol reaction, globulin, excess albumin)
  are likely to run STRONGER in PARETIC NEUROSYPHILIS (“general
  paresis”) than in DIFFUSE (especially meningovascular)
  NEUROSYPHILIS; in particular, the gold sol reaction is likely to
  prove “paretic” rather than “syphilitic.” The clinical course of
  paretic neurosyphilis (“general paresis”) is likely to terminate in
  death within a few years.=


=Case 15.= Pietro Martiro was a well developed and nourished man, 30
years of age, who had been doing erratic things and acting peculiarly
for a few weeks before entering the hospital. In the hospital, Martiro
proved to be very excitable and given to violence. He had marked
delusions of grandeur, saying he was worth many millions of dollars, was
the greatest singer in the world, the greatest athlete in the world, and
the like.

=Physically=, there was no disorder except overactivity of some
reflexes. The diagnosis of GENERAL PARESIS offered no difficulties, and
it was confirmed by the laboratory tests (positive serum and fluid W.
R., “paretic” gold sol reaction, 42 cells per cmm., an excess of
albumin, and a positive globulin test).

=Treatment=: The perfect physique of this case and the extremely brief
clinical duration (a few weeks) would naturally suggest a probably
favorable outcome. However, cases with marked delusions of grandeur have
very frequently proved to be cases with extensive brain tissue loss as
shown in certain studies with Danvers material.

In any event, the treatment in this case proved unavailing. Enormous
doses of salvarsan, twice a week, aided by mercury and potassium iodid,
were given. Although other cases had been helped by such intensive
treatment, Martiro went steadily downhill, nor was there the slightest
diminution in the intensity of any of the spinal fluid reactions. After
50 injections of salvarsan over a period of 30 weeks without
improvement, treatment was discontinued. A few months later, the patient
died.


               =PARETIC NEUROSYPHILIS (GENERAL PARESIS)=

                          =PHYSICAL SYMPTOMS=

                      EARLY HEADACHE
                      VISUAL DISORDER
                      HYPALGESIA
                      ADIADOCHOKINESIS
                      ATAXIA
                      NASOLABIAL FLATTENING
                      VOCAL CHANGE
                      SPEECH DISORDER
                      WRITING DISORDER
                      LOSS OF MANUAL DEXTERITY
                      PUPILLARY CHANGES
                      REFLEX CHANGES
                      SEIZURES
                      LATE: PARALYSIS, CONTRACTURE

                                                               CHART 9


               =PARETIC NEUROSYPHILIS (GENERAL PARESIS)=

                           =MENTAL SYMPTOMS=

      INTAKE IMPAIRED
      CONSCIOUSNESS CLOUDED
      FATIGUABILITY INCREASED
      HALLUCINOSIS RARE
      AMNESIA—RECENT! CHRONOLOGY AND STORAGE IMPAIRED. FABULATION
      OVER-SUGGESTIBILITY
      JUDGMENT IMPAIRED
      FANTASTIC DELUSIONS
      INSIGHT INTO ILLNESS NIL
      EARLY IRRITABILITY OR HEBETUDE
      QUICK SHIFTING EMOTION
      CHARACTER CHANGE
      CONDUCT SLUMP

                                                              CHART 10


    1. What is the duration of paretic neurosyphilis (“general
      paresis”)? If we omit the doubtful, early, and prodromal stages
      and count the beginning of the disease with the occurrence of
      definite symptoms, we find (Kraepelin) that almost half the
      patients with pronounced paretic signs die within the first two
      years of their disease. Kraepelin’s observations upon 244 cases
      are as follows:

                  Year:   1  2  3  4  5 6 7 8 9 10 14
                  Cases: 51 63 52 41 22 4 5 2 2  1  1

      The average duration of the disease in months has been calculated
      as varying from 24 to 32 months. Juvenile paresis runs a slower
      and more insidious course. The duration of paresis, according to
      many observers, diminishes with the increasing age of the patient.
      It is now held that a combination of tabes with paresis does not
      prolong the duration of the paresis. As noted above in the
      discussion of Case Harrison (9), our conceptions of the
      characteristic duration of paretic neurosyphilis must alter with
      the increase of our knowledge due to the early application of
      laboratory tests.

    2. What is the significance of the term _general paresis_? The case
      of Martiro is, of course, a good instance to show that the term is
      sometimes a misnomer. The characteristic generalized motor
      incapacity denoted by the term _general paresis_ is shown in
      patients in the institutions for the chronic insane in their last
      few months of life. The term _paresis_ is perhaps to be preferred
      to the term _paralysis_ because the paralysis is not complete but
      partial; but perhaps the best reason is that the word _paresis_ is
      a shorter word. When the mental side is to be emphasized, the term
      _paralytic dementia_ is employed. In this book we have used the
      term _paretic neurosyphilis_ to mean a more precise statement of
      the etiology of general paresis (general paralysis, paralytic
      dementia). The lay term, _softening of the brain_, like the terms
      _metasyphilis_ and _parasyphilis_ is in the present phase of our
      knowledge to be eschewed.

[Illustration:

  Euphoria in paretic neurosyphilis (“general paresis”). The head, arms
    and trunk were shaking with mirth; hence, the indistinct outlines of
    the photograph.
]

    3. If this fatal case be typical of general paresis (for more
      favorable results, see Part V), what is the toll of deaths from
      this disease in the community at large? A striking statement may
      be quoted from Dr. Thomas W. Salmon’s “Analysis of General
      Paralysis as a Public Health Problem:”

      “With the information in our possession at the present time, we
      are able to state that not fewer than 1000 persons in whom general
      paralysis is recognized die in New York State every year. Let us
      compare this with the lives lost from some other important
      preventable diseases. It means that _one in nine_ of the 6909 men
      who died between the ages of 40 and 60 in New York last year died
      from recognized general paralysis and that _one in thirty_ of the
      5299 women who died in the same age-period died from this disease.

      “The number of deaths from general paralysis in New York last year
      about equalled the number of deaths from typhoid fever. The
      following table gives the number of deaths due to the ten most
      important specific infectious diseases. Of course, deaths in
      measles, typhoid fever and scarlet fever will be found also under
      the names of some of the complications of these diseases, but it
      should be remembered that these primary diseases are not
      invariably fatal as general paralysis is. Many of the patients
      with measles who died from bronchopneumonia would have recovered
      but for this complication, while the paretics with
      bronchopneumonia would have died even if this complication had not
      arisen. No attempt is being made to compare the _prevalence_ of
      general paralysis with that of other diseases—we are trying only
      to estimate its share in the _mortality_.

              “1. Tuberculosis (all forms)         16,133
               2. Pneumonia                         9,302
               3. Bronchopneumonia                  7,217
               4. Diphtheria and croup              1,854
               5. Influenza                         1,381
               6. Measles                           1,071
               7. Typhoid Fever                     1,018
                  _General paralysis (recognized)_  1,000
               8. Scarlet fever                       837
               9. Whooping cough                      818
              10. Syphilis                           782”


               =PARETIC NEUROSYPHILIS (GENERAL PARESIS)=

                           =CHARACTERISTICS=

                      AMNESIA
                      QUICK SHIFTING EMOTIONS
                      CHARACTER CHANGE
                      CONDUCT SLUMP
                      NERVOUS DISORDERS
                      SPEECH DISORDERS
                      PUPILLARY CHANGES
                      REFLEX CHANGES
                      SEIZURES
                      CEREBROSPINAL FLUID PICTURE

                                                              CHART 11


                         =SYPHILITIC PSYCHOSES=

                      SYPHILITIC NEURASTHENIA
                      GUMMA
                      SYPHILITIC PSEUDOPARESIS
                      APOPLECTIC CEREBRAL SYPHILIS
                      SYPHILITIC EPILEPSY
                      SYPHILITIC PARANOIA
                      TABETIC PSYCHOSIS
                      HEREDITARY
                      PARESIS

                                                       KRAEPELIN, 1910

                                                       CHART 12


  =TABOPARETIC NEUROSYPHILIS (“taboparesis”) is CLINICALLY a
  combination of the symptoms of TABES DORSALIS and those of GENERAL
  PARESIS. The COURSE of TABOPARESIS is likely to be from a
  characteristic tabes dorsalis (often of years’ standing) to a
  characteristic general paresis; the ultimate paretic picture is
  likely to retain various characteristics of tabes. The LABORATORY
  TESTS in the paretic phase are characteristic of ordinary
  (non-tabetic) general paresis. The PROGNOSIS, after the paretic
  phase has arrived, is apt to be that of general paresis.=


=Case 16.= Joseph Sullivan, a waiter, 50 years of age, sought assistance
at the Psychopathic Hospital voluntarily. His complaint of severe and
lancinating pains in the legs, difficulty with his gait, and a feeling
of constriction about the waist, was forthwith suggestive of tabes
dorsalis. He was a rather poorly nourished, white-haired man, with a
drooping of the left side of the face. The pupils reacted sluggishly to
light, the right somewhat better than the left. A marked Romberg
reaction could be demonstrated. Ataxia in walking was marked. There was
some incoördination of the hands, considerable tremor, and writing was
poorly performed. The ankle-jerks and knee-jerks were absent. On the
whole, the diagnosis of TABES DORSALIS was clear enough.

The most appealing situation was =mental=. Sullivan was exceedingly
apprehensive about his condition on the ground that it was growing
progressively worse; if it was to get worse, Sullivan feared he would
commit suicide. From his own account, he had become irritable,
quick-tempered, and often unreasonable. As usual in these cases, the
question arose whether the depression was psychopathic or natural.


        =TABETIC SYMPTOMS AND SIGNS IN ORDER OF THEIR FREQUENCY=

                         =ANALYSIS OF 250 CASES=

                                                                PER CENT

  1. ROMBERG SIGN                                                   96.4
  2. ABSENT KNEE-JERKS                                              90.0
  3. LANCINATING PAINS                                              88.4
  4. STAGGERING GAIT                                                87.2
  5. ARGYLL-ROBERTSON PUPIL                                         80.0
  6. ATAXIA IN UPPER EXTREMITIES                                    68.2
  7. SPHINCTER DISTURBANCES                                         67.6
  8. SENSORY DISTURBANCES                                           58.2
  9. VISUAL DISTURBANCES                                            43.6
 10. PARESTHESIA AND NUMBNESS OF FEET AND LOWER EXTREMITIES         42.8
 11. GIRDLE SENSE                                                   31.2
 12. PTOSIS OF EYE-LIDS                                             23.2
 13. PARESTHESIA OR NUMBNESS IN HANDS OR UPPER EXTREMITIES          13.6
 14. STRABISMUS                                                     12.0
 15. VISCERAL CRISES                                                12.0
 16. LOSS OF SEXUAL DESIRE                                          11.5
 17. CHARCOT JOINTS                                                  9.2
 18. VERTIGO                                                         4.0
 19. MAL PERFORANS                                                   3.2
 20. PAIN IN JOINTS                                                  2.8
 21. RECTAL TENESMUS                                                 2.8
 22. MENTAL DEGENERATION (other than paresis)                        2.4
 23. HEMIPLEGIA                                                      2.4
 24. VESICAL TENESMUS                                                2.0
 25. DIFFICULTY IN ARTICULATION                                      2.0
 26. DEAFNESS                                                        1.2
 27. ANOSMIA                                                         0.8

                                                          BALDWIN LUCKE.

                                                                CHART 13


While in the hospital things shortly came to a crisis. In the midst of a
fit of depression, Sullivan attempted suicide by beating his head
against the wall. Whether this attempt could be regarded psychopathic,
however, remained in question. Sullivan had been drinking very heavily
although he had stopped about six weeks before admission, fearing that
the alcohol was causing a development of symptoms. The remedy was almost
worse than the disease because he then became more nervous, lost his
appetite, and had a marked insomnia.

According to the patient’s own history, he had had several attacks of
gonorrhœa and a syphilitic infection at the age of 19; that is, some 31
years before admission to the hospital. However, the first
_neurological_ symptoms of which the patient was aware came about 27 or
28 years after infection, namely, 3 or 4 years before admission, when
facial paralysis developed. At that time, he had suddenly felt a
peculiar sensation in the throat and became unable to swallow for a
time. His voice remained hoarse and low for some time, and his face
began to droop. The lancinating pains and the ataxia also dated back
several years.

    1. How shall we evaluate the mental symptoms? The prognosis of tabes
      dorsalis is relatively good so far as life is concerned, and it
      might even be possible for Sullivan by training to remain capable
      of being a waiter. The manual incoördination was not marked, and
      possibly the manual tremor was in part due to alcohol.
      Accordingly, the mental symptoms, such as emotional lability and
      memory defect, were in the foreground of attention. In point of
      fact, the laboratory examinations showed positive W. R. in the
      serum and the spinal fluid, which latter also contained 60 cells
      per cmm., positive globulin, and an excess of albumin. THE
      DIAGNOSIS MADE WAS THAT OF TABOPARESIS, meaning thereby a tabes
      associated with appropriate symptoms of a mental nature.

    2. How shall the term _taboparesis_ be used? Some use the term, as
      we feel erroneously, for instances of general paresis which happen
      to show crural areflexia (absence of knee-jerks). We feel that the
      best usage of the term is for instances in which well-defined
      symptoms of tabes (as well as of paresis) are present, namely,
      characteristic ataxia, lightning pains, and the like. If the term
      is used more loosely, as above mentioned, then practically every
      case of general paresis might perhaps be termed _taboparesis_,
      since almost every case of paresis does show involvement of the
      cord as well as of the cerebrum. Such involvement may lead to
      hyperreflexia, hyporeflexia, or areflexia according to the
      localization of the process. In true taboparesis, in which there
      is a commingling of the features of tabes with those of paresis,
      we should find the posterior roots of the spinal cord affected.
      The spinal lesions of paresis itself are more apt to be
      intraspinal; that is, confined to the nervous system within the
      pial investment.

    3. Bearing in mind that Sullivan was a waiter, what shall be said
      about the infectivity of these cases? It is counted as a rule as
      negative, since there are no open spirochete-bearing lesions. The
      longer the period since infection the less, as a rule, is the
      chance of contagion in syphilis; and as tabes and paresis occur
      fairly late in the disease, the infectiousness at this stage is
      practically negligible.

    4. Of what differential value is the insight shown by Sullivan into
      the nature of his symptoms? Kraepelin remarks that a genuine
      insight into the nature of the disease does not as a rule occur in
      paresis. At the beginning of the disease, there may sometimes be a
      correct understanding of the nature of the disease and of its
      probable outcome; but the presence or absence of insight into the
      fact of mental disease is by no means a differential sign of
      practical value.

    5. What is to be said of the occurrence of depression and excited
      states in paretic neurosyphilis? A variety of classifications of
      sub-forms of paretic neurosyphilis have been propounded.
      Kraepelin, for example, deals with four: the demented, depressive,
      expansive, and agitated forms, but remarks that the division is
      merely convenient for exposition. The institutional intake does
      not accurately represent the distribution of cases. Under
      psychopathic hospital conditions with the relatively easy resort
      to such institutions, the number of quiet cases increases; under
      the less advanced conditions in Heidelberg, Kraepelin took in 53%
      demented paretics as against 56% at Munich (73% women) under the
      easier conditions of admission. The admissions of demented
      paretics varied from 37 to 56%. The variations depend much upon
      the facility with which the cases can be brought to institutions.
      Where admission is beset with various legal restrictions, the
      quiet and demented cases are more apt to be treated for long
      periods at home. The depressive type of paretic neurosyphilis
      forms a much smaller group, according to Kraepelin, as only about
      12% of his Heidelberg admissions were of this type, and still
      fewer of his Munich admissions. Other authors give percentages as
      high as 16 and 19. The so-called expansive group is larger,
      Kraepelin finding 30% of his Heidelberg cases to be of this group,
      and 21 to 22% of his Munich cases. The rarest sub-form of paretic
      neurosyphilis is the agitated form: 6% of Kraepelin’s Heidelberg
      admissions; 14% among males and 5% among females in his Munich
      admissions, where the diagnosis of agitated paresis was entered on
      somewhat broader lines. French authors (Sérieux and Ducaste) have
      enlarged the number of sub-forms of paretic neurosyphilis as
      follows: Expansive 27%; sensory 24%; demented 24%; persecutory 3%;
      depressive 2%; circular 7%; hypochondriacal 7%; and maniacal 6%.


  =DIFFUSE (meningovasculoparenchymatous) NEUROSYPHILIS may look
  precisely like PARETIC NEUROSYPHILIS (“general paresis”) at certain
  periods of clinical and laboratory examination.=


=Case 17.= The police found Gregorian Petrofski crouching on his knees
on a Boston sidewalk, attempting to take pickets off a fence. Petrofski
knew little English; he said that he had slept in Poland the night
before. He did not appear to be alcoholic.

When he was examined, through an interpreter, he told how he had been in
America two days, and in Boston two years; that he was at the present
time in Poland, and that his brother had brought him to the hospital and
left him there.

The =physical examination= showed Petrofski to be well developed and
nourished. His pupils were somewhat dilated and reacted somewhat slowly
to light and accommodation. =Neurologically,= there was nothing else
abnormal found upon systematic examination although, through lack of
coöperation, sensory and coördination tests proved difficult if not
impossible. There was a large ulcer on the under surface of the glans
penis, with several small smooth scars on the upper surface. There was a
purulent discharge from the external meatus. There were exostoses of
both tibiae.

The initial diagnosis had to consider uremia and diabetes, which could
be easily excluded on examination. Alcoholism was excluded through
absence of alcohol on the breath. There remained such diagnoses as
epilepsy, some post-traumatic condition, or meningitis, to say nothing
of the hypothesis of syphilis raised by the tibial exostoses and the
lesions of the penis. The hypothesis of trauma was given up, as well as
epilepsy and meningitis upon the data of the lumbar puncture. The spinal
fluid proved to be clear but with enormous amounts of globulin and
albumin, 80 cells per cmm., a “paretic” gold sol reaction, and a
positive spinal fluid W. R. (the serum W. R. was also positive).
Accordingly, it was clear that the case was one of neurosyphilis.

Treatment was instituted with injections of mercury salicylate, a grain
and a half twice a week, and potassium iodid. After some weeks,
diarrhoea and salivation with marked symptoms of mercury poisoning set
in; the treatment was suspended, but later re-instituted. In a few weeks
Petrofski was apparently quite well, the spinal fluid tests had all
become negative, as had the serum W. R.

Petrofski now began to pick up a good deal of English, and gave a
consistent narrative of his past life, although the period just prior to
and during his early stay in the hospital has remained blank. Without
further treatment Petrofski has remained well for over a year.

    1. Does the “paretic” gold sol reaction mean general paresis? In
      connection with this general question, a brief summary of the
      significance of the gold sol reaction in this group may be made.
      (1) Fluids from cases of general paresis in the vast majority of
      cases will give a strong and fairly characteristic reaction,
      especially if more than one sample is tested. (2) Very rarely
      general paresis fluid will give a reaction weaker than the
      characteristic one. (3) Fluids from cases of syphilitic
      involvement of the central nervous system other than general
      paresis often give a weaker reaction than the paretic, but in a
      fairly high percentage of cases give the same reaction as the
      paretics. (4) Non-syphilitic cases may give the same reaction as
      the paretics; these cases are usually chronic inflammatory
      conditions of the central nervous system. (5) When a syphilitic
      fluid does not give the strong “paretic reaction” it is
      presumptive evidence that the case is not general paresis, and
      this test offers a very valuable differential diagnostic aid
      between general paresis, tabes, and cerebrospinal syphilis. (6)
      The term “syphilitic zone” is a misnomer, as non-syphilitic as
      well as syphilitic cases give reactions in this zone, but no fluid
      of a case with syphilitic central nervous system disease has given
      a reaction out of this zone, so that the finding may be used
      negatively; and any fluid giving a reaction outside of this zone
      may be considered non-syphilitic. (7) Mild reactions may occur
      without any evident significance, while a reaction of no greater
      strength may mean marked inflammatory reaction. (8) Tuberculous
      meningitis, brain tumor, and purulent meningitis fluids
      characteristically, though not invariably, give reactions in
      higher dilutions than syphilitic fluids. (9) The unsupplemented
      gold sol test is insufficient evidence on which to make any
      diagnosis, but used in conjunction with the W. R., chemical and
      cytological examinations, it offers much information, aiding in
      the differential diagnosis of general paresis, cerebrospinal
      syphilis, tabes dorsalis, brain tumor, tuberculous meningitis, and
      purulent meningitis. (10) We believe that no cerebrospinal fluid
      examination is complete for clinical purposes without the gold sol
      test.


          =FREQUENT SYMPTOMS IN DIFFUSE AND VASCULAR NEUROSYPHILIS=

                 =(“CEREBRAL” AND “CEREBROSPINAL SYPHILIS”)=

                      PUPILLARY DISORDER
                      HEADACHE
                      VERTIGO
                      INSOMNIA
                      DROWSINESS
                      CHANGE IN DISPOSITION
                          Irritability    Slow thinking
                      SEIZURES
                      PARALYSES
                          Permanent    Transient
                      APHASIA
                      HEMIANOPSIA
                      SENSORY DISTURBANCES
                      GASTRIC CRISES
                      SPHINCTER DISTURBANCES
                      INTRACRANIAL PRESSURE SYMPTOMS
                      POLYURIA, POLYDIPSIA, GLYCOSURIA
                      MÉNIÈRE’S SYNDROME
                      NYSTAGMUS

                                                              CHART 14


      See Appendix B for technical details.

    2. What is the relation of the tibial exostosis to neurosyphilis?
      The syphilographers have always stressed the tibial lesions in the
      diagnosis of syphilis. Although not so much attention has been
      paid to these and kindred osseous lesions in neurosyphilis, yet we
      have frequently found such lesions and they afford an important
      auxiliary means of diagnosis.


  =A POSITIVE SERUM Wassermann reaction with a NEGATIVE FLUID
  Wassermann Reaction may be found in NEUROSYPHILIS, particularly in
  VASCULAR NEUROSYPHILIS: the remaining signs in the fluid, although
  frequently positive, may even be negative.=


=Case 18.= Frederick Wescott was a promoter, an elderly looking man of
60 years. His health had been failing for 18 months. There had been
shortness of breath, dizziness, a tired feeling, inability to “get the
words he wanted,” and forgetfulness of names. About eight weeks before
examination, Wescott had had a convulsion, following which he had been
unable to express himself at all well. This convulsion was not
accompanied by loss of consciousness. Besides a marked motor aphasia,
there was agraphia.

=Physically=, Wescott showed arteriosclerosis and a blood pressure of
135 systolic, but, except very lively knee-jerks, no other reflex
disorders or anomalies were discovered. In particular, the pupils
reacted fairly well.

There was, perhaps, no special reason to implicate syphilis in the case,
yet Wescott gave a history of syphilis at 35 years. The W. R. of the
blood serum proved positive; that of the spinal fluid was negative, and
the albumin was but slightly increased; there was a very slight amount
of globulin, and there were 16 cells per cmm. in the fluid. The gold sol
reaction suggested syphilis.

We felt entitled to make a diagnosis of SYPHILITIC CEREBRAL
ARTERIOSCLEROSIS, regarding the convulsion or seizure eight weeks before
as due to a vascular insult. The laboratory picture in the spinal fluid
in Wescott’s case seems to be rather characteristic of this group of
syphilitic arteriosclerotics.

    1. What is the reason for the negative spinal fluid W. R.? The
      theory would be that the syphilitic lesion is localized in the
      vascular system and that the parenchyma is only secondarily, if at
      all, involved. The W. R. producing bodies are accordingly not
      found in the fluid.

    2. How frequently are several of the spinal fluid tests negative,
      while others are positive? Whereas, clinically speaking, the five
      tests in the spinal fluid (W. R., globulin reaction, excess
      albumin, pleocytosis, and gold sol reaction) are each indicative
      of a pathological condition in the central nervous system, yet a
      specially intensive study of the distribution of these tests has
      shown that they are prone to occur independently. Consequently, we
      must concede that they do not all represent the same inflammatory
      products and chemical conditions. The W. R. producing bodies, the
      gold sol reaction producing bodies, as well as the globulins and
      albumins, have been proved to be separate. Special work has also
      shown that _these tests disappear under treatment at different
      rates_. There is, unfortunately, no doubt that the rate and
      intensity, presence or absence, and the order of disappearance of
      these tests in either treated or untreated cases, do not at all
      parallel the clinical conditions of the patients.

    3. What is the prognosis in vascular neurosyphilis, such as in the
      case of Wescott? The prognosis is identical with that of cerebral
      arteriosclerosis in general, that is to say, bad, but with
      frequent periods of improvement. In the neurosyphilitic type of
      arterial disease thromboid formation is frequent. Where the lesion
      is chiefly perivascular infiltration, rather than disintegration
      of the vessel wall, improvement may very well occur as a result of
      treatment. Wescott showed slight improvement under treatment. He
      has already lived two years since his first convulsion, and three
      and a half years since the onset of symptoms.


  =DIFFUSE NEUROSYPHILIS (so-called “cerebrospinal syphilis”) is often
  marked by SEIZURES.=


=Case 19.= Agnes O’Neil, an unmarried woman of 28 years, was first
examined five weeks after the initial symptoms. It appears that she had
had certain seizures, with unconsciousness and twitching of the limbs
(otherwise not well described), followed by confusion of mind and
sometimes by a weakness of the left side and a difficulty in speaking.
Headache had been almost constant, as well as pains in the arms and
legs.

=Physically=, both in general and =neurologically=, there were no signs
or symptoms; mentally, we could discover no symptoms. Syphilis was
denied, although possible exposure to syphilis was admitted.

The =diagnosis= of some form of organic brain disease was clear with the
picture of convulsions followed by slight aphasia with headaches and
limb pains. With onset at 28, the most frequent cause for such
epileptiform seizures is certainly syphilis. Examination of the blood
and spinal fluid showed a positive W. R., in both. The albumin was also
somewhat increased. The clinical picture suggested a fairly generalized
meningitic involvement.

The =prognosis= in such cases of generalized meningitic involvement is
in general good, and this principle was illustrated in the O’Neil case,
in which the symptoms soon disappeared under intensive antisyphilitic
treatment. In fact the spinal fluid W. R. became negative in the course
of four weeks. The blood serum W. R., however, has remained positive
despite eight months of active treatment.


                =CONDITIONS IN WHICH CONVULSIONS OCCUR=

      NEUROSYPHILIS
      HYSTERIA
      EPILEPSY MAJOR (GRAND MAL)
      EPILEPSY MINOR (PETIT MAL)
      DEMENTIA PRAECOX
      TOXIC CONDITIONS:
          Asphyxia, Uremia, Alcohol, Absinthe, Lead, Mercury, etc.
      ORGANIC BRAIN LESIONS
          Apoplexy, Meningitis, Intracranial Growths
      STOKES-ADAMS DISEASE
      MALINGERING
      DISSEMINATED SCLEROSIS

                                                              CHART 15


    1. Are certain cases of syphilitic epilepsy really cases of
      Jacksonian epilepsy? As a matter of nomenclature, Jacksonian
      cortical epilepsy is usually the result of a focal and
      circumscribed irritative lesion in the cortex. Gumma, local
      syphilitic meningitis, and syphilitic vascular lesions, as well as
      scars consequent upon the latter, are among the causes of
      Jacksonian epilepsy, along with such other focal lesions as
      trauma, tumor abscess, tubercle, and the like. Even non-syphilitic
      Jacksonian epilepsy has been observed from time to time in cases
      of diffuse intracranial pressure. Jacksonian attacks also have
      been found in so-called genuine epilepsy. Accordingly, we must not
      conclude from the occurrence of Jacksonian convulsions, even
      though in a proved syphilitic case, that the convulsions in
      question are surely due to a focal lesion, for they may be due to
      diffuse syphilitic lesions.

    2. What is the significance of aphasia in Agnes O’Neil? Aphasia is
      not a characteristic symptom in ordinary Jacksonian epilepsy, but
      the aphasia is another sign of focal lesion and forms an added
      argument against the diagnosis of genuine or idiopathic epilepsy.
      See also discussion of aphasia in paretic neurosyphilis under Case
      Levenson (22).

    3. What is the behavior of the serum W. R. and the spinal fluid W.
      R. under systematic treatment? Sometimes, as in this case, the
      serum W. R. remains positive and the fluid W. R. becomes negative;
      but in other equally well-defined cases, the reverse holds true,
      and the serum W. R. reaction becomes negative whereas the spinal
      fluid reaction remains positive. The obvious conclusion is that we
      cannot always be sure even by faithful tests of either the serum
      or the fluid alone, whether the treatment has succeeded in
      abolishing the laboratory signs.

    4. Can this case be regarded as one of cure? Not by the definition
      adopted in this book or by the syphilographers who take into
      account not only the nervous system but the body which contains
      it. To be sure, the spinal fluid of Agnes O’Neil is now entirely
      negative and she is clinically free from symptoms; yet from the
      broad standpoint of syphilis therapy in general, this patient is
      not cured, as is evidenced by the positive serum W. R.


  =PARETIC NEUROSYPHILIS (“general paresis”) is often marked by
  SEIZURES.=


=Case 20.= Lester Crane, a plumber, 37 years of age, came to the
hospital with a slow and defective speech. Moreover, there seemed to be
some mental disorder since his answers to questions were not always
relevant. It appeared that he was seeing bugs on the wall.

=Physically=, Crane was a well developed and nourished man, with
overactive knee-jerks and a Babinski reaction on the left side.

It developed that there was an impairment in hearing. The pupils reacted
well both to light and to distance. The patient was very restless and
smiled in a silly fashion. His memory was decidedly defective in all
spheres, and he was very slow in the intake of ideas.

The plumber’s wife said that, at about the age of 23 or 24, he had a
spell of confusion lasting two or three days, with peculiar conduct,
unintelligible talk, and a good deal of weeping. The medical diagnosis
at that time took into account the fact that Crane was a plumber and was
“lead encephalopathy.”

However, according to his wife, Crane had acquired chancre at about 26
years, was treated mercurially for about three years and declared well.
He had remained well up to about 18 months before entrance, when,
without previous warning, the patient had a convulsion with the
continuous movements for about half an hour. He was semi-conscious for
about 18 hours and vomited continuously. There was amnesia for the whole
affair on regaining consciousness. In a week’s time, Crane was entirely
well. But six weeks later there was another convulsion. Upon removal to
a hospital, the diagnosis of general paresis was made, and the patient
was given the Swift-Ellis intraspinous treatment. This seemed to be very
successful, and the patient discontinued treatment after 14 weeks
(during which time there had been seven treatments) on the ground that
he was entirely well.

However, after discontinuing treatment, there was another convulsion in
about a month, and further convulsions occurred once a month. For six
months, however, the patient took no treatment, but finally returned to
the hospital and was given mercury. This treatment appeared to suspend
convulsions again for three months, but at the expiration of six months,
the patient had three convulsions in one day, and several more during
the following days. After the last of these convulsions, there had been
numbness on the right side of the body and considerable headache.

The diagnosis of PARETIC NEUROSYPHILIS (“general paresis”) is borne out
by the laboratory tests. The W. R. of the blood serum was, to be sure,
negative, but the W. R. of the spinal fluid was positive, and there was
a “paretic” type of gold reaction, together with other laboratory signs.

The case well demonstrates that group of paretic cases in which
convulsions periodically occur, leaving the patient worse after each
convulsion. Treatment with salvarsan was instituted, and mercury and
iodid was given by mouth. During the period of eight months which have
now elapsed since the beginning of this treatment, there have been no
convulsions; there has been a great improvement in the memory, the
hearing has improved, the W. R. in the spinal fluid is much less
intense, the gold sol test has become negative, and the other tests are
all less intense.

The patient, however, has not been entirely well, for in place of the
generalized convulsions, he has had minor seizures, beginning as a rule
with a tingling sensation in the right hand, extending up the arm, down
the trunk and leg, and through the right side of the face, with a bitter
sensation on the right half of the tongue. The patient maintains that
this sensation is absolutely confined to the right half of the body (in
this connection we may recall case Morton (1), in which there was also a
hemiplegia together with other apparently hysterical symptoms at several
times during the long course of a disease with abundant structural
correlations). During these minor seizures, the patient is unable to
talk, although he does not lose consciousness and is entirely aware of
everything going on about him. These attacks have of late been growing
somewhat less frequent.


                        =LOSS OF DEEP REFLEXES=

  NEUROSYPHILIS
  NEURITIS
      (alcohol, diabetes, diphtheria, lead, arsenic, tubercle,
         cachexia, etc.)
          Peripheral nerves sensory or motor
  PERIPHERAL NERVE PALSIES
  TEMPORARILY FROM COMPRESSION BY TOURNIQUET
  FRIEDREICH’S ATAXIA
  SUBACUTE COMBINED DEGENERATION OF POSTERIOR AND LATERAL COLUMNS
          Posterior column disease
  FOCAL LESION IN GRAY MATTER OF CORD
  INFANTILE PARALYSIS (ACUTE ANTERIOR POLIOMYELITIS)
  PROGRESSIVE MUSCULAR ATROPHY
      (chronic anterior poliomyelitis)
          Anterior cornua of cord
  AMYOTROPHIC LATERAL SCLEROSIS
  SYRINGOMYELIA
  THROMBOSIS OF ANTERIOR SPINAL ARTERY
  LANDRY’S PARALYSIS
          Anterior cornua and peripheral motor nerves
  MYOPATHIES
      (pseudohypertrophic and atrophic types)
          MuscLe itself
  AMYOTONIA CONGENITA
  FAMILY PERIODIC PARALYSIS
      (during attacks)
  INCREASED INTRACRANIAL PRESSURE
      (especially hydrocephalus and tumors of posterior fossa)
  PNEUMONIA
  IMMEDIATELY AFTER ATTACK OF MAJOR EPILEPSY
      (post-epileptic coma)
  TOXIC COMA
      (uremia, morphine, etc.)
  DURING SPINAL ANESTHESIA
  COMPLETE TRANSVERSE LESION OF CORD

                                                         PURVES STUART

                                                           CHART 16


    1. What is the cause of the negative serum W. R.? It is claimed that
      3 to 5% of all cases of general paresis yield a negative blood
      serum. In this particular case, there had been considerable
      treatment, including some Swift-Ellis treatment, so that it may be
      that this treatment had reduced a formerly positive blood serum W.
      R. to a negative one.

    2. What is the nature of the typical seizures of general paresis?
      The most frequent seizures are epileptiform and bear a general
      resemblance to cortical epilepsy; but more rarely these seizures
      resemble the ordinary epileptic attack or consist of a violent
      general shaking of the whole body. A variety of initial minor
      disorders usher in the attacks: the temperature is often
      increased. The attacks are over after one or at most after a few
      hours. Kraepelin speaks of one that lasted 14 days. Sometimes a
      _status paralyticus_ develops, suggestive of the _status
      epilepticus_. Another rarer form of characteristic seizure is the
      apoplectiform, which can hardly be told from an ordinary stroke,
      and may be followed by the usual post-apoplectic phenomena. A good
      many of the strokes leading to sudden death in middle life are
      probably cases of neurosyphilis although often set down as early
      arteriosclerosis of a non-syphilitic nature. Besides the
      epileptiform and apoplectiform seizures, there are certain
      seizures of a less definite and complete nature, ranging from
      simple fainting spells, dizzy spells and petit mal attacks, to
      various special forms of irritative muscular contractions and
      temporary speech disorders. Sometimes these attacks occur with
      complete preservation of consciousness. Transient paresthesias,
      visual field defects, and especially attacks of vomiting, which,
      according to Kraepelin, may precede paresis by years (of course in
      this connection gastric crises of tabes must be thought of), may
      be counted as sensory seizures.

    3. What is the proportion of paretic cases developing seizures?
      Figures vary from 30 to 90%. According to Kraepelin, seizures
      occurred in 30 to 40% of his cases at Heidelberg; he was of the
      impression that treatment in bed had reduced the number of
      seizures. 65% of paretics admitted to Munich (under very free
      conditions of admission) were determined to have shown seizures
      before their admission to the hospital. Seizures are said to be
      somewhat more frequent in men than in women. These paretic
      seizures are not due to either hemorrhages or vascular plugging—at
      least in the vast majority of cases—and must be ascribed to the
      effects of microscopic injuries.

    4. What is the effect of seizures upon the future course of paretic
      neurosyphilis? The current idea as expressed, for example, by
      Mercier, is that “immediately after each crisis the patient is
      much worse than he was before it, and thereafter there is some
      improvement, but he never improves up to the point at which he was
      before the occurrence of the crisis.” That is, “The course of the
      disease is one of sudden plunges, each deeper than the last, each
      followed by a gradual recovery that is less complete than the
      recovery from the previous plunge.”

    5. During what period of the disease are seizures most common? Late
      in the disease many cases have convulsions, even though there were
      none for the first year or two. In other cases the convulsion is
      the first indication of paresis.


  =DIFFUSE (non-paretic) NEUROSYPHILIS (“cerebrospinal syphilis”) is
  often marked by APHASIA.=


=Case 21.= Martha Bartlett, a woman of 40 years, was brought to the
Psychopathic Hospital aphasic, or at least unable to talk distinctly
enough to be understood, or even to give name and address. The police
had found her wandering aimlessly about the streets. Although she was
well-dressed, she was mud-bespattered and apparently had not changed her
garments for several days. It shortly developed that the patient,
although unable to express herself either in words or by writing, could
understand everything that was said to her and could indicate by the
monosyllables _yes_ or _no_ whether she agreed or disagreed with
statements made. It was thus determined that she was pretty well
oriented. She was able to understand both speech and printed words.
Although she approximated more than is at all common a pure type of
_motor aphasia_, it appeared that there was a slight involvement on the
sensory side, especially in the sphere of visual imagery.

=Neurologically=, the patient showed moderate strabismus, slight
deviation of the tongue to the right, and considerable tremor on
protrusion of the tongue. The right side of the palate hung lower than
the left. The ankle and arm reflexes were possibly more active on the
left side, and the left grasp was somewhat better than the right. Both
knee-jerks were active, but again the reflex on the left side was more
active than the right. No other abnormalities of reflex were determined.
There was no Rombergism but the gait was somewhat ataxic. For the rest,
the physical examination was normal. The blood pressure was 120
systolic, 85 diastolic.


              =CONDITIONS IN WHICH SPEECH DEFECT IS FOUND=

      NEUROSYPHILIS
      HYPOGLOSSAL PARALYSIS
      FACIAL PALSY
      PARALYSIS OF PALATE (POST-DIPTHERITIC)
      BULBAR PALSY
      PSEUDOBULBAR PALSY
      MYOPATHY—FACIO-SCAPULO-HUMERAL TYPE OF LANDOUZY AND DEJERINE
      MYASTHENIA GRAVIS
      FRIEDREICH’S ATAXIA
      LARYNGEAL TABES
      ALCOHOLIC INTOXICATION
      POST HEMIPLEGIC
      LENTICULAR DISEASE
      BILATERAL ATHETOSIS
      MULTIPLE SCLEROSIS
      DEAF MUTISM
      PARALYSIS AGITANS
      CHOREA
      STAMMERING
      TICS
      HYSTERICAL APHONIA

                                                              CHART 17


The ready suspicion was that the case was one of apoplexy of slight
degree with post-apoplectic phenomena. Upon investigation, this
suspicion was confirmed since it appeared that Mrs. B. had been
apparently quite well until about six months before admission, when
without particular warning she began to act strangely and promptly fell
into a series of convulsions. These convulsions would begin with
twitchings of the face, and then spread throughout the body. There would
be a period of unconsciousness for two or three hours. It is not certain
how many of these convulsive seizures the patient had. At all events she
is reported to have recovered therefrom completely, remaining well for
three months; whereupon, suddenly, while visiting a friend, she suffered
a paralysis of the left side of the body. She remained dazed and had
hospital treatment for about a week. Ever since this left-sided
paralysis, the aphasic condition above described has persisted.

Such a phenomenon has often been dismissed in the past as due to an
early arteriosclerosis, but most neurologists and internists of today
would look beyond the diagnosis of mere arteriosclerosis and consider
syphilis. The only suggestive feature in the case, aside from the
post-apoplectic reflex disorder and spastic phenomena, is the
irregularity and diminished light reaction of the pupils. Our suspicions
were confirmed by the positive serum W. R. The W. R. of the spinal fluid
proved, however, to be negative. There was a moderately strong gold sol
reaction of the syphilitic type. There was a slight excess of albumin,
and there was an exceedingly slight amount of globulin. There was but
one cell per cmm.

On the whole, it would seem best to consider the case of Mrs. Bartlett
to be one of CEREBRAL ARTERIOSCLEROSIS OF SYPHILITIC ORIGIN, and a case
in which there is no evidence of meningitis or meningoencephalitis.

    1. What is the explanation of the negative spinal fluid W. R.? It
      may be that none of the W. R. producing bodies have gone over into
      the spinal fluid. It has been shown by the work of Weston that the
      W. R. producing body is not identical with the bodies responsible
      for the other tests in cerebrospinal syphilis. Moreover, it has
      been clearly shown that these several tests of the spinal fluid do
      not run at all parallel with one another. Especially is it true
      that the chemical tests do not correspond at all with the degree
      or nature of the pleocytosis. On the whole, when involvement of
      the nervous system is entirely vascular, it is not only
      theoretically proper but also practically common, to find a spinal
      fluid negative to several tests.

    2. Omitting consideration of the syphilitic gold sol of this case,
      what conclusion could be drawn from the albumin and globulin
      findings? It would not be warrantable to assume syphilis since it
      is a common finding after cerebral hemorrhage due to
      non-syphilitic arteriosclerosis to find excess albumin and also
      globulin in the spinal fluid. Occasionally, also, pleocytosis
      occurs in cases of cerebral hemorrhage even when the hypothesis of
      an active meningitis can be excluded. We may recall in this
      connection the pleocytosis in so-called meningitis sympathica of
      certain brain tumors. (See also the case of Milton Safsky (48), a
      case of brain tumor in which there was an excess of albumin, a
      large quantity of globulin, and a pleocytosis of 146 cells per
      cmm.)

    3. What can be expected from treatment in these cases of vascular
      cerebral syphilis? The condition offers very little opportunity
      for therapeutic results. However, antisyphilitic therapy is
      indicated to prevent if possible further progress of the lesions.
      Since the lesions are, however, vascular, and since it must remain
      a question how far these vascular lesions are due directly to
      spirochetal action, and since in any event it may be difficult to
      reach the spirochetes thus active, perhaps it is best to place
      most reliance on potassium iodid. In any event, potassium iodid
      should be given. Salvarsan and mercury are also indicated. It is
      common to warn against administration of large doses of salvarsan
      in this type of case on the ground that further vascular ruptures
      may be produced. (See Friedberg, 108.)

    4. If we conclude that the aphasia of the Bartlett case is due to
      vascular disease, can we conclude a relation between this vascular
      disease and vascular tension? It is not safe to draw such a
      conclusion. The Bartlett case itself showed low blood pressure. To
      be sure, some cases of neurosyphilis show high blood pressure from
      which one draws the _à la mode_ clinical conclusion to the effect
      that the kidneys are probably involved in the arteriosclerosis;
      but other cases do not show a high blood pressure but may in fact
      show a low blood pressure. The vascular disease doubtless
      responsible for the aphasia in the Bartlett case is probably not
      at all an effect of blood pressure conditions, but is, on the
      contrary, an effect of local syphilitic vascular lesions.


  =PARETIC NEUROSYPHILIS (“general paresis”) is often marked by
  APHASIA.=


=Case 22.= Meyer Levenson, a traveling salesman of 36 years, had for the
last two or three years been undergoing a change of disposition, quite
interfering with his work. He had begun to take unreasonable aversions
to people, had become irritable and emotionally depressed, and often
fell to weeping without cause.

About nine months before hospital observation, it seems that a
trunk-cover had fallen on Levenson’s head, and there is some question as
to whether he did not have a convulsion at that time. However, a month
later he had a definite seizure, followed by speech disorder, a slight
paralysis, and a staggering gait. Four weeks later, however, he had
gotten over these post-convulsive difficulties and had gone back to
work.

At his work, he became tired easily, his gait and speech did not seem
entirely normal, and there was a considerable memory disorder. After
five more months, another attack of a convulsive nature, with twitching
of hands and face and tongue-biting occurred, and the attending
unconsciousness remained for two days. Again improvement followed,
though without ability to return to work. Four (?) months later there
were several severe convulsions and Levenson would remain unconscious
for a day or two at a time. Restlessness, irritability, and irrational
talking followed.

=Physically=, the patient was fairly well developed and nourished; blood
pressure 168 systolic, 68 diastolic; pupils reacted very sluggishly to
light. There was a marked motor aphasia, which the patient recognized as
a speech difficulty. On the whole, however, Levenson was very euphoric
and was entirely sure that he was improving and would surely get well.

Shortly after entrance, Levenson had a severe convulsion, with
unconsciousness. The movements were mainly on the right side of the
body, and there was a post-convulsive weakness of the right side for
several days, followed by a slow recovery of strength.

The course of the disease—convulsions followed by improvement—is very
characteristic of a paretic onset. The =laboratory findings= were in all
respects confirmatory. It was rather striking that a permanent _motor
aphasia_ followed the convulsions in this case, since the seizures of
paresis do not in the vast majority of cases leave permanent paralyses.
The course of the disease continued to show convulsions, which would in
each instance leave him at a lower terrace of capacity than had been
before shown. The patient died four years after the onset of symptoms of
a general asthenia. With the exception of the permanent motor aphasia,
this case might be regarded as a fairly typical one of general paresis.

    1. What is the general nature of speech disorder in paretic
      neurosyphilis? Speech disorder is, along with the pupillary
      changes, one of the most important clinical symptoms in paretic
      neurosyphilis. There are aphasic and articulatory disturbances.
      The aphasia that accompanies paretic seizures is of a transient
      nature as a rule. A case with such long-standing motor aphasia as
      shown by Levenson is not common. Paraphasia, with incorrect naming
      of objects, may last longer. The so-called “sticking” phenomenon
      is often observed.

      Word-deafness is said to be rarer but is difficult to test on
      account of the patient’s dementia. Agrammatism (incapacity to form
      correct sentences) is sometimes observed. But the most
      characteristic disorder is in the syllabic composition of words.
      Syllables are left out (“medaltricity” for medical electricity),
      or fused (“exity”), or doubled (“electricicity”). Besides the
      central speech disorders of which the above are examples, there
      are disorders in articulation, which at first occur as a
      consequence of paretic seizures or in states of excitement, but
      later become permanent. These are divided into paretic and ataxic
      disturbances.

    2. What is the structural basis of these forms of aphasia? It is
      believed that they are due to microscopic changes, not to coarse
      destructive lesions.

[Illustration:

  BROOKLINE, MASS.
]

[Illustration:

  BROOKLINE, MASS.

  Mss. of Levenson, case 22. Paretic neurosyphilis. Tremor, misspelling.
    Metathesis of letters (Bk, not Br) omission of letters (Book).
]

[Illustration]

[Illustration:

  God save the Commonwealth of Massachusetts

  Mss. of Safsky, case 48, brain tumor. Tremor not marked. Misspelling,
    omission of letters. Wrong letters (h in hweth).
]

[Illustration:

  Mss. of Halleck, case 31, cervical tabes. No brain disorder.
    Pen-holding and bearing on difficulties. Crowding of phrases result
    of ataxia.
]

[Illustration:

  Mss. of Collins, case 61, paretic neurosyphilis. One misspelling
    (-chussetts); not psychopathic? Characteristic tremor.
]


  =REMISSIONS of identical appearance occur in PARETIC and in DIFFUSE
  (non-paretic meningovascular) NEUROSYPHILIS.=


=Case 23.= Thomas Donovan, a merchant 44 years of age, acquired syphilis
according to his own story at the age of 31, and he was at that time
treated at a well-known watering-place with mercurial injections. Later
he continued treatment under his family physician, and at 34 was
pronounced cured. However, four years later—that is seven years after
his initial infection and in his 38th year—he had his blood examined and
it proved positive. He was accordingly treated by salvarsan and his W.
R. became negative. The story did not end there, however, for at 43,
mental symptoms appeared of the nature of depression and a diagnosis of
paresis was made. He was released from the institution against advice at
that time, and without treatment, made a partial recovery.

A sudden outburst of violence brought Mr. Donovan to the Psychopathic
Hospital; he was very surly, combative, and difficult to manage,
standing 6′ 2″, and weighing 210 pounds. He was oriented only fairly
well and his surliness was streaked with humor. He facetiously said that
the Psychopathic Hospital was the largest hospital in the country, and
that it was, in fact, a horse hospital; that he had come because he
liked the surroundings, not to make money; that he was the healthiest
man in the world, never having been sick; that the Psychopathic Hospital
was a club, for which you have to get somebody to propose your name.
There was amnesia and no knowledge of current events. He regarded the
food as poisoned, refused to eat, and was very irritable and untidy.

=Physically=, there were few abnormalities, but the pupils failed to
react either to light or accommodation, and the knee-jerks and
ankle-jerks were absent. There was a slight Rombergism. There was a
marked speech defect to test phrases. Both serum and spinal fluid W.
R.’s were positive; the fluid showed 41 cells per cmm., there were large
amounts of globulin and albumin, and the gold sol reaction was of the
“paretic” type.


                       =ATAXIA OR INCOÖRDINATION=

            NEUROSYPHILIS
            LESION OF PERIPHERAL SENSORY NERVES
            DIVISION OF POSTERIOR ROOTS
            TUMORS OR CHRONIC SCLEROSIS OF POSTERIOR COLUMNS
            SUBACUTE COMBINED DEGENERATION
            VESTIBULAR ATAXIA
            FRIEDREICH’S ATAXIA
            FAMILY PROGRESSIVE HYPERTROPHIC NEURITIS
            THROMBOSIS POSTERIOR INFERIOR CEREBELLAR ARTERY
            MARIE’S HEREDITARY CEREBELLAR ATAXIA
            LESIONS OF CEREBELLUM, TUMORS, ETC.
            WRITERS’ CRAMP
            PREHEMIPLEGIA
            MULTIPLE SCLEROSIS
            PSEUDO-SCLEROSIS
            HYSTERIA

                                                              CHART 18


                 =CONDITIONS IN WHICH VERTIGO IS FOUND=

                   NEUROSYPHILIS
                   HEAD TRAUMA
                   CEREBRAL ANEMIA AND HYPEREMIA
                   MENOPAUSE
                   ARTERIOSCLEROSIS
                   RENAL DISEASE
                   CEREBRAL HEMORRHAGE AND THROMBOSIS
                   INTRACRANIAL TUMORS
                   MULTIPLE SCLEROSIS
                   EPILEPSY (AURA)
                   TOXIC CONDITIONS:
                       alcohol, tobacco, constipation
                   PSYCHONEUROSIS
                   OCULAR DISTURBANCES
                   EAR DISEASE
                   MÉNIÈRE’S DISEASE
                   MIGRAINE

                                                              CHART 19


Salvarsanized serum was injected intraventricularly through a trephine
opening in the right frontal region. Injections were made through the
corpus callosum into the third ventricle. There was progressive
symptomatic improvement after each of four injections. In fact, after
the fourth injection the patient was allowed to leave the hospital
despite the fact that there was only a slight improvement in the spinal
fluid findings. The speech defect had entirely disappeared. (Speech
defect, according to many authorities, including Kraepelin, is of very
grave diagnostic significance.) His memory returned. Mr. Donovan is now
able to handle figures rather extraordinarily well. He now has a good
insight into his delusions and tells stories about them with great
humor.

    1. What is the definition of a remission in general paresis?
      Remissions form a foil to seizures; just as seizures mark a sudden
      advance in the severity of the disease or may even lead to death;
      so remissions may cause a sudden cessation of both mental and
      nervous phenomena in the disease. Whereas the seizures occur most
      often, according to Kraepelin, in the demented types of paresis,
      the remissions occur in all cases except in the terminal phase.
      Kraepelin quotes Hoppe as observing pronounced remissions of long
      duration in 17% of male and 15% of female paretics. Gaupp observed
      marked improvement in less than 10%, and very marked improvement
      indeed in only 1% of his cases. Kraepelin states that such
      improvements are most frequent in agitated and especially in
      expansive forms of paresis, and that they are rarer and less
      complete in the depressive and demented forms. Sometimes the
      improvement occurs over night, although the full extent of the
      remission becomes complete only gradually, perhaps in the course
      of months. The sensorium clears, the disorientation disappears,
      the delusions retreat, and the former delusions are treated as
      dreams and imaginations. There is often a good deal of persistent
      uncertainty as to events during the height of the disease. The
      nervous disorders are far more obstinate than the mental. Still,
      both speech and writing may often greatly improve.

      Cotton in New Jersey found, among 127 cases of paresis
      diagnosticated by modern methods during seven years, that
      remissions occurred in but five, or about 4%, lasting from a half
      to three years.

    2. Does a remission ever amount to a cure? The classical case quoted
      in this connection is one observed by Tuczek. This case developed
      a picture of paresis in 1876, at the age of 36; and a remission,
      or cessation, of symptoms, occurred in 1878; but in 1883, at 43
      years, the patient developed a tabes without any trace of mental
      disorder, which tabes gradually advanced. By the middle of 1898,
      when the patient was 58, certain symptoms of excitement and
      confusion occurred, which led to death with dementia, 22 years
      after the beginning of the disease. Nissl pronounced the cortex to
      be undoubtedly the characteristic cortex of a paretic. This
      observation seems to indicate that a clinical remission tantamount
      to a clinical recovery may occur without the death of the
      spirochetes engaged. This observation is to be held in mind in
      connection with all therapeutic work with neurosyphilis.

      Nonne states that during his clinical experience of 19 years he
      had followed 10 cases of paresis with apparent recovery; but of
      these ten cases, four had to be thrown out by Nonne because the
      apparent recoveries turned out to be only long and almost complete
      remissions, finally issuing in characteristic dementia. Of the
      remaining six cases, perhaps two should hardly be counted as
      paretic and Nonne rather preferred to term them cases of
      syphilitic dementia in the sense of a non-paretic cerebral
      syphilis. At the end, therefore, of his review of observations,
      Nonne found himself with four cases of true recovery from paresis.

      Spielmeyer holds that there is no theoretical reason why paresis
      might not be cured, since all the different changes that have been
      described in the disease can be halted, and many of them can be
      repaired. In particular, he reminds us that the acute infiltrative
      process, the neuroglia reaction, and the phagocytic action of the
      large mononuclear cells are distinctly removable processes. (See
      discussion below under Section V, for apparent cures and
      remissions occasionally secured under treatment.)


  =REMISSIONS of identical appearance occur in PARETIC (“general
  paresis”) and in DIFFUSE (non-paretic) NEUROSYPHILIS.=


=Case 24.= Michael O’Donnell, a laborer of 48 years, came home, one day,
at 5:30, complaining of severe headache. His wife told him he should lie
down and, taking him by the arm, tried to help him to the bed. At this
moment, O’Donnell lost control of both left arm and left leg, and fell,
unable to move but with consciousness preserved. The wife noted that the
left side of his face was drawn up and that he drooled. He was at once
carried to a general hospital, remaining there for about three weeks,
talking at random in a delirious manner and tied in bed. Two
intraspinous injections of salvarsan were given, and O’Donnell showed
considerable improvement and went home.

However, upon his return from the hospital, he became very wilful, would
not remain in bed, and on one occasion actually took the mattress from
the bed, carried it to another room, and then returned to his own room
and slept upon the springs. He became irritable and emotional, insisted
upon going to the hospital, did not go there but upon returning home
insisted that he had been there. That night, O’Donnell left the house
only partly dressed.

It appears that O’Donnell had been excessively alcoholic, but that
before August 15, when he sustained the left-sided hemiplegia above
mentioned, there had been no symptoms except that in February he had
once been very dizzy. It appears that there had been another dizzy
spell, three nights before the paralysis, accompanied by a fall and
unconsciousness for about 15 minutes.


                   =TRANSIENT OR FLEETING PARALYSES=

                 NEUROSYPHILIS
                 MYASTHENIA GRAVIS
                 MYOTONIA CONGENITA (THOMSEN’S DISEASE)
                 PARAMYOTONIA CONGENITA
                 MYOTONIA ATROPHICA
                 INTERMITTENT CLAUDICATION
                 OCCUPATION NEUROSES
                 FAMILY PERIODIC PARALYSES
                 ETANY
                 EPILEPSY MINOR
                 HYSTERIA
                 MULTIPLE SCLEROSIS
                 APOPLEXY
                 CEREBRAL THROMBOSIS

                                                              CHART 20


O’Donnell was brought to the Psychopathic Hospital some six weeks after
the paralysis, complaining merely of a slight headache and desirous of
treatment. There were no mental symptoms of any sort. =Physically=,
O’Donnell was in general not abnormal (there was a slight pre-systolic
murmur and a blood pressure of 190 mm. systolic). The pupils were
slightly irregular, the left larger than the right; both reacted
sluggishly. Both ears were moderately deaf; the tendon reflexes of the
left arm and leg were somewhat more lively than those on the right. The
systematic =neurological= examination otherwise revealed no
abnormalities. The urine was negative. The serum W. R. was positive but
the spinal fluid reaction was negative. There were but 2 cells per cmm.,
and there was a very slight trace of albumin.

    1. How shall we account for O’Donnell’s transient paralysis? We
      might invoke brain tumor, alcoholic pseudoparesis, or some form of
      neurosyphilis. The diagnosis of brain tumor seems quite untenable
      in view of the absence of premonitory symptoms and in the absence
      of intracranial pressure. As for alcoholic pseudoparesis it is
      true that the patient was excessively alcoholic.

      However, against these two diagnoses and in favor of the diagnosis
      of NEUROSYPHILIS, are the positive serum W. R. and the pupillary
      reactions (although these are short of the true Argyll-Robertson
      phenomenon). Dizziness with retention of consciousness and
      associated with the paralyses mentioned suggests rather a
      subcortical than a cortical lesion. We are inclined to regard this
      lesion as probably THROMBOTIC, and to place it possibly in the
      region of the internal capsule. We are inclined to regard the
      phenomenon as purely vascular and as not in this case associated
      with an encephalitis. We are, however, not entirely satisfied with
      the diagnosis.

    2. What shall be said as to treatment? A full-blown left-sided
      hemiplegia may be produced even when the thrombotic lesion is
      itself exceedingly small. It is common to explain this on the
      basis that there is an area of collateral edema about the small
      necrotic, thrombotic, or hemorrhagic area responsible for the
      lesion. In short, numerous neurones are functionally rather than
      structurally affected, or at all events capable of early
      restitution of function.

    3. What is the prognosis in such cases? It appears that now and
      again patients run for several years without further trouble, both
      with and without treatment. We are inclined, however, to advocate
      treatment rather than absence of treatment for a variety of
      reasons. In the first place, vascular lesions may at any time
      become associated with meningitic lesions, and treatment by
      salvarsan may perhaps be counted on to head off this process;
      secondly, the treatment with iodids may possibly aid in the
      resolution of a local thrombotic process.

    4. What are the prodromal symptoms of cerebrospinal syphilis?
      According to Nonne, headache, dizziness, sleeplessness, mental
      symptoms of the irritability group, loss of capacity as to mental
      work, whether severe or not, and loss of capacity for difficult
      thinking; also impairment of memory. Nonne does not regard these
      phenomena as characteristic of syphilitic vascular disease, and
      calls attention to the fact that in every organic disease the same
      subjective symptoms occur. The triad—headache, dizziness, and
      impairment of memory—is for example now counted as a prodromal
      symptom complex for arteriosclerotic apoplexy (Cramer). Of course,
      apoplectic attacks occur without such preliminary symptoms:
      particularly, according to Nonne, the nocturnal attacks.

    5. Can the fleeting paralysis be of service in differentiating the
      diffuse from the paretic form of neurosyphilis? Probably not. In
      both forms transient paralyses occur as well as the permanent
      ones. In general, however, the transient paralyses are more
      frequent in paretic neurosyphilis, whereas the permanent ones
      occur more often in diffuse neurosyphilis.


  =There are cases of NEUROSYPHILIS in which the laboratory signs are
  positive but in which there are no clinical signs or symptoms
  (PARESIS SINE PARESI?).=


=Case 25.= Richard Lawlor[6] was admitted to the Psychopathic Hospital,
October 29, 1914, being sent there from a general hospital where he had
gone on account of a self-inflicted wound of the wrist, apparently made
in a period of depression with suicidal intent. Routine notes follow.

=Family History.= Paternal grandparents both died of heart disease.
Maternal grandfather died at seventy-two of dropsy. Moderately
alcoholic. Maternal grandmother died of shock at fifty-six. Father died
at age of forty, after an illness of eight years, from heart disease.
Father all his life was subject to fainting spells and headaches. The
only paternal cousin died at thirteen months of brain fever. Mother,
aged forty-seven, is, to say the least, eccentric. Says “she has several
times been given up from tuberculosis.” Two maternal uncles died of
tuberculosis, one from rupture, one from heart disease. One uncle who
“doesn’t know anything after he has a teaspoonful of liquor.” Several
other uncles and aunts whose history is not obtained. Patient is
mother’s only child. Mother was twice married. There were several
miscarriages by both husbands; patient child by first marriage.

=Past History.= Patient born thirty-two years ago, full term, normal
delivery and development. Measles, mumps, and chickenpox in childhood.
Subject to headaches since seven or eight years old. Kicked in the face
by horse at seventeen or eighteen, not considered serious. Hit by a
baseball three or four years ago, leaving him hard of hearing on left
side. Married ten years ago; no children because he says his wife needed
an operation. He denies venereal disease by name and symptoms. For past
ten years has had attacks of depression lasting but a short time, but
quite severe. Never caused him to quit work as a barber and he felt
better when working. His married life he says was fairly happy except
for his wife’s extravagances, and on this account he left her a little
over a year ago, and she has applied for a divorce, which he is willing
that she should have, but does not wish to give her alimony. He admits
moderate alcoholism.

=Present Trouble.= Patient states that since he left his wife a year ago
he has felt sorry a number of times. He has wished he had her back. He
has felt lonely. He has had six or eight periods of depression in that
time similar to those he has had for many years, lasting two or three
days, and sometimes a week. These were always precipitated by some cause
for worry. In these attacks he feels nervous, sleeps poorly, has little
or no appetite, sweats during his work and everything looks black.
Several times in these attacks he has had suicidal ideas. Ten months ago
he considered taking corrosive sublimate. For a little over a week
before entrance to hospital he had been out of work and had been
“sporting.” The day before entrance he had a telephone message from his
lawyer which upset him somewhat and he walked the floor all night. He
had just been shaving when the idea of suicide came to him. He sat down
a minute when suddenly the thought “to hell with the world” came to him;
he took the razor and slashed his wrist. He does not remember drawing
the razor across his wrist. As soon as he saw the blood he felt sorry,
called his mother, and was taken to an emergency hospital and then sent
to the Psychopathic Hospital.

=Physical Examination.= Patient is a well developed and nourished man
thirty-two years of age. Head is normal as to size and shape; there are
no scars or marks of injury. Hair and skin not remarkable in any way.
Ears negative to external examination. Teeth well kept; two missing,
several gold fillings. Tongue very slightly coated. Throat negative.
Tonsils easily visible without evidence of inflammation or exudation.
Neck, no thyroid enlargement, no abnormal pulsations, no adenopathy.
Chest, symmetrical, expansion good, resonant throughout. Breath sounds
transmitted normally. No râles or rubs heard. Heart, no enlargement or
cardiac dulness. Sounds of good quality, no murmurs heard. Rate regular.
Pulses equal, regular and synchronous, and of good volume and tension.
Systolic blood pressure 130, diastolic 65. Abdomen, flat, soft and
tympanitic throughout; no masses; no tenderness. Liver edge not felt,
below costal margin. Spleen not palpable. Extremities negative, except
for incised wound on left wrist.

=Neuromuscular Examination.= Pupils are large, round, regular, equal and
react readily to light and accommodation. No nystagmus, strabismus or
ptosis. No weaknesses or paresis of facial muscles. The tongue projects
medially and shows no tremor. The triceps and biceps reflexes are
readily elicited, and are quite active, as are the knee-jerks and
ankle-jerks. On one occasion it was thought that the tendon reflexes
were slightly more active on the left than on the right. This was never
confirmed; always afterwards found equal. There was no tremor of
extended hands. Abdominal reflexes not elicited. Cremasteric present on
both sides. The plantar response is flexor. There is no Babinski, Gordon
or Oppenheim. No Romberg. Coördination tests well performed. No speech
defect. No sensory disturbances. Urine examination negative.

Wassermann reaction in the serum: Positive, with cholesterinized
antigen; negative, with syphilitic fetal liver antigen.

Wassermann reaction in fluid positive on two occasions. Examination of
spinal fluid, November 4: globulin +++, albumin ++, 100 cells per cubic
millimeter; large lymphocytes, 8 per cent; small lymphocytes, 90 per
cent; plasma cells, 0.7 per cent; endothelial cells, 1.3 per cent.
November 11, globulin +++, albumin +++, cells 18 per cubic millimeter.
November 26, globulin ++, albumin ++, cells 92 per cubic millimeter;
large lymphocytes, 13.1 per cent; small lymphocytes, 82.1 per cent;
plasma, 1.2 per cent; endothelial, 3.6 per cent.

Gold sol, November 4, 5555432100.

Gold sol, November 26, 3332100000.

=Mental Examination.= On entrance to hospital patient seemed slightly
depressed and a bit irritable. This condition lasted two days, after
which he was agreeable and apparently entirely over his depression. Even
during his mild depression, however, he talked freely. There was no
evidence of retardation. He told his story readily. Orientation was
intact. Memory excellent. Educational knowledge well retained. There was
no evidence of any hallucinations or delusions.

    1. Was Richard Lawlor insane?

      There was, then, on the mental and physical examination nothing to
      make a definite suggestion of a psychosis, and the most one could
      think of was a psychoneurosis or a cyclothymia of at least ten
      years’ duration. The findings in the cerebrospinal fluid and the
      Wassermann reactions, however, give us material for thought.
      Certainly one cannot call the man insane; all who saw him agreed
      on this point.

    2. If Richard Lawlor should some day develop mental symptoms, what
      would be the genesis of the new psychosis? Though writers such
      as Fildes and McIntosh, and Swift, have suggested an
      anaphylactic or hyperallergic explanation for the development of
      symptoms after a normal interval; such a hypothesis could hardly
      obtain in the present case. The hyperallergic hypothesis for the
      development of tertiary neurosyphilis would run to the effect
      that in the secondary stages there had been a definite disease
      of the nervous system, which, however, absolutely cleared up,
      leaving no inflammatory vascular or parenchymatous relics of its
      existence. Nothing would on this hypothesis remain except a
      hypersensitisation of the tissues. In some later period of the
      now clinically normal person, one or more spirochetes from a
      lesion outside the nervous system are carried into the nerve
      tissues and there set up an anaphylactic or hyperallergic
      reaction. It is obviously difficult to prove the correctness or
      incorrectness of the hyperallergic theory without numerous
      examinations of the spinal fluid, in clinically normal persons
      after the secondaries have passed. The present case, so far from
      demonstrating a normal fluid, demonstrates a highly pathological
      fluid, even though there are absolutely no clinical symptoms
      which could be regarded as of nervous origin. The burden of
      proof at the present time would seem to lie with those who claim
      hyperallergy in neurosyphilis. We prefer on present evidence to
      think that at the conclusion of the secondaries a disease
      process often remains in the nerve tissues despite clinical
      quiescence.

    3. What is the prognosis in the case of Richard Lawlor? The
      prognosis _re_ neurosyphilis is doubtful. We have, however, boldly
      termed the condition _PARESIS SINE PARESI_, meaning thereby to
      suggest that the patient is in considerable danger of the
      efflorescence of a true diffuse or paretic neurosyphilis. We have
      no means of telling, however, whether the positive symptoms would
      be those of a paretic or a non-paretic neurosyphilis. As data
      accumulate regarding these cases of _paresis sine paresi_, we may
      be able finally to come upon some case in which trauma shall bring
      out the clinical symptoms of neurosyphilis. For discussion of this
      matter, see the case of Bessie Vogel (52) in Part III of this
      book.

    4. Should Lawlor have been brought to a psychopathic hospital? It is
      a safe working rule to have any person who attempts suicide
      observed. A large percentage of suicides occur in psychotic
      individuals and a suicidal attempt is not infrequently the first
      recognized abnormality. Immediate observation is a necessary
      safeguard against another more successful attempt.


  =Demonstrates SYMPTOMS and LESIONS of PARETIC NEUROSYPHILIS
  (“general paresis”). Autopsy.=


=Case 26.= John Morrill, 49, an operative in a mill town in Essex
County, Mass., was described as a “Saturday night and Sunday drinker,”
with a history of very serious long sprees at the age of 43. It seems
that he had had what was called “sciatica” at 35, and was treated in
hospital for seven weeks at that time. The nature of this sciatica is in
doubt, but there was a history of syphilitic infection at 36 years (scar
of glans).

Morrill had been married twice, and two of the children were dead; one
daughter was described as “very nervous,” but there were four children
under ten years of age, all regarded as perfectly healthy.

Morrill had been a mill operative of average capacity, was industrious,
and had supported his family despite alcoholism. The syphilis had been
treated with reasonable thoroughness.

Aside from alcoholism, there had been no symptoms up to two months
before admission to Danvers Hospital. Then there had been insomnia,
fatigue, agitation, eruption on foot, loss of ten pounds in weight,
hypochondriacal fears, apprehensiveness for the future of the children,
incoherent talk; and just before admission, his talk was described as
foolish. He had taken to running away and hiding in bushes by a pond and
in the cellars of other people’s houses.

The patient was of medium height and weight, with thin grayish hair and
grayish irides; musculature was slender. The face was blank in
expression, the teeth poorly preserved with atrophy of gums, the tongue
coated, and the breath foul. There was a gummy secretion of the eyelids,
an area of brownish branny eruption over both clavicles, a number of
depressed scars over the limbs and back, and another area of scaly
eruption on the right heel and the sole of the foot. The heart area was
increased, and the sounds were faint at the base, with the first sound
accentuated at the apex. The urine showed a trace of albumin.

=Neurologically=, the Romberg position was maintained with a general
tremor and fluttering of the eyelids. In complicated movements, the
patient was slightly ataxic. The pupils were irregular, the left being
much larger than the right. There were no light reactions to be obtained
in window light. The reaction to accommodation was present, though
slight. Vision was poor, ¼-inch capitals could not be read by left eye
at reading distance. The knee-jerks were diminished equally; the
Achilles jerks were absent; the other reflexes were normal. Upon the
sensory side, the patient gave a history of pains in the legs at
irregular intervals for several years. These pains he described as of a
darting character. There was little or no sensory disorder, although the
outer surface of the right leg required a deeper pressure to elicit
sensation. There were no disorders of muscle sense.

If Morrill was to be trusted, he had been born in Ireland, and had come
to the United States at the age of 17. He married at 18; there had been
seven pregnancies by the first wife, with one stillborn child; one child
had died at five weeks. The four children by the second wife were
healthy. The first signs of neuritis had occurred at 45 and had received
the diagnosis neuritis, although no connection between the neuritis and
the syphilis had been noted.

The patient entered the hospital July 26, 1904, and was discharged,
improved, January 5, 1905. He returned a little more than a year later,
January 15, 1906, and died March 21, 1906. The total duration of the
disease from the onset of mental symptoms may therefore be stated as
somewhat under two years. When the patient appeared at the hospital the
second time, he showed a positive Romberg sign, an unsteady gait, an
ataxia that still was moderate, and somewhat more marked tremors,
involving fingers, tongue, and face. He was now unable to read ½-inch
type with the left eye. The knee-jerks, formerly diminished, were both
exaggerated, the left slightly more so. The Achilles reaction, not
obtained formerly, now appeared on the right side. The pupils reacted as
before. The sensory loss had become more marked, since sharp and dull
points could hardly be distinguished. Deep pinpricks were not felt in
the leg, and heat could not be told from cold.

The speech in 1904 had been somewhat defective (“truly rural” rendered
as “tooly lualal,” “sifted soft thistles” as “thoft thsistles”), and
there had been little further development of the speech defect. The
handwriting had lost appreciably in legibility and had become much more
tremulous. During the first period of hospital observation Morrill had
what might possibly have been visual hallucinations, but it was
impossible to tell whether his story of seeing his wife and children
trying to get in through the window was hallucinatory or a matter of
fabrication. Memory was decidedly imperfect and few details of recent
events could be produced. The association of ideas was almost a
so-called “flight” of apprehensive, fearful ideas, loosely connected,
incoherently expressed, and dealing chiefly with his work and his
children. Judgment was imperfect; the height of the room was estimated
as 24 feet, but the height and weight of persons were estimated with
fair accuracy, and also the length of small objects, whose lengths were
doubtless remembered rather than estimated. The estimate of time
elapsing during a medical examination was accurate, but the estimate of
longer durations involving over-night memories was hopelessly imperfect.
Emotionally, there was a dulling of sensibility, an appearance of
suspicion and apprehensiveness; the patient fancied himself to be in a
hopeless condition as a result of syphilis, but at the same time
accompanied his statement of his hopelessness with laughter. A sample of
his hypochondriacal ideas: “I am all gone; I am good for nothing; I am
all gone now; I can’t drink now; can’t write or talk at all; worse than
when you saw me first; nothing in my inside; all wrong through me again;
I aint got no swallow now; I can’t die even; my heart aint much good; I
can’t hear it beat; I don’t think it flutters; no life in these hands;
they are all cold and dead” (pointing to his arms and moving them
about). During such a portrayal the patient laughed in a silly way.

During the second hospital stay, Morrill was at first restless,
sleepless, profane, imperfectly oriented for time, possibly for place,
and also for the attendants. A few weeks later he became stuporous and
confused, and his feebleness and physical exhaustion were finally ended
by death, March 21, 1906. Death was preceded by a semi-comatose
condition; a left otitis media had developed.

At the =autopsy=, it appeared that death was due to an early
bronchopneumonia associated with acute splenitis and doubtless related
to the otitis media of the left side. The body at large showed, aside
from these acute lesions, a few chronic lesions, including slight scars
of the left apex, and chronic adhesive pleuritis, chronic diffuse
nephritis, and aortic and coronary syphilis. The aorta showed slight
linear and nodular markings, with a single small dark ulcer in the upper
thoracic region, but the aorta did not show the characteristic scarring
which syphilitic aortas often show. The femoral marrow was of a dark red
chocolate color. The thyroid appeared to be smaller than normal. A
slight sacral decubitus had developed.

The description of the head (E.E.S.) is given in full on account of the
encephalitic lesions shown. These encephalitic lesions may be summed up
as follows:

    Local cerebral =atrophy= and =sclerosis= of the frontal, orbital,
    and central regions, especially of the left operculum and left
    supramarginal gyrus.

    Extension of sclerosis to hippocampal gyri with effacement of
    substantia reticularis alba.

    Slight chronic internal =hydrocephalus=.

    Granular =ependymitis= (especially of floor of 4th ventricle).

    Compensatory edema of frontal and central pia mater.

    Cerebellar sclerosis (culmen monticuli, lobus culminis, lobus
    cacuminis).

    Spinal sclerosis (grossly evident in the posterior columns of the
    upper thoracic region and of the lumbar enlargement).

The details are as follows:

    Head:—Bald on top. Hair =gray=. Scalp normal. Calvarium thin, deeply
    excavated by arachnoidal villi to right of vertex. Diploë absent.
    Dura closely adherent in bregmatic region. Dura of usual thickness.
    Sinuses contain cruor clot. Arachnoidal villi slight. Pia mater hazy
    and over sulcal veins porcelain white over all of vertex except
    occipital poles and over flanks (notably left). Thickened also
    around circle of Willis, over culmen monticuli and in posterior
    cerebellar notch. Edema of pia corresponding to atrophy of frontal
    and central regions. Cerebral atrophy most marked in orbital
    surfaces of both frontal lobes, in left area of Broca, and in left
    supramarginal region. The ascending branch and the ascending ramus
    of the posterior limb of the left Sylvian fossæ both readily admit
    the thumb by reason of atrophy of adjacent substance. Induration
    corresponds closely with atrophy, but is not more marked about the
    left Sylvian fossa. There is sclerosis of both hippocampal gyri,
    with loss of the substantia reticularis alba. The culmen monticuli
    and lobus culminis are firmer than the clival regions, and the lobus
    cacuminis is again slightly firmer than the clival region.
    Cerebellum a little softer than usual. Pia strips with usual
    readiness from all regions. The subpial region of the frontal lobes
    is a trifle grayer than that of the rest of cerebrum. Ventricles
    slightly dilated. Surfaces evenly sanded. Floor of fourth ventricle
    shows numerous coarse, closely set granules. Brain wt. 1200 grms.
    Cord shows a slight increase of consistence over one or two upper
    thoracic segments and in lumbar enlargement corresponding with a
    slight graying out of posterior columns. In places there is a
    suggestion of graying out also in lateral columns. A few calcified
    plaques in posterior lumbar pia.

Analysis of these details shows a number of lesions that characterize
paretic neurosyphilis (among others, granular ependymitis, frontal
atrophy, chronic leptomeningitis), but the lesions are more than merely
frontal, extending as they do back as far as the postcentral regions on
both sides, and even as far as the left supramarginal gyrus. The
cerebellar involvement although frequent, can hardly be said to be
characteristic in paretic neurosyphilis. The spinal involvement is
characteristic of a case which is probably to be regarded as one of
taboparesis; that is, of paretic neurosyphilis following a number of
years after the establishment of tabetic neurosyphilis. The aorta is
almost constantly affected by sclerosis in paretic neurosyphilis. The
absence of diploë in the skull is not infrequent and the adherent dura
mater is often found.

Microscopically, the tissues showed the characteristic lesions of
PARETIC NEUROSYPHILIS; nerve cell destruction, fibrillar and cellular
gliosis, lymphocytic and plasma cell deposits about the small vessels.

    1. What are the clinical evidences of syphilis outside the nervous
      system? The brownish branny eruptions of the skin, the depressed
      scars and the scaly eruption on right heel and sole are very
      suggestive of syphilis. Such clinical evidences of syphilis are
      very important in systematic examination. Although the laboratory
      tests are of the utmost assistance in the diagnosis of syphilis,
      the clinical signs should not be neglected, and no physician
      should rest satisfied with laboratory signs alone. X-ray diagnosis
      of bone conditions sometimes succeeds when all other methods have
      failed.


  =GUMMA of cerebral cortex verified by operation; death.=


=Case 27.= The presenting picture in the case of David Tannenbaum was
that of deep dementia, in which condition the patient was brought to the
hospital. There was a meagre history to the effect that about four
months before admission, he had lost his job in a hotel through lack of
further work. We heard that at this time he had begun to suffer with
excruciating pains in the head; at first, worse at night, later, worse
by day. It appeared that this pain, though it came and went, was chiefly
localized on the left side of the head. For a fortnight, Tannenbaum had
been dragging his legs, until finally he had become unable to walk at
all.

_Pari passu_ with these developments, Tannenbaum had become mentally
confused and irritable, and his memory had become untrustworthy. For
several days before admission, an appearance of marked dementia was
presented, with slow incoherent, or at all events, irrelevant words, and
a complete disorientation for person. However, his vision had become so
poor that it would have been hard for him to have recognized any one.

It appeared that the family history was entirely negative; that the
patient was without education but had been physically very strong, and
had been fairly successful at first in the junk business, and later in
the clothing business; but latterly he had been less fortunate in the
clothing business, and finally had to resort to work as a laborer around
a hotel.

His wife had had eleven pregnancies with but one miscarriage.
Nevertheless, out of the eleven pregnancies, there were now but four
living children.

=Physically=, Tannenbaum was a rather small man; he was flabby and
looked as if he had recently lost weight. The skin showed areas of
pigmentation on the face and sides of the neck, and some dark
copper-colored circular areas, marble-size, in the neck (syphilitic?).
There was a slight radial arteriosclerosis. The heart was slightly
enlarged with distant and indistinct sounds. There was a small
pedunculated growth on the right side of the abdomen.

The pupils failed to react to flash-light but they reacted to sunlight.
They both were slightly irregular but were equal in size, and reacted in
accommodation. There was apparently almost complete blindness and
extreme deafness. Arm-jerks and knee-jerks were absent; there was an
occasional slight response of the left ankle-jerk, but the right
ankle-jerk was absent; the left abdominal reflex was very feeble; the
right absent; the cremasteric reflexes were absent, but there were no
other abnormalities in the systematic examination. Hand grips weak; gait
awkward, with right leg held somewhat flaccidly.

It was significant that percussion over the left frontal and parietal
regions was able to elicit great pain. Either through the patient’s
deafness or through sensory aphasia, spoken language was not understood.
The serum W. R. was positive, the fluid W. R. negative.

=Diagnosis=: The clinical symptoms seem clearly to indicate syphilis.
The local skull tenderness and impairment of vision might well suggest
intracranial pressure. Uniting these suggestions, we might automatically
arrive at a diagnosis of cerebral gumma. We have learned to be rather
cautious of making a diagnosis of gumma of the brain through its mere
rarity.

Decompression was suggested and executed. A deep growth resembling a
GUMMA, in the view of the surgeon, was discovered. No attempt could be
made to remove it. The patient died without recovering consciousness.

    1. What is the significance of the negative fluid W. R. in this case
      of cerebral gumma? The W. R. producing substances not infrequently
      fail to appear in the spinal fluid from a gumma of the brain. The
      serum W. R. was positive in this case, but even the serum W. R.
      may be negative in cases of gumma, both of the brain and of the
      body at large. It must be remembered that the serum W. R. may be
      negative in paretic neurosyphilis (general paresis); the serum W.
      R. is even more apt to be negative in cases of gumma.

[Illustration:

  Gummatous meningitis. Compression of hemisphere. Tissue destruction of
    underlying cortex.
]

    2. Is operative procedure to be advised in cerebral gumma? There are
      cases in which the acute and threatening symptoms of heightened
      intracranial pressure require operative treatment simply because
      the therapeutist cannot wait for the effect of antisyphilitic
      treatment. Moreover, antisyphilitic treatment of cerebral gumma is
      not always as successful as that of most syphilitic lesions.

    3. Could the intracranial pressure be caused by other syphilitic
      lesions than gumma? A heavy meningitis may cause symptoms such as
      produced by an intracranial tumor. In such a case one will usually
      find evidences of inflammation in the spinal fluid. Cysts caused
      by syphilitic lesions may also produce identical symptoms.

    4. What is the significance of cranial tenderness? Where
      sensitiveness to cranial percussion is not due to a scalp lesion
      it is very suggestive of a tumor underlying this point. A
      gummatous lesion of the cranium itself, may occur without causing
      pain or increased sensitiveness.


  =CRANIAL NEUROSYPHILIS (focal syphilitic extraocular palsy) without
  mental symptoms.=


=Case 28.= A chef, Paolo Marini, 28 years of age, reported that on
awaking one morning, everything appeared double to him and that his
right eyelid had begun to drop. In the following month Marini had begun
to feel weak and to have difficulty in swallowing, as well as at times
difficulty in breathing. The diplopia was found to develop when Marini
looked to the right. Mentally, the patient was in all respects normal,
and no other physical signs were found except the diplopia and ptosis
above mentioned. The W. serum test was positive, but the tests of the
spinal fluid were negative.

=Diagnosis=: “CEREBRAL SYPHILIS.”

    1. What is the anatomical cause of this condition? It is thought to
      be due in a number of cases to a small diffuse gummatous lesion at
      the _basis cerebri_. In the case of Marini this lesion appears to
      have been a little more extensive and to have interfered with the
      tenth and twelfth nerves also.

    2. Why is the spinal fluid negative in such a case as that of
      Marini? Head and Fearnsides believe that intracerebral lues is
      characterized by a negative spinal fluid, under which circumstance
      one has always to consider the possibility of brain tumor or
      migraine in addition to the suspicion of syphilis.

    3. What other causes besides syphilis should one consider for the
      sudden diplopia? Brain tumor, multiple sclerosis, cerebral
      arteriosclerosis, tuberculous meningitis, trauma and migrainous
      ophthalmoplegia, are not infrequently at the bottom of this
      condition. Cases also occur in which the etiology remains obscure,
      even at autopsy.

Under antisyphilitic treatment, Marini slowly improved.


  =The SIX TESTS in TABETIC NEUROSYPHILIS (“tabes dorsalis”) may run
  milder than in paretic neurosyphilis (“general paresis”) and
  characteristically run somewhat like those of diffuse
  (meningovascular) neurosyphilis; in particular, the fluid Wassermann
  Reaction and the gold sol reaction are apt to run milder. The
  clinical course of tabes dorsalis is protracted and the prognosis as
  to life is good.=


=Case 29.= Mario Sanzi, 55 years of age, had been having what he called
rheumatism since his 43d year. This rheumatism affected only the hips
and legs, had at times been very severe, and for two years past had been
almost constant. Before that time, pains had come at intervals, lasted a
variable period, and suddenly disappeared. They were of knife-thrust
character, and could probably be called “lancinating.” In a given
attack, these pains would come at intervals of seconds or more. There
was also a certain unsteadiness in locomotion and inability to control
the vesical sphincter.

=Physically=, the patient was entirely normal so far as could be made
out except =neurologically=. Argyll-Robertson pupils, absence of
knee-jerks, and ankle-jerks, Romberg sign, and characteristic gait, left
no cause for doubting the diagnosis of TABES DORSALIS. The blood and
spinal fluid both proved positive to the W. R., though the W. R. in the
fluid gave a negative reaction with 0.1 cm. and became positive with 0.3
cm. or more. The globulin was somewhat increased though less markedly so
than in paresis. The gold sol reaction was “syphilitic” but weak. It is
to be noted that the disease had run a 12–years’ course before a doctor
had been consulted. The primary infection occurred at 32 years, namely,
11 years before the symptoms began. At the time of his primary
infection, Sanzi had received several years of treatment, chiefly in the
form of mercury by mouth.

    1. What is the value of mercurial treatment of syphilis in the
      prevention of tabetic or other forms of neurosyphilis? “Fournier
      strove for many years to convince the medical profession that a
      syphilitic patient should be treated for at least two years after
      his infection, whether the syphilis seemed latent or patent. The
      method of treating only the symptoms he characterized as the
      opportunist method; treatment in the absence of definite symptoms
      the preventive method, as preventing the later manifestations.
      That prolonged treatment does prevent is shown by Fournier’s
      figures analyzing 2396 cases presenting tertiary signs. These he
      divides into three groups: Group I, comprising 1878 cases, or 78
      per cent of the whole number, having no treatment or inadequate
      treatment—that is mercury for less than one year; Group 2,
      comprising 455 cases, or 19 per cent, having moderate
      treatment—that is, mercury for one to three years; and Group 3,
      comprising the remaining 19 cases which represent only 3 per cent
      of the whole number, having treatment for more than three
      years.”[7]

      In the light of what we now know concerning latent neurosyphilis,
      it would seem well for patients to be followed from time to time
      with the W. R. on blood and spinal fluid after the supposed
      completion of the treatment of primary and secondary syphilis. The
      examination of the spinal fluid is not superfluous, as our
      experience with the so-called _paresis sine paresi_ abundantly
      shows. At the present day it is not good practice to assure a
      patient that he is cured after two years of ordinary mercurial
      treatment without resort to frequent spinal fluid tests, even
      though the serum W. R. be negative.


  =TABETIC NEUROSYPHILIS (“tabes dorsalis”) is often quite ATYPICAL
  clinically and may even show no single symptom warranting the old
  clinical name “locomotor ataxia.”=


=Case 30.= Stephen Green is a case of TABES DORSALIS with active
knee-jerks and without locomotor or muscle-sense disorder. When observed
at the age of 45, it appeared that there were but two complaints: lack
of control of the vesical sphincter and shooting pains in the legs. It
appeared that the urinary disorder dated back ten years, when there had
been difficulty in passing the urine. Sounds had been passed at the
time; occasionally there had been incontinence during after years,
ascribed by Mr. Green to the passing of the sound. However, the
physician at that time stated that the incontinence was a symptom of
tabes dorsalis. The incontinence had recently become worse, especially
marked at night, though also occurring in the day; much worse during
excitement, and very much worse after taking alcoholic drinks. Besides
incontinence, there is also difficulty at times in passing the urine, as
well as dysuria.

As for the pains in the legs, they had been first noticed some three or
four years ago and considered to be mild rheumatic effects. Now,
however, they have grown progressively worse and have been the effective
cause of giving up business. The pains are sharp, darting, pinching, and
burning, and last, say, about a second with an interval of about the
same length. The attack will continue sometimes for many hours.

There is a strabismus of the left eye, ascribed by the patient to an
accident with an umbrella (there had been operation without relief). The
pupils showed the Argyll-Robertson effect and were markedly irregular.
Despite the divergent strabismus with diplopia, the eye movements were
well performed although not in parallel axes. Ankle-jerks could not be
obtained even on reinforcement, but the knee-jerks were lively, and the
other deep and skin reflexes proved normal. The blood and spinal fluid
tests were characteristic of tabes dorsalis.

It appears that the syphilis was acquired by this patient 15 years
before; that is, 5 years before neurological symptoms began. Three
courses of treatment had been taken at a well-known watering-place, and
mercury pills had been taken for two years by mouth. The patient is
married; has no children; there have been no pregnancies.

    1. What causes may be assigned for the absence of children in the
      family of a tabetic? There may be lesions of the genital apparatus
      (orchitis, or more specialized toxic lesions). But impotence such
      as characterized the present case must also be taken into account.

    2. What is the therapy for tabetic pains? Pyramidon is nowadays much
      in favor; morphine may be used; some authors recommend that the
      patients be instructed to chloroform or etherize themselves
      slightly for relief of the pain. Surgery of the nerve roots may be
      resorted to in extreme cases. Intraspinous therapy, suggested by
      various authors, seems to exert beneficial effect in many cases.

    3. Is the lack of control of the vesical sphincter an unusual
      initial symptom? On the contrary, the more careful the clinical
      observation, according to some observers, the more likely is the
      examiner to find that vesical symptoms were the earliest or among
      the earliest complaints of the patient. Baldwin Lucke found
      sphincter disturbances to be initial in 8¼% of his long Blockley
      series. He found sphincter disturbance to occur in some stage of
      the disease in 67.6%, being exceeded in frequency only by
      staggering gait (87.2%) and lancinating pain (71.6%). According to
      Lucke, the most frequent _initial_ symptom is lancinating pain in
      the lower extremity, which, it will be noticed, occurred also in
      our case of Stephen Green as an initial symptom along with vesical
      disturbance. Lucke’s figures show that paresthesia of the lower
      extremities (17.6%) and weakness of the extremities (16.4%) are
      the next initial symptoms in frequency.

    4. Could the early treatment in the case of Stephen Green be
      considered as adequate? No better answer can be given to this
      question than by quoting from Dr. Joseph Collins,[8] who probably
      has done more than any other one man in this country in insisting
      on the need of proper treatment of syphilis. As to the adequate
      treatment of syphilis he says:

      “It consists in the proper use of salvarsan and mercury begun at
      the earliest possible moment after infection and kept up till all
      biochemical evidence of the disease has ceased, while the
      metabolism of the individual is maintained as nearly normal as
      possible. But the physician does not do his whole duty when he has
      accomplished this. He must solicitously watch the individual to
      see that no evidence reappears for months and even years after the
      apparent cure. As an index of such reappearance the Wassermann
      test of the blood serum and of the cerebrospinal fluid is the
      safest guide.

      “Until there is a definite unanimity of belief among physicians as
      to when the treatment of syphilis shall be begun, and some concert
      of action as to what constitutes the adequate treatment of
      syphilis, we cannot hope to make any considerable progress in the
      prevention of syphilis of the nervous system, save by educating
      the individual toward infection.”


  =TABETIC NEUROSYPHILIS may produce symptoms chiefly if not entirely
  in the region supplied by the CERVICAL plexus (“cervical tabes”).=


=Case 31.= Paul Halleck, 35, was a salesman who had begun to find it
hard to carry his sample case, since he was unable to tell whether or
not he had it in his hand. There was not only an anesthesia of the
hands, but they felt numb and there was often a tingling sensation. Of
late it had become hard for Halleck to dress himself or to write, and
these symptoms had been slowly growing worse. There was no other
complaint. There was, however, a history of a chancre about 7½ years
before, which had been followed by a rash and a sore throat. There had
been treatment with mercury and potassium iodid alternating for a period
of two years.

=Physically=, there was no evidence of disease except =neurologically=.
The pupils were unequal (the right larger than the left) and reacted
slowly to accommodation and not at all to light. A marked ataxia of the
hands was shown in coat-buttoning. The finger-to-nose test showed a
marked dysmetria. Arm-jerks as well as knee- and ankle-jerks were
absent. There was a slight swaying in the Romberg position but no true
Romberg sign. There was no difficulty in locomotion. Both blood and
spinal fluid proved positive to the W. R.; globulin and albumin were
increased. The gold sol reaction was syphilitic, and there were 85 cells
per cmm.

This case is probably not a pure example of CERVICAL TABES, since the
knee-jerks are also absent, and we may suppose a degree of lumbar spinal
cord changes in addition to the cervical changes. It well illustrates,
however, that the tabetic involvement of the cord may be quite
generalized and that it may strike high as well as low.


  =ERB’S SYPHILITIC SPASTIC PARAPLEGIA.=


=Case 32.= Margaret Neal, a maid-of-all-work, 36 years of age, was
committed to a home for inebriates on account of her excessive
alcoholism, but she was shortly transferred to the Psychopathic Hospital
on account of difficulty with locomotion. We found a very marked
spasticity in walking, with a characteristic scissors gait. The pupils
were somewhat irregular, and although both reacted to light, the left
reacted far more slowly than the right and the reaction failed to hold
well. The arm reflexes were very active, and the knee-jerks and the
ankle-jerks were particularly exaggerated. There was a double Babinski
reaction, as well as Oppenheim and Gordon reflexes and a bilateral ankle
clonus. There seemed to be tenderness over the nerve trunks in the back
of the leg, below the knee. There was no evidence of incoördination, no
Rombergism, no disturbance of sensation, no disorder of the special
senses, and not even a tremor of the tongue or hands.

=Mentally=, the patient was entirely negative.

=Diagnosis=: Symptomatically, it is entirely clear that the patient was
suffering from SPASTIC PARAPLEGIA. One would have to consider besides
spinal syphilis, also amyotrophic lateral sclerosis, syringomyelia, and
spinal cord tumor. However, there appeared to be no definite wasting of
muscles, and the fact that the sensations were intact seems to rule out
also syringomyelia. There was none of the characteristic pain associated
with a cord tumor. There was, in fact, a strong clinical premonition
that the case was one of spinal syphilis, simply because syphilis is the
most common cause of spastic paraplegia in the adult. The pupillary
anomalies were also highly suggestive.

The serum W. R. proved to be weakly positive, as was also the gold sol
reaction in the zones characteristic of syphilis. The spinal fluid
examination yielded 14 cells per cmm. There was a positive globulin test
and a moderate increase in albumin. The W. R. of the spinal fluid was
negative.

    1. Why was the spinal fluid W. R. negative in this case of spinal
      syphilis? The explanation of negative W. R.’s in spinal syphilis
      is not easy. Possibly, however, in the course of years the
      intensity of the process has been reduced and possibly the W. R.
      has been one of the first tests to disappear.

    2. How shall we explain the nerve trunk tenderness? We might
      consider this to be due possibly to an inflammation about the
      posterior roots. On the whole, partly on account of the situation
      of the pains below the knee, it seems probable that the nerve
      trunk tenderness of this case is the residuum of an alcoholic
      neuritis.

=Treatment=: Under injections of mercury salicylate, there was a rapid
improvement. In fact, in the course of several months, the patient
regained an ability to walk long distances. There still remains a
certain spasticity, but the abnormal spinal reflexes above mentioned are
no longer present.


  =SYPHILITIC MUSCULAR ATROPHY, probably due either to spinal
  parenchymal lesions, or to root neuritis, or to both.=


=Case 33.= Joseph Graham, now 50 years of age, seemed no longer to be
able to do good work as a teamster. His arms had become weak and the
muscles had become tremulous and apparently wasted. There was also pain
in the left leg and hip. It appears that this latter symptom had been
thought to be rheumatism, having begun about 8 years before with a
sudden sharp shooting pain in the left hip, about the region of the
sciatic notch. Graham had rubbed the hip with liniment, but without
reducing the so-called rheumatism. The trembling of the hands had begun
some years later, but no wasting had been noticed except during the past
year. The pain in the leg had suddenly become so severe that a month
before medical observation he had quit work. The question immediately
arose whether Graham was not suffering from some familial form of
muscular atrophy; but according to his representations, there was
nothing of the sort in the family.

=Physically=, there was little to note. =Neurologically=, there was
more. The pupils were somewhat irregular in outline, and the right was
larger than the left. The left pupil failed to react to light, and the
right pupil reacted very slowly and with but a slight excursion. There
was no tremor of the tongue and no evidence of facial palsy nor was
there smoothing of the nasolabial folds. It was somewhat remarkable,
that in the absence of these signs, there was a marked speech defect.
The atrophy of arms, forearms, and hands was well marked, especially the
atrophy of the thenar and hypothenar eminences of the right hand. The
extended hands, especially the right, showed a marked coarse tremor.
Fibrillation was found in the muscles of the hands, forearms, arms, and
pectoral muscles. There was no dysmetria, and the diadochokinesia was
normal. Strength was diminished (dynamometer right hand, 32 kg., left 31
kg.). There was little or no atrophy of the legs, although the left
thigh was perhaps slightly atrophic and the gluteal muscles of the left
side were somewhat flabby. The patellar and Achilles reflexes were
absent on both sides. There was a slight swaying in Romberg position.
Gait was normal. There was a marked tenderness on the left side of the
sciatic notch, as well as over the entire distribution of both external
and internal popliteal nerves. This area of skin was also hyperesthetic.
There were no other neurological signs on systematic examination.

=Diagnosis=: The sensory disorder, the speech defect, and the pupillary
abnormalities seem to render the diagnosis of progressive muscular
atrophy doubtful. Nor was there any dissociation of sensations to
suggest a syringomyelia. Under such circumstances, one must fall back
upon the question of syphilis. Both blood and spinal fluid proved to be
positive to the W. R.; the globulin was increased and the albumin
markedly so; there were 61 cells per cmm., and the gold sol reaction
read 4 4 4 4 3 2 1 0 0 0.

    1. Is there a relation of SYPHILITIC MUSCULAR ATROPHY to amyotrophic
      lateral sclerosis? Spiller, some years since, claimed such a
      relation, and it would seem with some justice.

    2. How shall the present case be classified? There is evidence of
      root pains (left hip). We may naturally suppose that these root
      pains are reasonably good clinical evidence of a meningitic
      lesion, of which the spinal fluid clinically gave a confirmation.
      The fibrillation in this case somewhat suggests, however, a
      central origin for the muscular atrophy. Accordingly, it would be
      difficult to definitely classify the present case as either one of
      meningovascular syphilis or one of central syphilis. It will be
      remembered that Head and Fearnsides classify muscular atrophy
      under both these headings.


  =The period of SECONDARY SYPHILIS is frequently (over a third of all
  cases?) MARKED BY approved signs of NEUROSYPHILIS precisely like
  those of full-blown paretic or diffuse (meningovascular non-paretic)
  neurosyphilis. These signs occur sometimes in association with
  severe clinical symptoms, sometimes without clinical symptoms.=


=Case 34.= John Bennett, 28, was brought to the Psychopathic Hospital
much confused. His brother, who came with him, said that he had been a
very heavy drinker but had given up drinking about four months before.
He had recently had a cold but was otherwise in good health up to the
night before admission. On this night, Bennett had become suddenly
excited and went into his mother’s room, at the common home, and began
to curse her. However, he was put to bed safely, but on the next morning
began to moan continuously. After some hours of moaning, he was brought
to the hospital. Here he remained difficult to manage, being irritable,
noisy, and resistive. Questions he either would not or could not answer,
and there was even no evidence that he understood questions. However,
within a few hours, it was clear that he was slowly coming out of the
confused state. On the following day, it was possible even to rouse him
and get his name. The confusion gradually cleared still further and, by
the end of three days, he had become mentally absolutely well so far as
could be determined.

He then informed us that he had had a chancre about five or six months
before, followed by a secondary skin eruption; that he had received four
injections of salvarsan (the last, a month before admission) and three
injections of mercury. At about the time of the last injection of
salvarsan, he had developed headache with pain and slight stiffness in
the back of his neck; and a fortnight later, he began to have dizzy
spells, followed during the last week by difficulty in hearing. There
was amnesia for everything that happened after his spell of sudden
excitement on the evening before admission, and this amnesia was never
lifted for the four days that followed.

=Physically=, Bennett was very well built and muscular. Nor were there
any evidences of disease outside the nervous system. There was some
slight stiffness of the neck and slight pain on movement of the head,
which probably ought to be attributed to meningitis. The =neurological
examination= showed tendon reflexes all normal, and normal sensations.
There were, in fact, no neurological signs except that both pupils were
dilated; the left was larger than the right. Both pupils reacted to
light but reacted very poorly. They reacted much better to
accommodation.

The W. R. proved to be positive, as might well be expected in a man
whose infection had taken place less than six months before. The
globulin and albumin of the cerebrospinal fluid were in great excess, of
a degree which we clinically express by ++++. The W. R. of the fluid
also was strongly positive down to 0.1 of a cmm. The gold sol reaction
was the “paretic” type, and there were 228 cells per cmm.

    1. How early may clinical evidence of neurosyphilis set in after
      infection? Craig found one case of “brain syphilis” occurring one
      month after infection. Frye claims a case of tabes dorsalis
      developing six weeks after infection. Craig states that he has had
      three cases of brain syphilis occurring within six months, and six
      within a year of infection.

    2. What effect did the salvarsan injections have in causing or
      preventing the symptoms in this case? Nonne sums up the
      neurorecidive question as follows: Since the introduction of
      salvarsan therapy for neurosyphilis, paralyses of various cranial
      nerves are seen more frequently. This higher frequency is in part
      only apparent since more attention has been paid of late to
      auditory and labyrinthine disorders. On the whole, however, it
      must be considered that salvarsan does mobilize spirochete foci
      which without salvarsan therapy would perhaps have remained
      latent. Probably we are here dealing in some instances with fresh
      infections of neurosyphilis, in other cases with a Herxheimer
      reaction. Ehrlich believed that these latent foci occur
      particularly in places with stagnant blood current; as, for
      instance, in the narrow bony canals. This hypothesis, sufficient
      in some instances, is less satisfactory for cases of peripheral
      neuritis, for example.

    3. What treatment is indicated? Intensive antisyphilitic treatment
      is strongly indicated. Whatever may be the truth concerning the
      production of neuro-recurrences (“neurorecidives”) it is certain
      that the symptoms usually vanish with a continuance of salvarsan
      therapy. The important point is to give efficient treatment, and
      in a case like Bennett’s improvement is fairly certain unless some
      serious insult occurs before the remedial efforts have been given
      time. It is still an open question whether intraspinous treatment
      is more efficient in such cases than intensive intravenous
      injections of salvarsan. In Bennett’s case diarsenol was injected
      intravenously twice a week in 0.6 gm. doses, reënforced with
      intramuscular injections of mercury salicylate and potassium iodid
      by mouth. Under this treatment improvement began slowly and in a
      few months he was symptomatically well and after three months his
      tests were practically negative.


  =JUVENILE PARETIC NEUROSYPHILIS (“juvenile paresis”) with OPTIC
  ATROPHY.=


=Case 35.= Mary Coughlin, a blind girl of 16 years, was brought to the
hospital in a state of great excitement, laughing and crying
alternately. The neurologist is entitled to think of blindness, and
particularly of the optic atrophy which Mary showed, as probably due to
syphilis. However, there was no history of syphilis in the father, who
died in an accident at the age of 40, or the mother, who died at 45, of
heart trouble. An elder sister was married and well; two younger sisters
were living and well. The fifth sibling, a boy, had died in infancy.
There had been no miscarriages. In fact, the only point in favor of
syphilis was the somewhat far-fetched point that the younger brother of
the patient had died in infancy.

The patient’s history was rather suggestive of some other diagnosis. Her
birth had been normal, she walked and talked at 13 months, was at school
from six to twelve, reaching the seventh grade, and was considered
bright. At three years of age, she had been run down by a car and
dragged under the fender for a considerable distance. Her head was hurt
but the patient did not lose consciousness in the accident. Fainting
spells began at 11, in which spells the patient would lose consciousness
for a minute or two. About this time, the patient’s eyesight had begun
to fail, and for some four years she had been entirely blind. Headaches
had come on of late.

The Coughlin case, except for the above-mentioned suspicion of
syphilitic optic atrophy, might be regarded as an unusual example of a
post-traumatic disease.

We found her to be fairly well developed and nourished; there was a
deformity of the lower half of the sternum and of the third and fourth
ribs on the right side. There were no other physical phenomena found
upon systematic examination. The left pupil still reacted to light; the
right failed to react, but this lack of reaction could not be regarded
as of Argyll-Robertson nature on account of the finding of optic atrophy
with the ophthalmoscope.

=Mentally=, it appeared that the patient’s retention of school knowledge
was poor, though her blindness for four years had doubtless given her
little opportunity to keep such information fresh. Rather strangely,
Mary gave utterance to many delusions: first, expecting to receive her
sight by an operation on the head; second, to write a book of her
doings; third, to buy a house for the children; fourth, would pay $3000
for the house, earning the money by working at a tailor’s or as a
trained nurse; fifth, to go on the stage to earn money by dancing;
sixth, will have lots of money.

One of Mary’s characteristic statements is as follows: “Won’t it be
lovely when I can see Dr. H.’s face in heaven or some other lovely
place? Dr. H. was a grand doctor to me, and when we get together again
we are going to Tremont Temple and keep us together. I am going to do
some dancing and play the piano. I am going to graduate at the high
school and go to Trinity College in Washington, and I hope I shall be a
faithful keeper of mother’s tomb.”

The patient was at times euphoric and expansive.

At this stage, what with optic atrophy, euphoria, and expansive
delusions, we should perhaps be entitled, had Mary been an adult, to
offer the diagnosis GENERAL PARESIS. In fact, on the whole, any other
than a syphilitic cause for the optic atrophy was exceedingly doubtful.
Brain tumor of a nature to produce optic atrophy might very improbably
last so long as five years. There was no evidence of any intoxication at
the time when the blindness occurred.

The W. R. was positive in the blood and spinal fluid; there was a
positive globulin test, and an excess albumin as well as 15 cells per
cmm.

    1. What is the significance of Mary’s trauma at three years? So far
      as we are aware, none.

    2. What light could be thrown by a W. R. study of the family? In
      some instances, much light is thrown; in the present case all
      three living sisters of the patient have been examined and their
      serum W. R.’s have been found negative.

    3. What is the prognosis of juvenile general paresis? Death within a
      few years, as in general paresis in adults. The patients live
      rarely more than four or five years after the onset of symptoms.
      Mary Coughlin died a year and a half after the above examination,
      namely, in her eighteenth year, some seven years after the onset
      of symptoms.

    4. What can be said of treatment? A few favorable results have been
      reported after intraspinous therapy (Swift-Ellis). Too little work
      has been done with systematic treatment of juvenile neurosyphilis,
      both paretic and non-paretic, to permit important conclusions at
      this time.

    5. How can we explain the infection of this sibling whereas the
      others, both younger and older, escaped? It would seem that we
      would have to discard the hypothesis of a congenital infection and
      consider that it was acquired accidentally during the lifetime of
      the patient. Considering the prevalence of syphilis it is rather
      to be wondered that more such cases of “innocent” infection do not
      occur in children. We may recall how many instances of juvenile
      gonorrhea occur. In a case as this where the symptoms calling
      attention to syphilis necessarily occur so long after the original
      infection it is practically impossible to trace the origin of the
      infection.


  =The diagnosis of JUVENILE PARESIS is often easy.=


=Case 36.= Theresa Mullen, an under-sized girl of 12 years, presented a
remarkable appearance due to congenital amputations of the fingers and
toes. She lay in bed, drivelling and making unintelligible cries. It
appeared that the patient weighed about 12 pounds at birth and was very
fat; that she had been fed on condensed milk, had survived cholera
infantum, whooping cough, and, as the parents said, “two kinds of
measles.”

Theresa had gone to school at 5 years, reaching the third grade at the
age of 9; but at this time, she began to lose ground and was put in a
class for backward children. Moreover, at about this time, the teachers
noticed spells of causeless laughter and meaningless twisting back and
forth. Theresa would also scream at night, looking about the room; once,
rising and crying, “Take him away, that black thing,” though no
appropriate object was present. There had been little or no complaint of
headache. Theresa had been deteriorating for some time, and for a year
past had been having increased difficulty in walking. For two months the
child had not spoken intelligible words; for the last week, she had been
incontinent.

The =diagnosis= was almost obvious from the manual and pedal deformities
taken in connection with the saddle-back deformity of the nose. It was
interesting in connection with the contentions of W. W. Graves, that the
scapulae were scaphoid in type.

Accordingly, the history given by the parents seemed consistent enough.
The parents were both 36 years of age, having married at 23. The first
pregnancy was a miscarriage at two months, of unknown cause. Theresa
came next; thirdly, came a miscarriage at three months; fourthly, a
girl, who is not strong or well physically, has suffered much from
headaches and sore throat, but is fairly bright. The fifth pregnancy
resulted in a boy, who is bright but of under-size. Three more
pregnancies resulted in miscarriage.

Taking into account the above-mentioned physical characteristics, the
personal history, and the family history of Theresa, the diagnosis could
hardly be in doubt even in the absence of a lack of pupillary reaction
to light on the right side, infantilism of genitalia, positive W. R.’s
of serum and spinal fluid, positive globulin, and excess albumin, 34
cells per cmm. and the paretic type of gold sol reaction which were
found.

The =prognosis= of this case appears to be rapid deterioration,
terminating in death within a few months. Now and again, however, some
such cases spontaneously improve. Such a case as that of Theresa Mullen
is always disheartening in itself but suggests the social value of
Wassermann tests in the other members of the family. The other children
of the Mullen family proved to be suffering also from syphilis, since
their blood sera all showed a positive W. R.

    1. What is the characteristic age of onset in JUVENILE PARESIS? An
      impression has prevailed in some quarters that the typical onset
      of juvenile paresis is in the adolescent years, and Clouston’s
      first case (1877) developed in a boy of 16. Thierry’s 58 cases,
      developing from the 8th to the 20th year, averaged 14 years of age
      at onset. Mott’s 22 cases from the 8th to the 23d year, averaged
      17 years at onset. According to Clouston, juvenile paresis
      develops most often at puberty (15 to 17 years). It is sometimes
      claimed that cases developing symptoms early live longer, and that
      juvenile cases developing symptoms after the 20th year run a short
      course. For a case developing in the 5th year, see John
      Friedreich, Case No. 77.

    2. What may be concluded from the physical signs (congenital
      amputations) present in this case before the development of mental
      symptoms? Some cases of juvenile paresis appear to show no
      physical signs whatever in childhood. While these amputations
      might be the accidental result of a difficult delivery, it is more
      probable that they are due to a syphilitic process.

[Illustration:

  Juvenile paresis—congenital amputation of digits. This case reached
    fourth grade in school before deterioration.
]


  =CONGENITAL SYPHILIS is apparently capable of producing simple
  FEEBLEMINDEDNESS (that is, a form of disease non-paretic,
  non-tabetic, without special tendency to progression, and without
  tendency to vascular insults).=


=Case 37.= Isaac Goldstein was a small boy of six years and seven
months, with a father known to be suffering from general paresis. The
child was very irritable and nervous and very difficult to manage, but
would hardly have been the subject of medical attention except in a
family study suggested by the paresis of the father.

The child had been born at term and had apparently undergone a normal
development. Physically, he showed no definite signs of congenital
syphilis. In fact, the physical examination was to all intents and
purposes negative. The W. R. of the serum, however, proved to be
positive. Mental tests showed that his mental age was that of a child of
a little over five years. Taking all things into account, it is probable
that he should be regarded, therefore, as somewhat retarded mentally.

    1. Is syphilis answerable for the mental retardation in this case?
      Provided that the family is free from feeblemindedness and mental
      disease, it would seem that the retardation of a congenital
      syphilitic should perhaps be regarded as syphilitic in origin. Of
      course, the institutions for the feebleminded have not shown
      exceedingly high percentages of syphilitic children in various W.
      R. surveys; still, the percentage of positive reactions in
      institutions for the feebleminded is clearly higher than the
      incidence of congenital syphilis shown in the population at large.
      Hence, we may conclude that syphilis is one of the etiological
      factors in the production of feeblemindedness. Dr. W. E. Fernald,
      of the Waverley School for the Feebleminded, has recently pointed
      out that the syphilitic cases belong rather in the lower grades
      (idiots and imbeciles) of feeblemindedness than in the higher
      (morons).

    2. Can we guess what the pathological anatomy and histology of the
      brain may be in such cases? The Waverley studies now in process
      seem to indicate that some cases have little or no gross
      alterations, but show a few slight traces of lymphocytic
      accumulations discovered upon extended search, and a certain
      tendency to the appearance of rod cells in various foci. But the
      whole matter is still _sub judice_. It is a question whether these
      traces of chronic inflammation are the residuals of a more active
      process or the beginnings of a process that is about to be more
      active.

    3. How characteristic is a positive W. R. in the serum of a child
      without physical stigmata of congenital syphilis? If we limit the
      term _stigmata_ to the major and more important signs, we must
      reply that it is not unusual to find positive W. R.’s in sera of
      physically normal-looking children. Except in family studies, such
      cases will often escape notice, either because there are no
      stigmata whatever, or because such stigmata as exist are of a
      minor nature and regarded as unimportant anomalies. Some of these
      cases occur in the clinics later in life as so-called _syphilis
      hereditaria tarda_. If one wishes to discover these cases with
      late development of symptoms before their full bloom, the most
      obvious method is to examine carefully the children of known
      syphilitics.

[Illustration:

  Scaphoid Scapulae.
]


  =JUVENILE TABETIC NEUROSYPHILIS (“juvenile tabes”); TREATMENT.=


=Case 38.= The point in presenting Archibald Sherry, a JUVENILE TABETIC
of 12 years on admission, is perhaps to exhibit pride in therapeutic
results.

There was little or no doubt of the diagnosis; in an adult, the
phenomenon would be called tabes dorsalis with a question of general
paresis. The right pupil was larger than the left and reacted neither to
light nor to distance. There was a slight tremor of the tongue and of
the outstretched hands. The knee-jerks and ankle-jerks could not be
obtained, nor could the periosteal reflexes in the legs. There was a
slight unsteadiness in the gait and in various finer movements, and a
slight ataxia of the legs. There was not a classical Romberg sign but
there was slight swaying in Romberg position. The teeth were
Hutchinsonian. For the rest, the physical examination was practically
negative.

The family history was of interest. On the paternal side there was
nervousness as well as alcoholism and degeneracy. The maternal
grandmother had cancer. Archibald’s father was immoral and alcoholic.
There was a girl four years older than Archibald, who, though nervous
and unstable, has shown no signs or symptoms of syphilis and does not
yield a W. R. in blood or spinal fluid.

Archibald himself was born at term, a large child, who, however, lost
weight rapidly, developing a marked skin eruption on head and back three
weeks after birth. This skin disease lasted for a month and a half and
then spontaneously disappeared. Archibald remained weak and sickly, not
walking until three years of age. However, he did well in school up to
the end of his 11th year, when he failed to keep up with the children.
He had been an amiable child and had gotten on well with his playmates.
Some time in his 10th year physical disability had begun; there was
numbness in the legs with weakness; at times, actual inability to walk.
The right pupil was noticed by the mother to have increased in size; the
eyelashes had turned white. There was pain over the left eye and a
feeling of weight on top of the head. Speech became difficult or even
confused.

Consistently enough, the W. R. both in blood and spinal fluid was
positive. Globulin and albumin were present in large amounts; there were
150 cells per cmm.

Granting that this be in some sense a case of juvenile tabes we may
raise a doubt whether the case is one of congenital syphilis. The W.
R.’s of the blood of both father and mother are negative. Syphilis is
denied by them. The nervous and unstable older sister failed to show
definite symptoms of syphilis or a positive W. R. There had been no
miscarriages or stillbirths. The question arises whether the
Hutchinsonian teeth do not indicate congenital syphilis. It appears,
however, that it is possible to develop Hutchinsonian teeth if syphilis
is acquired before the teeth are formed. We have no data as to how or
why this particular baby should have acquired syphilis, if he did so
acquire it, at the age of three weeks. On the whole, sceptics may doubt
our suggestion that the case is one of acquired juvenile tabes. Possibly
the question is academic so far as treatment is concerned.

=Prognosis=: The rarity of juvenile tabes is such that little can be
said as to prognosis. Three and a half years have passed since a few
injections of salvarsan were made. The pains above mentioned rapidly
disappeared, the gait became steadier, the attacks of confusion ceased,
and the speech improved. Unfortunately, on account of a lack of
coöperation on the part of Archibald’s mother, we have been unable to
continue treatment. However, we have from time to time followed the
patient in his home and he seems to have shown no falling back after the
initial improvement. It would be of great value could we know the
situation in the spinal fluid at the present time.

    1. Is there any explanation why paresis should occur in some
      juveniles and tabes in others? There is no available explanation
      for this difference nor any for the characteristic early optic
      atrophy of juvenile tabetics.




      Be frustrate, all ye stratagems of Hell,
      And, devilish machinations, come to nought!

                                  Paradise Regained, lines 180–181




       III. PUZZLES AND ERRORS IN THE DIAGNOSIS OF NEUROSYPHILIS


This part of the case collection, dealing with puzzles and errors, is
ushered in by six cases (39–44) drawn from a group of errors in
diagnosis made some years since at the Danvers Hospital. These six are
autopsied cases. Attention is called to the fact that modern methods of
diagnosis might have prevented the errors.


  =DIFFUSE NEUROSYPHILIS (“cerebrospinal syphilis”) versus PARETIC
  NEUROSYPHILIS (“general paresis”). Autopsy.=


=Case 39.= Caroline Davis, dead at 49 years, was a case of error in the
diagnosis of general paresis. Like Cases 40 to 44, Case 39 was
diagnosticated by the full Danvers staff as a case of general paresis;
however, it must be added, before the days of the W. R. and the modern
methods of systematic diagnosis. As will transpire in the sequel, there
is a large question whether Case 39 is not after all really a case of
neurosyphilis, possibly not of the paretic group. The details are as
follows:

Caroline Davis was a normal school girl till 15, apt in studies, mill
worker till marriage at 18; one child, dead (cause unknown). Habits
good. Moderate deafness set in in the forties and in 1901 patient became
completely deaf in three months’ time. In 1905 she became unable to take
care of her house and had a shock in which the right leg was affected.

On commitment patient showed good development and nutrition with slight
enlargement of capillaries of cheeks, redness and roughening of skin of
right ankle. Teeth absent. Slight radial and brachial arteriosclerosis.
Urine negative. Sluggish pupil reactions to light both directly and
consensually. Deafness absolute, bone conduction defective. Arm reflexes
brisk, knee-jerks equal, brisk. Bilateral Babinski reaction more marked
on the right side, tremor of tongue, Romberg’s sign, gait defective.
Speech stumbling, writing clear, without tremor.

Communicated by writing only. Consciousness normal, disorientation for
day of month, for place (misnames hospital) and for persons (recognizing
nurses, not patients).

Patient wrote many letters complaining of pain, headaches and especially
of pain in the abdomen and side. The patient was thought to show a
slight defect of memory, but her deafness rendered diagnosis difficult.
The patient died suddenly on May 23, 1908, shortly after supper, falling
backwards, and dying in five minutes with marked respiratory distress.

=Post Mortem Findings.= The =cause of death= was not clear. The heart’s
blood and cerebrospinal fluid were sterile. There was a small hemorrhage
in the anterior part of the right ventricle derived from a small artery
of the caudate nucleus. There was about 400 cc. of blood between the
dura mater and the pia mater. There was a slight sclerosis of the basal
and Sylvian arteries. The brain substance was uniformly softer than
normal.

It is possible that the hemorrhage had taken place some time before the
patient’s fall and that the brain substance had swollen in consequence.
Just before the fall she had a weeping spell.

The =anatomical diagnoses= were as follows:

Obesity, unequal pupils, fresh wound near left ear, edema of legs,
slight focal adhesive pleuritis, hypostatic congestion of lungs, chronic
endocarditis, chronic myocarditis, congestion of kidneys, congestion of
pancreas, subacute splenitis, chronic adhesive pelvic peritonitis,
hematoma and cystic condition of Fallopian tubes, =calvarium dense= and
thick, subdural hemorrhage, slight =chronic leptomeningitis=, general
=cerebral atrophy=, marked in tips of =frontal lobes=, old =cyst of
softening= between left corpora albicantia and optic chiasm, small
punctures of left ear drum, drums opaque, =chronic spinal
leptomeningitis=; brain weight, 1190 grams.

There were marked firm interadhesions between dura and pia throughout. A
lumbar puncture soon after admission in 1907 had shown:

                                            Per cent
                    Endothelial cells             10
                    Lymphocytes                   30
                    Plasma cells                   0
                    Phagocytes                     0
                    Polymorphonuclear cells       51
                    Unclassified                   9
                    Fibroblasts                    0
                    Cells in 100 fields          125

It will be noted that the lumbar puncture yielded no plasma cells and
yet showed 30% of lymphocytes. Alzheimer, in 1904, attempted to
distinguish the histology of the cerebral syphilitic from that of the
general paretic, maintaining that _lymphocytosis was the characteristic
feature of the ordinary neurosyphilitic_, _whereas plasma cells were
associated with the lymphocytes in the paretic_. This case showed
=lymphocytic= deposits. To be sure, they were decidedly subordinate in
the cerebral cortex, cerebellum, and basal ganglia, to the marked
evidences of nerve cell destruction, although there were perivascular
infiltrations about a few of the larger vessels in the white matter of
the cerebral cortex.

The spinal cord, however, showed a most severe infiltration, especially
in the gray matter, where the infiltration accompanied severe nerve cell
changes and arterial changes. The pia mater of the spinal cord was also
packed with mononuclear elements, among which, however, no plasma cells
could be found.

But although the inflammatory changes in the shape of lymphocytosis were
relatively more prominent in the spinal cord than in the cortex, yet the
cortex yielded evidence of an exceedingly marked destructive process.
Perhaps no layer of any of the areas of the cortex examined failed to
show some atrophic alteration. The upper layers of the cortex were
everywhere more severely diseased than the lower layers. Here we are
dealing with an instance of an active meningomyelitis and subcortical
encephalitis. It is, of course, probable that the W. R., had it been
performed, would have been positive in this case. On the basis of the
histology, we are inclined to regard the clinical picture in this case
as belonging among cases of NON-PARETIC DIFFUSE NEUROSYPHILIS.

This case, as also the next several, is especially instructive in
teaching the difficulty in differentiating paretic and non-paretic
neurosyphilis. Not only is this difficulty met in clinical diagnosis,
but in pathological diagnosis as well.

The histological diagnosis depends in large part on the work of the
Nissl-Alzheimer school, which has received great recognition. At the
present time, however, there is beginning to be considerable doubt as to
the entire validity of this teaching. At any rate there are many
borderline cases in which the differentiation is well nigh impossible.
In this case note chronic meningoencephalitis, with cortical
degeneration, in the absence of plasmocytosis.

From the clinical standpoint the intensity of the W. R., the character
of the gold sol reaction, and the result of therapy have added new
points in differentiation. Much more work controlled by autopsies is
still needed, however, to put us on sure ground in borderline cases.


  =VASCULAR NEUROSYPHILIS(?) versus PARETIC NEUROSYPHILIS (“general
  paresis”). Autopsy.=


=Case 40.= Case 40 like Case 41 was an error in the diagnosis of general
paresis which might be regarded as academic rather than practical. Both
were cases of arteriosclerotic brain disease with severe cerebellar
involvement. Case 40 had a spinal cord that was not quite normal. There
was a tabetiform lesion in the cervical spinal cord (not elsewhere),
together with a unilateral degeneration suggesting in some respects a
radicular origin. The most striking feature, however, of Case 40 as in
Case 41, was a lesion of the cerebellum. In Case 40 the dentate nuclei
were in large part destroyed by cysts of softening, although the
cerebellar cortex was fairly well preserved on both sides. The details
of Case 40 are as follows:

H. F., male, gear maker, born 1850.

=Heredity.= Maternal grandmother insane. Mother insane at 52, became
demented and lost use of limbs, died at 71. Aunt insane.

=Personal History.= Common school education. Capable workman till within
a few months. Early in life alcoholic. Drunk almost every week until
1899 or 1900. Irritable, nervous, selfish, loose in relations with
women. Venereal disease denied by wife. Married in 1883. Three frail
children. No miscarriages. Neuralgia in 1901 or 1902.

January, 1904, patient left carriage shop on account of mistakes in
work, became more pleasant, childish, fearful, talkative, did funny
things, later became vagrant, stole from fruit stores, smoked cigarettes
picked up in the street, and became restless and irritable.

Committed to Danvers, June 24, 1904, with slightly enlarged heart,
somewhat heightened blood pressure, and a slight sediment of epithelial
cells in urine.

Romberg’s sign was present, but there was little or no demonstrable
incoördination otherwise. Very slight tremor of fingers. Left knee-jerk
absent, right obtained on reinforcement. Achilles jerk absent. Triceps,
wrist and normal plantar reflexes present. Pupils react to
accommodation, but very slightly, if at all, to light. Sensations normal
except in legs. The legs show preservation of tactile and temperature
senses, but abolition of pain sense except over dorsum of foot.

Speech showed slurring of syllables and “brigrade” for “brigade.”
Disorientation for time, place and in part for persons. Admitted that
his work had been deficient but regarded himself as well. Emotionally
variable, crying at times and suddenly becoming jocular. Eloped July 3
and somehow reached his wife’s house in a neighboring city.

Euphoria persisted. The pupils continued Argyll-Robertson, and the
knee-jerks remained absent. Became oriented for place and partially as
to time (month and day of week correct).

During 1905 failure became rapid, with ataxia of legs, persistent
euphoria, and loss of weight.

Convulsions, regarded as general paretic, developed in 1906. Death
sudden, December 7, 1906.

=Post Mortem Findings.= The =cause of death= was streptococcus
septicemia, probably derived from a gangrenous bronchopneumonia or
related with a small thrombus of the right auricular appendix. There was
also an acute purulent otitis media, mastoiditis and sphenoidal
sinusitis, as well as extensive decubitus. From this decubitus or from
the intestinal tract may have been derived the numerous colonies of
_bacillus coli communis_ which developed on plates from the
cerebrospinal fluid.

=Arteriosclerosis= was little in evidence, being confined to the
coronary, right vertebral and carotid arteries (slight in all). _Cysts
of softening existed in the posterior part of each dentate nucleus_ and
may probably be interpreted as indicating vascular disease.

=Chronic disease outside the nervous system= was prominent and in part
suggestive of senile findings; milky patches of pericardium, adhesions
about liver and gall-bladder, adhesions about spleen, adhesions and
fibrous thickening of parietal peritoneum, adhesions in both pleural
cavities, chronic diffuse nephritis, hypertrophy of bladder wall, dense
calvarium, dural adhesions.

The =nervous system= showed several unexpected features. The _absence of
chronic leptomeningitis_ was striking: the pia mater was everywhere
delicate and transparent except that the walls of the cerebellar and
chiasmal cisternæ were thickened and that there were slight opacities
along the sulcal veins of the convexity. Brain weight 1090 grams. There
was a generalized =sclerosis and pigmentation of the cerebral cortex=.
The sclerosis varied in degree and was most marked in the prefrontal
regions, the anterior halves of the superior frontal gyri, the middle
third of the right precentral gyrus, the region of the splenium on the
left side, and the sagittal rami. If the _bacillus coli communis_ found
in the cerebrospinal fluid had any effect upon the consistence of the
brain, obviously hard to prove in a brain of leathery consistence at the
outset, it was shown only in the right Rolandic area in the vicinity of
the sclerotic part of the precentral gyrus. =Granular ependymitis= of
all ventricles. Weight of cerebellum, pons and bulb, 135 grams.

Perhaps the most remarkable feature of all in the case was the
occurrence of =cysts of softening= in the posterior part of each
=dentate nucleus=. For discussion, see Case 41.


  =VASCULAR NEUROSYPHILIS (?) versus PARETIC NEUROSYPHILIS (“general
  paresis”). Autopsy.=


=Case 41=, like Case 40, was one of arteriosclerotic brain disease with
severe cerebellar involvement. Here is another case in which the Danvers
staff made a diagnosis of general paresis without dissenting voice.
There were some tabetic symptoms, and the spinal cord at autopsy did
show a moderate lymphocytic infiltration of the meninges, entirely
consistent with the picture in the spinal fluid. In this case, the
dentate nuclei of the cerebellum were not destroyed as in Case 40, but
were affected by cell atrophies of variable degree in different parts of
the nuclei. There was also a severe gliosis of the cerebellar cortex.
The left hemisphere of the cerebellum was more severely diseased than
the right. The cortex showed far more marked and generalized cell
atrophies throughout the layers than did Case 40. The details of this
case, which was that of a colored coachman, Samuel North, are as
follows:

He was born in 1871. Learned to read and write at school. Stableman and
coachman. Alcoholic till 1902. Took much quinine, possibly impairing
hearing thereby. Memory impaired and growing worse since 1902. Gait
unsteady for a longer but unknown period. August 13, 1907, wandered
about, instead of attending boot-black stand, muttered, talked
incoherently. In the next few days talked about religion and apparently
had hallucinations of hearing. Committed August 16, 1907.

On commitment stoop-shouldered, flat-chested. Gait staggering.
Unsteadiness in Romberg’s position. Incoördination of arms and fingers.
Coarse tremor of tongue. Tremor of lower jaw. Exaggeration of left
knee-jerk and diminution of right. Exaggerated Achilles jerks. Spurious
left ankle clonus. Questionable Babinski reaction of left side.
Abdominal and epigastric reflexes present but cremasteric absent. Left
pupil smaller than right and fails to react to light. Reaction of right
pupil sluggish. Moderate defect of hearing of both sides.

During the first week the patient developed hallucinations of sight and
hearing, but of no other senses. Disorientation for time, place, and
persons. Answers to arithmetical problems given with assurance but as a
rule incorrectly (as 17 and 32 are 90; 18 divided by 3 is 88).
Handwriting scarcely legible. Memory poor, especially for recent events
(recalled a lumbar puncture as an exercise in baptism). Impressibility
and attention poor. Euphoria.

Death after gradual failure July 29, 1908.

                   Lumbar puncture showed: Per Cent.
                   Endothelial cells               9
                   Lymphocytes                    81
                   Plasma cells                    6
                   Phagocytes                      0
                   Polymorphonuclear cells         4
                   Unclassified                    0
                   Fibroblasts                     0
                   Cells in 100 fields           700

=Post Mortem Findings.= The cerebrospinal fluid showed a pure culture of
_Bacillus coli communis_, and the heart’s blood showed many colonies of
an unidentified bacillus. Culture from mesenteric lymph nodes sterile.

The =cause of death= is somewhat in doubt. There was an early pneumonic
process with fibrinous pleurisy, and there was an early acute
hemorrhagic ileitis with a very slight overlying peritonitis and slight
corresponding enlargement of mesenteric lymph nodes. There was an
infection of the meninges with _Bacillus coli communis_.

Evidences of =chronic disease outside the nervous system= were: coronary
and pulmonary arteriosclerosis, chronic fibrous endocarditis, mitral
sclerosis, aortic sclerosis with calcification, chronic splenitis,
chronic interstitial nephritis, hepatic atrophy (wt., 900 grams),
thickening of cartilaginous portion of right auricle (old trauma), scars
of apices of lungs.

The =calvarium= was dense and the =dura mater= everywhere adherent. The
=arachnoidal villi= were but slightly developed, but there was one small
focus of cortical herniation through the dura mater of the left middle
cranial fossa. The =pia mater= was delicate except for slight opacities
along sulci. There was some pial thickening over the region of the
interparietal sulci on both sides. There was pial pigmentation
anteriorly and superiorly.

There is no gross evidence of intracranial arteriosclerosis, except (1)
that afforded by the lesions of the dentate nuclei of the cerebellum
mentioned below and (2) the swerving to the right of the basilar artery,
possibly due not to arteriosclerotic lengthening of the artery but to an
unusual shape of the pons (see below).

The =brain= weighed 1245 grams (cerebellum and pons 165 grams). =The
anatomical diagnoses of central nervous system= were:

Slight general encephalomalacia (post mortem imbibition of fluid, 31
hours). Slight gliosis of right prefrontal and frontal gyri. Slight
gliosis of right optic thalamus. Generalized granular ependymitis,
especially near fornix and about foramina of Monro. Anomaly of pons (not
gliotic, but possessing far more white matter on the left side than the
right). Severe arteriosclerosis confined to the dentate nuclei of the
cerebellum.

As we now look over the data in Cases 40 and 41 we are inclined to ask
the question, whether modern systematic diagnosis would not have shown
these cases to be NEUROSYPHILITIC? One is inclined to answer this
question in the affirmative, on the basis that Case 40 showed somewhat
questionable Argyll-Robertson pupils, and Case 41 showed unilateral
Argyll-Robertson effect. Both cases showed Romberg sign, but the dentate
nucleus and other cerebellar disease in each case may in some way have
contributed to or imitated this phenomenon. Whether Case 40 was a
tabetic must remain a question, but Case 41 must be regarded as a case
with spinal and meningeal changes highly characteristic of syphilis.


  =VASCULAR NEUROSYPHILIS plus TABETIC NEUROSYPHILIS (“tabes
  dorsalis”) simulating paretic neurosyphilis (“general paresis”).
  Autopsy.=


=Case 42.= The case of Elizabeth Brown was at one time carefully studied
by Dr. A. M. Barrett in his work on mental diseases associated with
cerebral arteriosclerosis and, like Case 43, was one in which tabes
dorsalis was a factor. Elizabeth Brown’s maternal grandfather and mother
were insane; there had also been insanity in a sister. Mrs. Brown was
struck on the head at 44, and was unconscious for an hour, but there
were no sequelae to this accident. At 48, there was a shock, or
apoplectiform attack, followed by unconsciousness for two hours and by
left hemiplegia, right ptosis, and thick speech. Mrs. Brown began to
walk again after two weeks, but was found to be forgetful and
fabulatory. She seemed at times to be hearing music, and somewhat
repeatedly became helpless and unable to walk. She could not remember
from day to day, showed incontinence of urine and feces, and was brought
to the Danvers Hospital. The physical and mental deterioration was
progressive. There were some signs of organic brain disease. The
musculature was especially flabby on the left side. The left angle of
the mouth drooped, and the left nasolabial fold was smoothed out. The
arm movements were ataxic, the tongue protruded to the left, the right
pupil reacted but slightly to light (eye blind from cataract), the
knee-jerks, Achilles, wrist, and elbow reflexes, were absent. The
patient was unable to stand, and there was a marked tremor of the hand,
tongue, and lips. There was a zone of anesthesia for pain and tactile
stimulation extending round the body, from the 3d to the 6th rib, and
there were symmetrical areas of anesthesia on the inner surface of the
forearms and the legs.

The =autopsy= showed a =general arteriosclerosis= with =chronic= and
=acute meningitis=. The brain weighed 1110 grams; the =pia mater= was
moderately thickened; the basal vessels were highly arteriosclerotic.
The brain itself, however, normal externally, upon dissection, showed a
number of small cysts irregularly scattered in the white substance. The
basal ganglia were porous, and there were several small cysts in the
pons. =Microscopically=, there was evidence of severe vascular disease,
involving not only the arteries but also the veins. It was the
superficial rather than the deep arteries that were more often attacked.
There was a marked =perivascular gliosis=. Extensive search yielded _no
evidence of lymphocyte infiltrations_, either in the brain or in the
spinal cord.

The spinal cord showed degenerations in both the lateral and posterior
columns, of which the explanation may possibly be like that in our
paradigm, Case 1.

Is the case of Elizabeth Brown one of neurosyphilis? We cannot
definitely say on account of the non-availability of the modern
systematic tests, but it may well be that the case, although certainly
not one of paretic neurosyphilis, was one of TABES WITH VASCULAR
COMPLICATIONS.


  =TABETIC NEUROSYPHILIS (“tabes dorsalis”) with symptoms of cerebral
  origin producing a picture resembling taboparetic neurosyphilis
  (“taboparesis”). Autopsy.=


=Case 43.= Robert Allen was the fifth case of error in the diagnosis of
general paresis analyzed some years since from the staff meeting records
of the Danvers Hospital. The Allen case resembles the case of Elizabeth
Brown in that there was a combination of tabetic phenomena with cerebral
lesions of a non-paretic character at autopsy. But although there seemed
to be an utter absence of inflammatory cells (lymphocytosis) in the case
of Elizabeth Brown (42), there were some slight perivascular cell
accumulations in the Allen case, with a few mononuclear cells suggestive
of lymphocytes. The cerebrum, however, failed to show plasmocytosis. It
was seriously diseased, showing a marked neuroglia proliferation about
the atrophic nerve cells.

Robert Allen was a printer coming from a long-lived race. The following
are the main facts:

Married in 1875 (two children, healthy); again married in 1893 (one
child, healthy). Compositor from 1890. In 1898 and 1899 girdle and
lancinating pains. Thereafter for several years gait was unsteady.
During 1904 and 1905 freedom from pains and improvement in gait but
gradually increasing irritability and nervousness. Stopped work on last
of March, 1905, owing to sudden increase of irritability, emotionality,
boastfulness, expansive schemes, and ataxia.

Habits: no tobacco, very little alcohol at long intervals. No drug
habits, no sexual irregularity known.

Committed to Danvers April 3, 1905, with slight muscular development,
poor nutrition, acne, irregular, poorly preserved teeth, gingivitis,
flat-foot, slight radial arteriosclerosis, slight arcus senilis, a few
hyaline casts, leucocytes, epithelial cells, and trace of albumin in the
urine, scar in sulcus, and enlarged inguinal lymph nodes.

Ataxic gait, Romberg’s sign, fibrillary twitching of chest, abdominal
and facial muscles when standing; right pupil slightly larger than left,
pupillary margins irregular, light reactions (electric bulb test) both
consensual and direct absent, slight pupillary reaction in
accommodation; biceps, triceps and wrist reflexes lively and equal;
abdominal, cremasteric and plantar reflexes normal, knee-jerks, Achilles
and front taps negative even on reinforcement.

The patient himself stated that his ataxia began in 1904, that he had
been under treatment for swelling of legs and feet and pain in limbs
since 1903, and that there had been some trouble with limbs since 1895.
He had been told that his disease was lead-poisoning. About three weeks
before commitment patient said he had had an attack of unconsciousness.

The patient’s speech showed considerable defect. Words were pronounced
slowly with slurring and tripping especially of the labials. Orientation
perfect. School knowledge well retained. The easier arithmetical
problems were accurately performed. Memory imperfect for minor recent
events. Estimations of space and time often very imperfect. Variability
of mood, sometimes euphoric, sometimes tearful and irritable. Occasional
expansive estimates of personal powers (“Can lift three five-hundred
pound weights with one finger”). Indistinct expansive financial ideas.

The patient continued oriented, euphoric, expansive, untidy, till
October, 1905, but on October 12 developed an infection at the site of a
callus on the sole of the foot and died with pyemic symptoms, October
17.

=Post Mortem Findings.= The =cause of death= was streptococcus
septicemia with acute ulcerative colitis, acute splenitis, bilateral
purulent pleuritis, multiple infarctions of lungs.

There were no signs of =chronic disease outside the nervous system=
except a moderate thickening of the mitral valves, and slight dural
adhesions.

The brain weighed 1450 grams. The vessels at the base showed a slight
degree of sclerosis. There was a slight opacity of the frontal,
parietal, and temporal pia overlying slightly atrophied convolutions,
whose surfaces showed in a few places slight cuppings. The ependyma over
the thalami and the floor of the fourth ventricle was finely roughened.
The spinal cord showed a typical TABES DORSALIS.

Although we probably cannot regard either Case 42 or Case 43 as a case
of paretic neurosyphilis, and although it must remain doubtful whether
they are cases of any form whatever of neurosyphilis (in the absence of
the modern tests), yet it seems clear that both these cases may very
well have been cases of neurosyphilis on account of the existence of a
definite tabetic process in each. The symptoms of these cases, like
those of Cases 38 to 41, suggest how difficult it must be _to make a
clinical diagnosis of general paresis safely without employing available
laboratory tests_. Yet how frequently in the past have neurologists
brought data concerning various phenomena in long series of so-called
paretics in which the error of diagnosis was certainly between 5 and 15%
and frequently still greater. The entire question of the symptomatology
of paretic and non-paretic neurosyphilis, therefore, needs re-opening
and revision.


  =CEREBRAL GLIOSIS (probably non-syphilitic) producing the clinical
  picture of paretic neurosyphilis (“general paresis”). Autopsy.=


=Case 44.= John Hughes was a hostler, and later assistant with a
wholesale drug company, with which he remained for 32 years. He had been
moderately but constantly alcoholic all his adult life up to 50 years of
age, and at 45 had had an attack of so-called nervous prostration, in
which his head had troubled him and he had been seclusive. At 49, he had
a serious attack of otitis media, associated with delirium, swelling of
the feet, and what was called rheumatism. After this attack of otitis
media, Hughes appears to have been not altogether right.

At 53, after a quarrel with his employer, Hughes quit work, began to
trade a little in hens and pigs, became forgetful, especially of recent
events, and did “a variety of peculiar things.” He was a married man but
he had no children. There had been miscarriages but of unknown origin;
venereal disease was denied. At 55, a week before admission, Hughes had
a spell of unconsciousness for several hours, after which his speech was
thick, and restlessness, insomnia, and a wandering tendency set in.
Visual hallucinations, fabulation, tremors, “excited-looking” eyes, are
described. He would sweep things from the dining-room table, pulled a
hot stove into the middle of the floor, attempted to sweep paint off the
floor, and cut up a carpet with a knife.

The patient on commitment November 5, 1904, was well developed and
nourished. The mucous membranes were rather pale. Bruises and
excoriations of limbs. Harsh breathing at the base of each lung.
Enlargement of heart; sounds irregular. Accentuation of aortic second
sound; tension fair, rate 80. Slight brachial arteriosclerosis. Abdomen
slightly distended. The urine contained a faint trace of albumin and
many hyaline casts.

Moderate tremor of extended hands. Slight tongue tremor. Romberg’s sign
absent (slight swaying). Considerable ataxia of extremities (inability
to stand with foot on opposite knee). Vision poor. Hearing could not be
tested accurately. Prompt pupil reactions with direct light. Slight
consensual reaction in left pupil, absent in right. Deep reflexes equal
and lively.

Quiet and orderly at first. Later restless and noisy. Questions were
answered at times relevantly, more often irrelevantly. Patient
irritable, intractable. Required repeated urging to take nourishment.
Consciousness clouded. Orientation imperfect. Attendants are possibly
“officers.” Date September, 1995. Slight errors in repeating alphabet.
Mistakes in Lord’s Prayer with rhyming tendency. Simple arithmetical
tests answered automatically with many mistakes. More complex
combinations incorrect. Handwriting tremulous (noted as “typical of
general paresis”). Auditory hallucinations (answering invisible
persons), “All right, I’m coming.” Amnesia and confabulation. Q. “Have
you had breakfast?” A. “No,” (later) “Yes, I had a very light
breakfast.” Q. “What did you have?” A. “Anything that came along. A few
green peas and beans that were left, bread and butter and pie. I had a
good breakfast. Guess feed is very high.” Q. “Give names of your sisters
and brothers.” A. “There are three or four I never see. I will have to
think them up.” (Later)—“Lillie, Abbie, Julia, George.” On repetition of
question, “Elizabeth, Julia, Annie and Lizzie.”

Delusions somewhat doubtful. At no time euphoria.

The patient remained only nine days in the hospital, developing diarrhea
a week after admission.

=Post Mortem Findings.= The =cause of death= was bilateral
bronchopneumonia of hypostatic distribution, accompanied by bronchitis
and acute splenitis. The intestinal tract was normal (despite the
diarrhea). No cultures. The heart showed acute myocarditis.

The vessels in general showed no sclerosis, except that the aorta showed
a few patches with calcification near bifurcation. There was a moderate
degree of mitral sclerosis. The kidneys showed a moderate degree of
chronic interstitial nephritis. The heart weighed 530 grams and there
was moderate dilatation of all the valves.

There were some evidences of chronic disease outside the nervous system,
namely, an obliterative pleuritis on the right side, chronic
perisplenitis, and chronic external adhesive pachymeningitis.

The =nervous system= showed a pia mater thin and transparent, with a
moderate congestion of larger and smaller vessels. No noteworthy change
of the brain substance or of the ventricles was found, except that the
cerebral substance was of unusual firmness (autopsy twelve hours after
death).

It is clear that the brain was not wholly normal, exhibiting a general
induration due in part to subpial gliosis and in part doubtless to
perivascular gliosis. =Microscopically= the tissues showed features of
great interest, especially multiple focal neuroglia cell proliferations
of a perivascular distribution, considerable subpial fibrillar gliosis
of an unusually focal type, and a rather general subpial cellular
=gliosis=. Histologically, it seemed that this chronic progressive
process had started, not so much in relation with dying nerve cells, as
in relation with blood vessels. The =perivascular= deposits of neuroglia
cells were confined almost exclusively to the infragranular cortex
layers. It seems plain that the diagnosis of general paresis was not
justified. It is probable that the diagnosis of neurosyphilis is not
justified. The explanation may be that now and then cases of cerebral
sclerosis may clinically imitate the neurosyphilitic process. It must be
borne in mind that the diagnosis in this case was made, like the other
cases at head of Part III, without the advantage of modern systematic
methods. Clinically speaking, of course, there was no definite
Argyll-Robertson pupil, although the consensual reaction, slight on the
left side, was absent in the right pupil. The general picture appeared
to be one of the so-called demented form of paretic neurosyphilis.


  =Differential diagnosis between NEUROSYPHILIS and NEURASTHENIA.=


=Case 45.= Albert Robinson, a man of 28 years, was shipwrecked on one of
the Great Lakes. The ship was on the rocks for eight days, and Robinson
was under a great strain. Ever after the wreck, Robinson had felt severe
pain in the head, neck, and back, and a feeling of great weakness
whenever he exerted himself physically or mentally, and seven months
after the wreck, he had several attacks of fainting.

For a number of weeks he had worried a good deal about his inability to
make money, especially as money was badly needed on account of his
wife’s approaching confinement. A few days before entrance, Robinson had
become very forgetful, and was unable to recall, the night before
entrance, where he had been during the day. On the whole, however, on
mental examination no actual evidence of memory defect could be shown to
exist.

=Physically=, Robinson was entirely negative, except for some hard
glands in each groin. =Mentally=, there was little to show except
depression, worry over his financial condition, and his inability to
work. The serum W. R. proved negative.

=Diagnosis=: On the whole, the diagnosis of psychoneurosis (see case
Harrison (9)) due to the shock at the time of the shipwreck seemed to be
proper. To be sure, the patient gave a history of a chancre at 25,
treated for two years, after which he was declared cured.

However, following up the clue of admitted syphilis, rigorous
questioning elicited the fact that a few months before there had been
diplopia, lasting part of a day.

=Lumbar puncture= seemed desirable. The fluid was clear but contained
125 cells per cmm. with appropriately increased amounts of albumin and
globulin. The spinal fluid W. R. was positive. The diagnosis of
CEREBROSPINAL SYPHILIS seemed established.

The lesson of this case appears to be that perhaps we should never
exclude syphilis until we have made an examination of the cerebrospinal
fluid. The W. R. of the blood in meningovascular (non-paretic syphilis)
is negative in many cases (the figure is sometimes set as high as 40%).

=Treatment=: After a half dozen injections of salvarsan, all symptoms
disappeared and Robinson went back to work, claiming to be in a better
condition than for some time past.

    1. How shall we explain such a symptom as the transient diplopia?
      This diplopia is probably an example of a neurorecidive, but it
      will be observed that it occurred without salvarsan therapy. See
      discussion above under the case of Bennett (34), where the general
      result of the neurorecidive inquiry launched by Ehrlich early in
      the history of salvarsan therapy showed that precisely similar
      phenomena had always occurred in neurosyphilis, whether under
      treatment or not. The anatomical and histopathological explanation
      of such phenomena is, of course, doubtful, but a review of the
      findings in the case of Alice Morton (1) will show how many
      apparently serious symptoms in neurosyphilitics are actually
      irritative or at least due to lesions which are entirely
      recoverable. We may suppose, first, a local proliferation of
      spirochetes; second, a local over-formation of toxic substances,
      directly or indirectly the product of spirochetosis; thirdly, a
      local exudation; fourthly, a local proliferation; fifthly, a
      combination of these phenomena, any or all of which may be
      regarded as but transient. We have sometimes found at autopsy very
      little exudate except in small areas; sometimes not more than a
      few mm. or cm. in superficial extent. Note, for example, the small
      areas of lymphocytosis demonstrable in but two foci in the case of
      Alice Morton, the paradigm placed at the beginning of this book.


  =NEUROSYPHILIS(?) in the SECONDARY STAGE of syphilis. HYSTERICAL
  symptoms. Diagnosis?=


=Case 46.= Alice Caperson was a colored girl of 18 years. She had
acquired syphilis five months before admission to the hospital, and the
secondary symptoms of this syphilis had just disappeared before
admission.

Very shortly after acquiring syphilis, the young negress began to act
peculiarly. She describes herself as having a sort of nightmare, both
when asleep and also when awake. For instance, she saw her dead
grandmother. It appeared at first like a seraph; then it came nearer to
her and seemed to fill out; and then was dressed precisely as her
grandmother had been. This seraph appeared as though trying to tell her
something, but she could not make out what the something was. The vision
had appeared on two or three occasions.

Our examination detected little beyond instability and irritability of
mood with some depression. The patient readily fell to weeping. She soon
made friends in the wards, however, and got on well. =Physical
examination= was entirely negative but the W. R. of the blood serum was
positive. The W. R. of the spinal fluid was negative, as was the gold
sol reaction; there was an excess of albumin and a positive globulin
test; there were seven cells per cmm.

The psychiatric diagnosis of a case like that of Alice Caperson would
waver between hysteria and dementia praecox. However, as for dementia
praecox there are hardly any typical symptoms. There is insight into the
hallucinations, which are hypnagogic. There are, however, no hysterical
stigmata.

The spinal fluid reaction is typical of the secondary stage of syphilis.
It is commonly said that in every case of syphilis the nervous system is
involved at some period, if only to the degree shown in the present
case. However, such involvement tends to disappear both with and without
antisyphilitic treatment, just as do the secondary skin symptoms. So far
as syphilis is concerned, the prognosis under radical treatment is as
good as usual. We are inclined to regard the case as one of the
HYSTERICAL or PSYCHOPATHIC group and inasmuch as cases occurring in the
developmental stage of a patient’s life are of fairly good general
prognosis, we are inclined to regard the prognosis in this particular
case as good under proper therapy and hygiene.

    1. What is the relation of neuroses to syphilis? Neurasthenia,
      chorea, hysteria, and epilepsy are often grouped (for example, by
      Nonne) as neuroses bearing at times important relations to
      neurosyphilis. (For the relations of neurasthenia, chorea, and
      epilepsy, see cases of Greeley Harrison (9), Margaret Green (72),
      and David Borofski (49), respectively.) As for the hysteria shown
      in Caperson, Charcot enumerated syphilis among _agents
      provocateurs_ of hysteria along with alcohol, lead, arsenic, and
      the like. Fournier has also considered the problem. It is clearly
      necessary to show that before infection there were no hysterical
      symptoms, and that the hysteria developed during the operation of
      the syphilitic process, and it is probably necessary to show that
      the symptoms will clear up under antisyphilitic treatment, if we
      are to concede the existence of a syphilitic hysteria.

    2. What are the evidences of neurosyphilis in the secondary and
      primary stages of syphilis? As above stated, the findings in
      Caperson are typical enough. Wile and Stokes at first stated that
      60 to 70% of the secondary syphilitics show changes in the spinal
      fluid; in a further article they maintain that probably every case
      shows such changes and that clinical symptoms of neurosyphilis of
      the secondary period can probably be determined. They claim that
      it is probable also that the same holds for primary syphilis
      itself. The importance of these claims lodges partly in the
      relation of these early signs of neurosyphilis to the whole
      question of latency and to the question of _paresis sine paresi_.
      For a discussion of _paresis sine paresi_ see cases Lawlor (25),
      Vogel (52).


  =Differential diagnosis between NEUROSYPHILIS and MANIC-DEPRESSIVE
  PSYCHOSIS.[9]=


=Case 47.= As in other instances (compare Martha Bartlett (21) and Annie
Monks (85)) so also in the case of Ethel Hunter, a woman 61 years of
age, there was no initial suspicion of neurosyphilis. Mrs. Hunter was
brought to the hospital stuporous as a result of an overdose of
paraldehyd. The paraldehyd had been administered by a physician to
combat insomnia and agitation. As soon as Mrs. H. had recovered from the
drug stupor, this agitation appeared once more, and it was clear that
she was suffering from marked depression. There was tremendous worry
over the sickness of a woman with whom the patient lived. The patient
was very self-accusatory, blaming herself for many things that had
happened in the household. Besides her agitation, depression,
self-accusations, and insomnia, the patient showed a good deal of the
symptom frequently termed “retardation”—a kind of lagging of all mental
processes found, according to Kraepelin, in manic-depressive psychosis.

Accordingly, the diagnosis of manic-depressive psychosis might well have
been rendered. The fact that the psychosis so far as known began in the
involution period was not against the diagnosis since the so-called
involution-melancholia of this period is at least in a certain fraction
of cases nothing more or less than a form of manic-depressive psychosis.
However, the =physical examination= made the diagnosis of
manic-depressive psychosis a little doubtful. There was a superficial
thickening of the arteries (blood pressure: systolic, 170; diastolic,
104), which thickening would not in itself be against the diagnosis of
manic-depressive psychosis. (In point of fact, arteriosclerosis is
rather common late in this disease and previous attacks could not be
excluded on the basis of available history.) The contracted pupils were
irregular and both reacted sluggishly to light, although better to
accommodation; the right pupil was larger than the left. The arm
reflexes were pretty active. The left knee-jerk could not be obtained,
nor was the right knee-jerk more than very sluggish. The Achilles
reflexes could not be obtained. Although there was not a positive
Romberg sign, there was a considerable swaying in Romberg position.
There was no speech defect. The other reflexes showed nothing abnormal.
On the whole, we had to conclude that, although Mrs. Hunter might be an
instance of manic-depressive psychosis, still there was much of
neurological interest in the case.

This conclusion was emphasized when the W. R. of the blood serum was
found to be positive. The spinal fluid W. R. was also positive, and the
gold sol index was of the “paretic” type. There were 74 cells to the
cmm. Globulin stood at ++++, and albumin at ++++.

This case, therefore, again illustrates, as well the protean nature of
GENERAL PARESIS (the diagnosis rendered), as the doubtful value of
making a psychiatric diagnosis without due consideration of the physical
examination and laboratory findings. How easy might it have been, at
least some years ago, to consider that this patient of 61 years had
suffered a slight shock at some previous time (left knee-jerk absent),
but was as a matter of fact a case of manic-depressive psychosis with a
vascular complication!

Note: We must again duly insist that the merely sluggish light reactions
of the pupils in such a case as this do not especially point to general
paresis. The literature seems to establish that sluggishness of light
reaction precedes the classical Argyll-Robertson pupil. Yet it does not
do to say that, if the Argyll-Robertson pupil pretty conclusively points
to neurosyphilis (for exceptions see cases Falvey (55), Murphy (60)),
then a sluggish pupillary reaction to light looks in the same direction.
Sluggishness may precede stiffness in many, or perhaps all, cases, but
sluggishness of pupils is a frequent phenomenon outside the syphilitic
group of cases.

    1. What part is played by emotional shock and psychic causes in the
      starting up of general paresis? The answer to this question cannot
      be definite. That a paretic process can be started up after trauma
      is admitted on all sides; but we here suppose actual physical or
      chemical brain disturbance permitting increased spirochetosis or
      inflammatory reaction. In the case of psychic shock, or what might
      be called _psychogenic general paresis_, our best resort will be
      to the indirect effects of hormone action, or of vasomotor and
      other autonomic disturbances produced directly or indirectly by
      emotion. We are clearly here dealing with material too speculative
      to be of practical service at this time.

    2. Was the depressive drug therapy in the case of Hunter
      justifiable? The paraldehyd had been administered by a physician
      apparently on purely symptomatic grounds to combat the insomnia
      and agitation of this woman of 61 years. With all due
      acknowledgment of the difficulties of private practice, we must
      insist that when ordinary measures in the relief of insomnia and
      agitation are insufficient to curb these conditions, then a
      positive danger ensues with the larger doses. As a rule, with
      these larger doses and with the withdrawal of sensory stimulation,
      the patients relapse into a stupor of grave moment. We need only
      recall the situation in delirium tremens where adequately
      depressive drugs often tend to kill the patient.


  =Case for diagnosis. Errors in the diagnosis of NEUROSYPHILIS are
  possible even when abundant clinical and laboratory data are
  available.=


=Case 48.= The first error chosen for demonstration is that in the case
of the machinist, Milton Safsky.

Safsky, about 8 months before his entrance to the hospital in the 42d
year of his life, had begun to lose strength, to grow thin and pale, and
to suffer from an extreme and continuous thirst. He was said to have
drunk as much as 6½ gal. in a day, and passed appropriately large
quantities of urine. After a time, his management at a general hospital
became difficult, as Safsky became confused, cried “hysterically,” and
was at times very noisy. He sustained a marked memory loss, seemed to
show visual hallucinations, and complained of headache, both frontal and
occipital, and of pain about the eyes. Sometimes the patient was very
euphoric and expressed what seemed to be delusions of grandeur, saying
he was wealthy and owned many machine shops.

Some symptoms, e.g., polydipsia and polyuria amounting to a diabetes
insipidus, associated with headache and arrested attention, suggested
possibly a new growth in the pituitary region. The mental symptoms might
naturally be supposed to be due to some infiltration or pressure effect
of intracranial growth. After admission to the Psychopathic Hospital,
the patient was found difficult to arouse, although he could eventually
be aroused. His orientation proved to be as poor as his memory. From
time to time, the patient became a bit more intelligent and able to
execute requests.

The =physical examination= was in general almost entirely negative.
=Neurologically=, the pupils were markedly contracted and reacted slowly
to light, though they were otherwise normal. The deep reflexes were all
somewhat lively, though equal. The umbilical and cremasteric reflexes in
particular were present. Systematic examination revealed no other reflex
disorder, nor any disturbance of sensation. There was a coarse tremor of
the extended hands. There were no phenomena of importance in the visual
fields.

As against the diagnosis of growth, pituitary or extrapituitary
(diabetes insipidus and headache), a hypothesis of neurosyphilis had to
be considered. Not only were the contracted, slowly-reacting pupils and
the active deep reflexes suggestive, but the euphoria with grandiose
ideas looked entirely consistent. As for the polyuria, one had to think
of the so-called syphilitic polyuria of the textbooks, which is regarded
as a more or less characteristic result of syphilitic involvement of the
_basis cerebri_. Moreover, the W. R. in the spinal fluid proved to be
slightly positive; 146 cells per cmm. were found therein; there was a
large quantity of globulin, and a very marked increase in albumin. These
observations seemed to be exceedingly suggestive of a cerebral syphilis.

However, as the case progressed, the diagnostic situation changed. The
W. R. upon a second puncture fluid proved negative. After some weeks,
characteristic symptoms of intracranial pressure developed; the
diagnosis of BRAIN TUMOR had to be taken as established, and there is no
doubt of its correctness.

    1. What is the explanation of the weakly positive W. R. in Safsky’s
      spinal fluid? An explanation is not easy to find. Possibly we may
      regard the reaction as an example of error in technique. It is
      even possible that it may have been produced by exudative products
      in the spinal fluid.

    2. What precautions may be taken against an error in diagnosis such
      as was first made through the positive spinal fluid Wassermann in
      the case of Safsky? First, repetition of the W. R.; secondly, it
      is very unusual to find a weakly positive W. R. in a case with
      such marked excess of albumin and such very marked increase of
      globulin as was shown by this case.

    3. How can we explain the inflammatory products in the puncture
      fluid? Superficial brain tumors are frequently associated with a
      so-called _meningitis sympathica_. The products of such meningitis
      are exhibited: _viz._, globulin, albumin, and pleocytosis, exactly
      as shown in Safsky.


  =Can PARETIC NEUROSYPHILIS (“general paresis”) appear clinically
  EARLY (e.g., two years) after the initial syphilitic infection?=


=Case 49.= David Borofski, a street car conductor, 27 years of age,
suddenly had a convulsion while at work in his car. For four months
Borofski continued to have rather numerous convulsions, was finally
compelled to discontinue work, and resorted to the Psychopathic
Hospital. It appears from his own story that, about two years before, he
had had a chancre, for which he had been treated at a general hospital
syphilis clinic, and of which he was told he was cured. With a
progressive loss of memory and with convulsions, Borofski became much
concerned about himself, and was finally persuaded by his fellow-workers
to come to the Psychopathic Hospital.

The convulsions were described as follows: The patient gives a short
cry, has convulsive movements for about ten minutes, remains unconscious
for perhaps half an hour, and wakes with headache, dizziness, and a
feverish appearance. Sometimes the attacks were more severe, with
frothing at the mouth, biting of lips, and loss of sphincter control.
There were also slight attacks, occurring almost every day, without loss
of consciousness; these latter attacks consisted of dizziness, inability
to speak for a few seconds, and some arm twitching.

=Physically=, Borofski was well developed and nourished, with a blood
pressure of 160. The only abnormal phenomena =neurologically= were
absent knee-jerks and ankle-jerks, sluggish pupillary reactions, and
slight tremor of the hands.

=Mentally=, despite suggestive complaint of amnesia, the memory was
found to be fairly good but knowledge of current events and school
knowledge was poor. The simplest problems in arithmetic Borofski gave
up.

The first diagnosis in such a case would naturally be epilepsy. However,
when an epileptic or epileptiform attack occurs for the first time in
adult life, the chances are probably against an idiopathic epilepsy.
(This is not a universal rule but will serve.) Borofski himself,
moreover, gave a history of syphilis. And the very nature of the
attacks, with arm twitching and without loss of consciousness, would not
readily fit into the frame of the idiopathic group. The absence of
certain reflexes and the sluggish pupils are naturally also suggestive
of syphilis, although not convincing.

The W. R. of the serum proved positive, as did that of the spinal fluid.
The gold sol reaction was characteristically “paretic”; there was an
excess of albumin and a positive globulin, and there were 15 cells per
cmm. There could be little or no doubt of the diagnosis of some form of
neurosyphilis. The laboratory picture was consistent either with general
paresis or with cerebrospinal syphilis. So far as we are aware in the
present stage of knowledge, the two conditions can hardly be
differentiated unless we choose to rely on therapeutics. However, it is
exceedingly rare for general paresis to occur only two years after the
original infection. If we can trust this statistical fact, we shall
perhaps be wiser to term the case of Borofski one of DIFFUSE
CEREBROSPINAL SYPHILIS, and not one of paresis.

=Treatment=: Borofski was put on antisyphilitic treatment consisting of
0.6 gram of salvarsan twice a week and potassium iodid, together with
intramuscular injections of mercury salicylate. The convulsions then
ceased. After four months Borofski returned to work, and he has remained
at work for a year. He has never regained his former health.

Fifteen months after beginning of treatment the laboratory tests were
again made (there had been more than 60 injections of salvarsan), and
the cell count and gold sol reactions were found to be negative.
Globulin and albumin were also in smaller amounts than in the original
examination. However, the W. R. of the serum and the spinal fluid
remained positive.

Head and Fearnsides state that cases of cerebrospinal syphilis should
return negative spinal fluid tests after six months of treatment. Upon
this criterion of Head and Fearnsides, Borofski would not be a case of
cerebrospinal syphilis; but it is probably impossible to separate
various forms of neurosyphilis into categories on any such grounds.

    1. Shall case David Borofski be regarded as one of paretic
      neurosyphilis (“general paresis”)? He has returned to work and has
      remained at work, though without regaining his former health. In
      any event, however, he does not offer the typical picture of
      inevitable decline and death presented by the typical case of
      Pietro Martiro (15) presented in our discussion of systematic
      diagnosis. However, we could not upon laboratory grounds, or even
      upon the ground of clinical observation, distinguish Borofski from
      Martiro; Borofski has greatly improved; Martiro is dead. Borofski
      developed his obvious neurosyphilis only two years after the
      original infection. The conservative syphilographer might,
      accordingly, reply that David Borofski is not a typical case of
      paretic neurosyphilis (“general paresis”) either in the length of
      the incubation period for his neurosyphilitic symptoms, or in his
      outcome.

    2. What is the cause of such convulsions as those developed by David
      Borofski? Evidence from clear cases of general paresis with
      convulsions leads to the hypothesis that such convulsions as those
      developed by Borofski are not necessarily based upon frank
      destructive lesions such as would be produced by the plugging of
      terminal arteries. They may well be produced through the
      activities of minor lesions, only demonstrable by microscopic
      methods, either through properly disposed cell losses or by the
      pressure of exudate, or even by endotoxins or other substances
      derived from the bodies of dead or living spirochetes.

    3. Aside from the well-known syphilitic epilepsy due to meningitis,
      is there a non-meningitic epilepsy (such a disease as Fournier
      formerly described under the term parasyphilitic epilepsy)? We
      dismiss from discussion the so-called symptomatic epilepsies which
      are the result of a gross organic disease of the brain substance
      or its membranes, and which do not differ so far as we are aware
      from organic epilepsy produced by other gross lesions of an
      identical size and structure. These symptomatic epilepsies may be
      partial, or even may present the appearance of generalized
      epilepsy. We may also leave out of account those epileptic
      pictures which are produced in general paresis itself, and which
      may be viewed as nothing but partial phenomena of general paresis.
      The kind of so-called “parasyphilitic” epilepsy that Fournier
      described is a kind of epilepsy that cannot be distinguished from
      genuine epilepsy, in which the sole disease-phenomenon throughout
      a long period of time consists of epileptic convulsions. It
      appears that these “parasyphilitic” imitations of genuine epilepsy
      occur in individuals with a very long post-infective “incubation
      period,” but that there are some cases in which the epilepsy
      appears, on the contrary, in the very earliest stages of syphilis.
      The attacks are a little less common than those of idiopathic
      epilepsy; they have the same apparently causeless beginning; are
      associated with complete amnesia; and are followed by
      characteristic dazed states. The patient’s intelligence, however,
      suffers little. Now and then a case reacts well to antisyphilitic
      treatment energetically pushed. (Spontaneous long remissions in
      non-syphilitic epilepsy must be remembered.) Petit mal attacks
      occur sometimes between the more severe attacks. In short, it
      would appear that there is a group of syphilitic epilepsies in
      which the brain shows no gross structural lesions, which
      accordingly do not exhibit any Jacksonian appearances, and which
      last a comparatively long time without changing their character,
      and often without being especially altered for the better by any
      form of antisyphilitic treatment. This condition is sometimes
      known as a post-syphilitic epileptic neurosis. Nonne had been able
      to collect up to 1902 some 12 cases from his own service.

    4. Would it be proper to call Borofski a case of taboparesis? Absent
      knee-jerks in a victim of paretic neurosyphilis should not be used
      to suggest a diagnosis of taboparesis. This question of
      terminology has been discussed above, under Sullivan (16).

    5. What is the mechanism by which the amnesia of a case like
      Borofski is produced? The answer runs in the same terms as the
      answer to the questions concerning the cause of convulsions. The
      amnesia in general paresis has surprising functionality. A study
      of autopsied cases of general paresis has shown that amnesia is
      practically as common in cases without marked destruction of brain
      tissue as in cases with atrophy of classical extent and depth. The
      clinical recovery in this case was practically complete in respect
      to memory. We must regard the amnesia as not due to the
      destruction of storage cells bearing the so-called neurograms
      (Morton Prince).

    6. What is the explanation of the persistently positive W. R.’s of
      the serum and spinal fluid associated with diminished globulin and
      albumin tests, a negative gold sol reaction, and normal cell
      count? See discussion under Case Martha Bartlett (21).

    7. How atypical is the early development of paretic symptoms in
      David Borofski? C. B. Craig has collected, in 100 cases of brain
      syphilis (a list including both paretic and non-paretic cases),
      some data on this point. The shortest period reported by Craig was
      in a case in which the neurosyphilitic symptoms appeared one month
      after infection. Craig found three cases where symptoms appeared
      in six months, and six cases within a year. The longest
      post-infective period of Craig’s list was thirty years. Our case
      of Chatterton (73) developed symptoms 33 years after infection and
      Washington (66), forty years after infection. Nonne casts some
      doubt on statements to the effect that tabetic symptoms may occur
      three to four months after infection. It seems to be admitted that
      pupillary anomalies and reflex changes may occur in the early
      secondaries and may recover under antisyphilitic treatment.
      Nonne’s case of longest post-infective interval, like that of
      Craig, was one of 30 years.

      Myerson has reported a 20–year old patient who acquired chancre
      April 1, 1911 (spirochetes demonstrated); salvarsan was
      administered April 20th. There were no secondary symptoms, but in
      May, headache, visual disturbance, vertigo, and other symptoms
      developed (neurorecidive). Upon June 20th, that is, 11 weeks after
      development of the chancre, aphasia and astasia developed, with
      numbness of the left side. At this time, the pupils were slightly
      irregular and unequal but reacted normally. The signs in the fluid
      were positive. Upon this question see our cases of Bright (121)
      and Bennett (34).


  =Hemitremor following hemiplegia in PARETIC NEUROSYPHILIS (“general
  paresis”). Autopsy.=


=Case 50.= Achilles Akropovlos, 39 years, had symptoms six months before
commitment to Danvers Hospital. There were attacks of confusion,
difficulty in walking, and speech defect, resulting in an entire
incapacity to work and eventual commitment. Rather unusual and striking
was a very marked tremor, apparently limited to the right side of the
body. =Physically=, Akropovlos was normal, but =neurologically= he
showed, in addition to the marked right-sided tremor, a marked speech
defect, and a degree of ataxia. The tendon reflexes were very active,
but there were no abnormal reflexes, and the pupils reacted normally.
According to the history, the difficulty had followed a slight attack of
apoplexy. =Mentally=, there was a marked confusion. The blood serum and
the spinal fluid were both positive to the W. R.; globulin was present,
and albumin was increased; there were 43 cells per cmm. There was hardly
any diagnosis to make except general paresis.

Death followed 18 months later, or two years after onset of symptoms.
Increasing weakness, emaciation, and dementia preceded death. Autopsy
confirmed the diagnosis of PARETIC NEUROSYPHILIS.

    1. What is the usual cause of death in general paresis? Intercurrent
      disease very frequently occurs in general paresis, and such
      intercurrent disease is then given as the cause of death. As a
      matter of fact, however, one feels that in many of these cases the
      intercurrent pneumonia or infection—frequently of the
      bladder,—bedsores, sepsis, and the like, are merely accidental
      incidents in a condition that is leading to death, and which has
      caused a lowered resistance to infection. In certain instances
      where nursing is exceptionally good and where no such infection
      occurs, the patient continues to grow weaker and weaker, paralyses
      of all the muscles follow and finally paralysis of deglutition or
      respiration may lead to death. The emaciation and paralyses may be
      of such a grade that the patient is entirely devoid of fat and
      unable to move at all. Not infrequently vascular crises occur, and
      one of these may be responsible for death.

    2. What was the cause of the hemitremor? The hemitremor suggested an
      irritative or destructive lesion in the motor path. Delving into
      the history it was learned that the patient had had a shock
      followed by a right hemiparesis. This had cleared up leaving the
      tremor as a residuum. The autopsy disclosed a reddish-brown
      pigmentation and fibrous thickening of the pia over the left motor
      area, confirming the idea of a previous hemorrhage. As a rule the
      shock phenomena occurring in paresis clear up more completely and
      no gross lesion is visible post mortem. However, cerebral
      hemorrhage must be expected in any person suffering from syphilis,
      and is no rarity in paretic neurosyphilis.


  =PARETIC NEUROSYPHILIS (“general paresis”) with NORMALLY REACTING
  PUPILS. History of trauma. Autopsy.=


=Case 51.= Daniel Wheelwright, a barber of English extraction, 57 years
of age, had had a sunstroke at 15. At 42, there had been pneumonia,
after which an attack of rheumatism was said to have kept the patient
from work for a year. There was trauma of head (falling wrench) at 44.
This blow on the head was the assigned cause of the mental disease,
symptoms of which, however, did not develop until about the first of
September, 1905, about three months before entrance, January 9, 1906,
and about six months before death, March 20, 1906.

It seems that the patient had begun to change in manner; he had become
despondent and apathetic, silent, and somnolent. Two weeks later, he
stopped working, began to read the papers once more, and became somewhat
more cheerful.

About Thanksgiving, Wheelwright got up at midnight, and remained up,
lighting all the fires and talking continuously. During the next two
weeks, he talked much to himself, laughing out at times. About two weeks
before Christmas he went out and started to make a sidewalk of old
boards, working in his shirtsleeves, without a hat. He would work until
midnight making screens for windows. During the day, he would go out and
give money to passing children; would offer to pay the grocer twice as
much as articles were worth.

On the day before Christmas, he put out all the fires and lights in the
house, sent all the family to bed, and opened all the doors. Christmas
morning, he rose early and got the washtubs ready. He helped his
compliant wife to do the washing, then put out all the fires and opened
the windows. After Christmas, he began to tell how rich he was going to
be through starting a garden and by making butter. He bought six or
seven quarts of milk daily, and procured carrots and oranges, grinding
them up to color the milk. January 9th he was committed to Danvers
Hospital.

=Physically=, there were few symptoms. =Neurologically=, there was a
tremor of tongue, fingers, and face. The knee-jerks were lively. The
pupils reacted normally; the patient was restless, pacing up and down.
There was a speech defect demonstrable with test phrases. Orientation
was imperfect for time and for place. Handwriting was poor, memory
impairment was marked, but the patient was given to fabrication as to
past events. A characteristic sample of statements:

“Do you know that this is an insane hospital?” “Yes; there are two or
three men here out of their heads. I could cure them with my hands but
they won’t let me. I could get all the sick men on their feet just by
rubbing them. I can do anything with my hands. I can build a house by
just sitting down and thinking about it. I can whip all the men in this
place. I have better sense now than I ever had in my life.”

Again, “How long have you been here?” “Over three months; they have put
me in heaven three times since I have been here. They killed me, crushed
my heart, and turned my blood to water. I am all right now. I let the
sun shine on my heart and it brought it together. I can whip every man
in here as fast as they come up.”

Again, “I will make a million dollars on my garden when I get it. I can
make a million dollars on half an acre. I can do anything. I can move
this house by just thinking of it.”

During a special examination, the patient told how he had fastened wings
on his hands and feet, and how he had gone to heaven; he told how he had
soared high above the earth, and how differently the stars look when up
near heaven than they do from the earth. He spoke of seeing angels and
of the beauties of heaven.

The diagnosis of PARETIC NEUROSYPHILIS was confirmed at autopsy.

    1. What is the significance of the normally reacting pupils? While
      it is usual to find pupillary anomalies in neurosyphilis, these
      changes are not an essential part and it is not rare to find
      normal pupils in all forms of neurosyphilis. It is less frequent
      to find a normal pupil in tabetic than in diffuse or paretic
      neurosyphilis. In paretic neurosyphilis it is the rule to find
      pupillary changes during some stage of the disease, but not
      necessarily early. At times the pupillary sign may be one of the
      earliest signs of neurosyphilis—again it may occur only as a late
      symptom, if at all. One of the most important of the pupillary
      signs is irregularity of contour. While this does not always mean
      neurosyphilis it is highly suggestive and certainly indicates
      careful examination even though the W. R. in the blood be
      negative.

    2. What was the relation of trauma to the development of the
      neurosyphilitic symptoms? It is, of course, the rule in all forms
      of mental disease to have some factor offered by the patient or
      relatives as the cause of the psychosis. Often these assigned
      causes are minor events thought of only after the later appearance
      of symptoms. In this case it was not thought that the trauma had
      any causal effect. For a discussion of trauma and neurosyphilis
      see cases Joseph O’Hearn (90), Levi Sussman (91), and Joseph
      Larkin (92).


  =NEUROSYPHILIS, probably PARETIC, with symptoms highly suggestive of
  MANIC-DEPRESSIVE PSYCHOSIS.=


=Case 52.= Bessie Vogel[10] was admitted to the Psychopathic hospital
New Year’s day, 1915, in a very much excited condition. The family
history is very meagre, and all that is of significance is that mother
has always been very “nervous.” The records in part:

=Past History.= Very healthy as a child, and except for occasional
throat trouble and headache had no physical ailments until eight years
ago, when she had an operation for appendicitis, and two and one-half
years ago was operated upon for hernia and adhesions. Following this she
began to show a lack of energy, neglected her housework, was much
depressed, wept frequently, complained constantly of pain in various
places, and was ill-tempered. In about five months she improved, and
then after a couple of weeks at the shore seemed entirely well.

=Present Illness.= In November, 1914, that is, about seventeen months
after the recovery from the previous depression, she again began to show
practically the same symptoms. She was depressed, could not sleep, and
would get up in the night and sew; was self-centered and hypersensitive,
then became restless and nervous; wanted to go shopping and out for
dinner; went to New York and then to New Bedford. Symptoms became more
marked; she became very ill-tempered, threatened her husband when angry
over trifles, threatened suicide, then began to get active and spent
money extravagantly. At the end of two months, that is, Jan. 1, 1915,
she was admitted to the hospital.

=Physical Examination.= A small, thin woman, appearing to be about 45
years old (actual age 37). Aside from the absence of teeth and the
operation scars, the general examination is negative. =Neuromuscular
system=: The pupils are round, regular, equal, and react to light and
accommodation, but do not hold very well. Extraocular movements well
performed, no palsies of facial muscles, tongue protruded medially
without tremor. Uvula is raised symmetrically. Biceps and triceps and
supinator reflexes are present and brisk. Patellar and Achilles reflexes
are equal on the two sides and brisk. Abdominal skin reflexes not
obtained. Plantar reflex active and flexor in type. No Babinski, Gordon,
or Oppenheim. No tremors.

Wassermann reaction serum positive. Examination of spinal fluid: clear,
globulin ++++, albumin ++++; cells, 130 per cmm.; small lymphocytes,
79.9%; large lymphocytes, 14.1%; polymorphonuclear leucocytes, 4.6%;
plasma cells, 0.7%; endothelial cell, 0.7%. W. R. positive. Gold sol
reaction, 55555522 +-.

=Mental Examination.= On admission patient showed great psychomotor
activity, was very playful, marked flight of ideas, was expansive, very
emotional, very erotic. She slept very little, appetite was poor, and
she lost weight rapidly. Orientation and memory intact. No
hallucinations elicited. In about three weeks improvement began, and at
the end of eight weeks she appeared practically recovered. On April 9,
1915,—that is, 13 weeks after admission,—she was allowed home on visit.
On leaving, she appeared normal in every way. There was no evidence of
psychotic symptoms, she had good insight, and physically there was
absolutely nothing of a neurological nature that was abnormal.

This case, with the history of a previous depression and its clinical
picture during the acute stage, and its recovery, is certainly in every
respect typical of manic-depressive insanity, and only the positive
result of the six tests causes us to put it in the group of GENERAL
PARESIS. Only the further course will shed any light as to the correct
significance of these findings, and even then we shall not be too sure
that we had not been dealing with a manic-depressive psychosis in a
latent neurosyphilitic. We would strongly emphasize the point that at
the present time this patient presents no mental or physical signs of
cerebrospinal syphilis or general paresis; but the six tests are still
positive. This case differs from the ordinary general paresis remission
in that there is not a single physical sign of paresis present.

There are many transitional cases between this case which shows no
symptoms or signs of neurosyphilis except the laboratory tests, and the
typical case of general paresis. Thus we have cases with slight
character change and no physical signs except rare “seizures.” On the
other hand, in many cases the presence of abnormal neurological
phenomena without definite mental signs is first noted. Certain remitted
cases show only some slight pupillary or reflex abnormality. We believe
we have here added the last link in the chain between the primary and
quaternary symptoms.

This case is illustrative of several which we have published elsewhere
under the name of _paresis sine paresi_ or latent neurosyphilis to
illustrate how all the laboratory signs of neurosyphilis may be present
in a patient without any physical or mental symptoms that may be
correlated with these findings.

We summarize our discussion of this as follows:

1. There is a group of cases showing the laboratory signs characteristic
of central nervous system syphilis: (_a_) positive W. R. in the serum,
(_b_) positive W. R. in the spinal fluid, (_c_) pleocytosis, (_d_)
excess of albumin, and (_e_) of globulin in the spinal fluid, (_f_) gold
sol reaction of central nervous system syphilis, and which show no sign
or symptom of neurosyphilis.

2. We believe these cases represent a form of chronic cerebrospinal
syphilis, probably paretic in type.

3. They have the greatest theoretical and practical significance in the
consideration of the life history of neural syphilis, in the concept of
_Allergie_, in regard to results of treatment, and finally as to the
evaluation of the laboratory tests.

4. Here is perhaps offered the last link to form a complete chain
between the symptoms of the primary stage of syphilis and its final
termination of life as the result of the diseases cerebrospinal syphilis
or general paresis.


  =SYPHILIS (?); EXOPHTHALMIC GOITRE; neurosyphilitic old lesion of
  optic thalamus; unilateral induration and atrophy of left cerebral
  cortex. Autopsy.=


=Case 53.= Carrie Pearson, a housewife 25 years of age, died at Danvers
Hospital less than a week after admission, and it was at first stated
that her symptoms had lasted but two weeks before admission. In point of
fact, a further investigation showed an important succession of
symptoms, lasting some four years.

Carrie had been considered a healthy child, going to school at the usual
age, and progressing well with her studies. She however, left school in
the ninth grammar grade, at the age of 15, and went to work in a
milltown. She married a worthless person at the age of 18, and lived
with her husband for three years. There was one child born a year after
marriage. Two years later, however, a tremendous goitre had developed
such that her neck was described as “out square with the face,” and at
the same time the patient’s eyes had become prominent.

About two weeks before admission, she had gone to a neighboring town to
take care of a sick woman, but during her endeavor to be a nurse, she
had broken out into a mania, tearing up furniture and bedding, and
talking irrelevantly for a period of four days. She also showed insomnia
and continually tore off her clothing from her body.

Upon =examination=, the marked enlargement of the thyroid gland together
with the prominent eyeballs, husky voice, and pulse rate of 150 per
minute, were entirely consistent with the diagnosis of exophthalmic
goitre. The patient described herself as “Carrie Nation.” Asked to write
her name, she took the pen and tried to spatter ink, wrote hurriedly and
carelessly her maiden name and several words without apparent meaning.
Asked to write, “God save the Commonwealth of Massachusetts,” she wrote:
“God save the common pal U S Spe Manor Gen, or til pat. Since Lord, or
no prime in Hear to the God Tel. Ho. n and or Mabel, or gal.” After
this, she took paper and wrote meaningless scrawls, saying that it was
Japanese writing. There was much motor restlessness with
distractibility, pointing and grimacing, mimicking the actions of those
about her.

Death occurred from exhaustion, and the case might not have been
regarded as unusual except for the autopsy, which showed a peculiar
brain lesion, described below. The point of greatest interest in the
case was the fact that syphilis is, although not proved to exist by
laboratory tests, beyond question a factor in the case. Although the
woman had given birth to a normal child, who is still alive, yet in the
period of a few years her breasts had atrophied, her hair had
disappeared from the axilla and from the pubes; varicose veins had
developed in both legs. Whereas there was little or no fat over the
chest or back, the omentum and mesentery were very plentifully supplied
with fat. It is probable, then, that we are dealing with a case of
exophthalmic goitre somehow of syphilitic origin. The brain lesion is
consistent with this hypothesis.

    =Autopsy=, March 3, 1907. Four hours post mortem.

    Body length, 165 cm. Body of a well developed and well nourished
    young woman. Lividity in dependent parts. Purplish discoloration of
    left thigh to knees. Skin rough and scaly. Petechial eruption over
    chest. Neck thick, protrudes anteriorly. Varicose veins over upper
    parts of calves on both legs. Eyes protruding, not covered entirely
    by lids. Pupils equal, dilated. Subcutaneous fat very deep over
    lower part of body. Very little fat over chest and back. Breasts are
    very small, apparently atrophied. Normal amount of hair on head,
    slight amount over pubes. Axillary hair absent. Fat on section of a
    light yellow color. Omentum extends to pubes, plentifully supplied
    with fat. Large amount of mesenteric fat. Appendix normal.
    Intestines smooth and glistening. Slightly injected. No fluid in
    peritoneum. Uterus small, retroverted.

[Illustration:

  Cortical hemiatrophy—A, relatively normal right precentral (“motor”)
    cortex; B, atrophic left precentral.

  Note in B:

  1. Absence of giant pyramids of Betz (corticospinal, upper motor
    neurones).

  2. Superficial (subpial) condensation of tissues with sclerosis
    (gliosis). The tissues in all areas examined _on the left side_
    yielded this effect.
]

    HEAD: HAIR in good quantity. SCALP normal. CALVARIUM shows diploë.
    DURA MATER over left cerebral hemisphere inseparably adherent to
    calvarium, over right hemisphere normal. Arachnoidal VILLI
    moderately developed. PIA MATER shows injected veins, notably in the
    sulci of the right hemisphere. Pia mater everywhere thin and clear.
    VESSELS at base of normal appearance.

    BRAIN weight 1180 grams. Spread on a board, the right hemisphere
    tends to flatten so that it measures 1.5 cm. more from side to side
    than its fellow. Besides more marked venous injection, the right
    hemisphere shows also flatter and slightly more plastic
    convolutions. The posterior poles of the hemispheres are a little
    firmer than the parts anterior. The orbital and hippocampal gyri on
    the right side are a little firmer than the surrounding parts. On
    section the gray and white matter shows no lesions, excepting the
    slight plasticity of the tissues at large on the right side and a
    well marked induration, with retraction under the knife, of the
    occipital and hippocampal white matter. The basal ganglia of the
    left side are normal. On the right side a sagittal section
    demonstrates a rounded area of induration, with ill-defined borders,
    measuring perhaps 1.5 cm. from above downwards by 2 × 2 cm.,
    situated largely in the lenticular nucleus and involving the greater
    portion of the globus pallidus, a small segment of the putamen below
    and behind and the regionary part of the anterior commissure with
    surrounding tissues. The most striking feature of this lesion is the
    occurrence in the middle of a cluster of vacuoles or cystic clefts,
    with smooth pale interiors, ranging from pinhead to 0.25 cm. or even
    0.5 cm. in greatest diameters. There are six to eight clefts to a
    surface of section. The color of the lesion differs little from that
    of the globus pallidus itself, but the tissue is a trifle
    translucent. It is impossible to demarcate the lesion with the eye.
    Induration is demonstrable several mm. beyond the visible part of
    the lesion. The consistence of the lesion slightly surpasses the
    usual consistence of the olivary bodies.

    CEREBELLUM, PONS and BULB weight 165 grams. Cerebellar tissue a
    trifle more plastic than usual. The right olive is not so prominent
    as usual.

    =Note.= THYROID: Weight 125 grams. Both lobes and isthmus enlarged.
    One lobe more than the other; lobe on one side measuring 6 × 4 cm.

                            Anatomical Diagnoses

    Enlargement of thyroid gland.

    Exophthalmos with dilated pupils.

    Fatty degeneration of thoracic muscles.

    Slight aortic sclerosis.

    Dilatation of right heart.

    Hypertrophy of left ventricle.

    Slight tricuspid endocarditis.

    Bicuspid aortic valve.

    Hypostatic pneumonia.

    Acute and chronic splenitis.

    Fatty liver (central necroses?).

    Acute nephritis.

    Chronic gastritis.

    Small breasts.

    Axillary hair absent.

    Petechial eruption of chest.

    Varicose veins.

    Chronic external adhesive pachymeningitis of left side.

    Moderate swelling of right hemisphere with venous injection.

    Slight occipital gliosis of both sides.

    Slight gliosis of orbital and hippocampal gyri of right side.

    Sclerosis with atrophy of occipital and hippocampal white matter of
    right side.

    Gliotic lesion (1.5 × 2 × 2 cm. of right lenticular nucleus
    involving anterior commissure).

    1. Was the exophthalmic goitre in Carrie Pearson due to syphilis?
      Unfortunately we have no clear proof that Carrie Pearson was
      syphilitic. She was stated to have been syphilitic by the
      physician who treated her before her commitment to Danvers
      Hospital. There is, however, no proof of syphilis, inasmuch as the
      patient died in the pre-Wassermann period.

    2. Is the thalamic lesion probably syphilitic? No lymphocytosis or
      plasmocytosis characterizes the lesion, which is the only lesion
      of the sort in the Danvers collection. It would not do to call a
      lesion syphilitic just because it is _sui generis_. In any event,
      the clinical analysis of the case faced the claim of syphilis as
      an actual factor in the patient’s life and as a possible factor in
      the goitre.


  =It is well known that the ARGYLL-ROBERTSON PUPIL is characteristic
  of the so-called “PARA-SYPHILITIC DISEASES” (“general paresis” and
  “tabes”); does this sign occur in other neurosyphilitic conditions?=


=Case 54.= Julius Kantor was a shoemaker of 35 years, who came to the
hospital for treatment because his family physician had found a positive
W. R. in Kantor’s blood serum. He had had a cough for a number of years,
and during the last year a little blood had been found in the sputum;
whereupon Kantor had been placed under active anti-tuberculosis
treatment. The enterprising family physician had found the positive W.
R. in the first days of his treatment for tuberculosis. There was, in
fact, a history of a chancre nine years before, which had not been
followed by any secondary or tertiary symptoms, and which had been but
scantily treated.

There were no mental symptoms.

Kantor was =physically= fairly well developed and nourished. There were
a few piping râles in the left upper chest, both in front and back, and
also a slight dulness with increased vocal and tactile fremitus. No
tubercle bacilli, however, could be found on repeated sputum
examination.

=Neurologically=, the pupils were myotic and both showed the
Argyll-Robertson reaction. There were no abnormal reflexes whatever, and
there was neither ataxia nor speech defect. Not only the blood but also
the spinal fluid W. R. proved to be positive; there was a marked
increase in the albumin and globulin; there was a gold sol reaction of
the syphilitic type, and there were but three cells per cmm.

    1. In view of the headache in case Kantor, what other causes of
      headache are to be considered? It is certain that irritations of
      the dura mater can produce headache, and the physiological
      observation of the sensitiveness of the membranes and the
      non-sensitiveness of the brain substance is an ancient and
      classical observation. Internal hemorrhagic pachymeningitis
      produces severe headache. The relations of this disease to trauma,
      to arteriosclerosis, and possibly to syphilis (alcohol perhaps
      should also be considered) in certain instances have not been
      entirely cleared up. Syphilitic headaches are, according to
      Lewandowski, dependent also upon a dural affection or upon a
      periosteal affection. The headaches of brain tumor are also
      commonly related to dural conditions, either directly due to the
      pressure of the tumor itself, or indirectly to the heightened
      intracranial pressure consequent upon the tumor. It is clear that
      the tension under which the dura mater lies is not always
      localized in the region of a brain tumor or a syphilitic lesion.
      Head has claimed that brain tumor produces headaches of two kinds,
      according to whether the disease affects the dura mater or is
      dependent upon an increase of pressure in the brain. It does not
      appear that the pia mater has any relation to headaches, but
      meningitis, in which the inflammation is confined to the pia
      mater, is nevertheless associated with headache; the headache is
      here supposed to be due to the increase in brain pressure, and
      thus actually to an effect wrought upon the dura mater. Vasomotor
      disorders and various types of cephalic hyperemia are thought to
      produce a kind of headache, but Lewandowski calls this kind of
      headache somewhat in question. Reflex headaches are stated to be
      produced indirectly by a process of radiation from interior
      lesions in the brain. There are certain headaches called nodal
      headaches (_Schwielen-Kopfschmerz_). Hypermetropia, caries of the
      teeth, adenoids, and diseases of the nose and axillary cavities,
      to say nothing of thoracic and abdominal diseases, are also
      counted among conditions that may produce headaches. In this
      connection, Head has claimed differential zones of headache
      corresponding to certain diseases.

      The brain itself may produce headache through intoxications,
      through conditions produced by a variety of diseases; may follow
      neuroses. Alcohol may produce headaches in some persons even when
      it is taken in very small doses. Certain uremic cases yield
      headaches, as do also gouty and chlorotic conditions. According to
      Lewandowski, the headaches of arteriosclerotics are due possibly
      to vasomotor disturbances in the membranes, or one may think of
      nutritive cerebral disorders. A peculiar form of headache is that
      of fatigue after mental work, allied to which is the neurasthenic
      headache; constitutional headaches have been assumed to occur, to
      say nothing of hysterical headaches. There remains also the
      important question of migraine, for which a vasomotor explanation
      has been proposed.

    2. Was Kantor suffering from tuberculosis of the lungs? The
      hypothesis of lung syphilis ought certainly to be very seriously
      considered. Upon repeated sputum examination, no tubercle bacilli
      have yet been found.

    3. Is Kantor a case of general paresis? In the absence of mental
      symptoms, and in consideration of the mildness of the reactions,
      it is certainly not easy to make the diagnosis of general paresis.
      However, the diagnosis of tabes dorsalis is not justified either.
      Accordingly, we may answer our question: whether the
      Argyll-Robertson pupil occurs in other neurosyphilitic diseases,
      by pointing out that in the case of Julius Kantor, as in the case
      of Henri Lepère (105) and Frederick Stone (106), the
      Argyll-Robertson pupil has been found in syphilitic conditions
      that are neither typically paretic nor typically tabetic.


  =Does the Argyll-Robertson pupil necessarily indicate
  neurosyphilis?=


=Case 55.= Daniel Falvey, 44 years of age, was an almshouse transfer to
the Danvers State Hospital in the year 1904, when the principle of state
care was adopted in Massachusetts. As in most of the almshouse transfers
of that day, little could be discovered as to antecedents. He had been a
mill-worker from the time of his immigration in 1890, at 30 years of
age. He had been somewhat alcoholic. There was a shock some 17 months
before his death, which occurred about seven weeks from the date of
transfer.

Not only was he unable to walk unsupported, but when supported there was
a slight dragging of the left leg and the gait was noted to be somewhat
propulsive. The tongue and hands were tremulous, and the left grasp was
somewhat weaker than the right. Both knee-jerks were increased although
neither more than the other. There was no sensory disorder.

Although but 44 years of age, Falvey presented the appearance of a much
older man. His heart was somewhat enlarged and there was a degree of
peripheral arteriosclerosis. On the whole, no special attention was
attracted to this case clinically and he was regarded as an example of
arteriosclerotic dementia, like many another among the transfers.
However, we owe to Dr. H. M. Swift the important observation of the
Argyll-Robertson pupils. The case was studied long before the Wassermann
method was available, and is here reported merely to call attention to
the fact that the stiff pupils may have other neural origin than
neurosyphilis.

The autopsy material in the case was worked up by one of the
authors.[11] The autopsy had been performed by Dr. A. M. Barrett, who
found on section through the brain stem at the anterior border of the
pons a mass springing from and continuous with the pineal gland, lying
in the third ventricle and the aqueduct of Sylvius. Upon further study,
this mass was found to begin posteriorly in the pineal body itself, from
which the mass could hardly be told in the gross except by an injected
border.

This mass proved upon microscopic examination to be a psammoma, which
histologically resembled a glioma rather than a sarcoma. Throughout the
mass there was a variable content of fibrillary intercellular substance
having the histological reactions of neuroglia fibrillæ. The
histological details (mitosis, large giant cells with multiple nuclei,
etc.) do not here concern us. We deal with a neoplasm springing from the
pineal gland growing on the posterior half of the third ventricle, the
anterior orifice of the aqueduct of Sylvius, and the space between the
velum interpositum as far back as the posterior corpora quadrigemina.
There is no evidence in the body of old syphilis; although it is
possible that the stiff pupils were neurosyphilitic, it seems probable
that they were related to the pineal tumor. At all events, there are in
the literature evidences that the pineal-quadrigeminal group of tumors
and other lesions may bring about pupillary disturbances. On this
account, we here include the case. The tumor hardly led to an error in
diagnosis since neither neurosyphilis nor brain tumor was at all
expected clinically.

    1. Can alcoholism produce identical results? See Case Murphy, (60),
      one of alcoholic pseudoparesis.

    2. What is the nature of stiff pupils? A pupil is called stiff in
      the sense of the Argyll-Robertson pupil if it fails to react to
      illumination either of itself or of the other eye and at the same
      time if it reacts properly in convergence and accommodation. Of
      course the stiffness of a blind eye must not be regarded as an
      Argyll-Robertson pupil. In a case of right-sided Argyll-Robertson
      pupil, therefore, the left pupil reacts properly both to direct
      illumination of itself and to illumination of the right eye, but
      the right eye fails to react to illumination of either eye. Such
      an Argyll-Robertson right pupil will remain of the same width both
      in darkness and in light. Clinicians agree that the
      Argyll-Robertson is diagnosticated rather too frequently than too
      seldom, and this by reason of the fact that a sluggishness of
      light reaction is interpreted as stiffness. The sign, as is well
      known, has come to be regarded as almost pathognomonic of tabetic
      or paretic neurosyphilis. Nonne, however, has found among 510
      cases of alcoholism, nine instances of Argyll-Robertson pupil and
      19 cases of sluggish light reactions. The pathological anatomy of
      this sign is still doubtful although a number of schematic
      accounts are available; among hypotheses, one may think of an
      elective effect of the tabetic or paretic degeneration upon reflex
      collaterals. The explanation would then resemble that for absent
      knee-jerks and kindred reflex disorders. We should then
      hypothesize a loss of the finer processes of the terminal
      arborizations about the cells of the nucleus of sphincter nucleus
      iridis. However, the situation of the sphincter iridis has not yet
      been absolutely determined.

      When a pupil is said to be entirely stiff it means that it reacts
      neither to light nor accommodation. This condition not
      infrequently follows the partial stiffness or Argyll-Robertson
      reaction.

    3. Is the Argyll-Robertson pupil more tabetic than paretic? This has
      been claimed at times, but in point of fact, the Argyll-Robertson
      pupil is very frequent in paresis, and so also are posterior
      column changes. According to statistics of Bumke, 36% of tabetics
      fail to show the Argyll-Robertson pupil, and 38% of paretics.
      When, however, finer methods, such as those standardized by
      Weiler, with photographic records, are employed, the number of
      cases without at least a tendency to the Argyll-Robertson pupil
      becomes much smaller.

      In connection with the important question as to the classical
      Argyll-Robertson pupil and pupillary sluggishness to light, it may
      be inquired what are the ocular signs in neurosyphilis? Joffroy
      has tabulated the signs in 300 general paretics as follows:

                           Sign.                           No. of  Per
                                                           cases. cent.
 Alterations of light reflex                                  235     78
 Inequality                                                   205     68
 Abolition of light reflex (bilateral or unilateral)          156     52
 Abolition of light reflex (bilateral)                        133     44
 Irregularity of pupil                                        117     39
 Irregularity of both pupils                                  109     36
 Diminution of light reflex                                   108     36
   ditto (bilateral)                                           79     26
 Alteration in accommodation reflex                            79     26
 Diminution of accommodation reflex                            52     17
 Mydriasis                                                     41     13
 Myosis                                                        40     13
 Diminution of light reflex (unilateral)                       35     11
 Abolition of accommodation reflex                             35     11
 Diminution of accommodation reflex (bilateral)                29      9
 Abolition of accommodation reflex (bilateral)                 26      8
 Diminution of accommodation reflex (unilateral)               23      7
 Fundus changes                                                21      7
 Vascular changes                                              16      5
 Abolition of accommodation reflex (unilateral)                12      4
 Paresis of the third nerves                                   10      3
 Ptosis                                                         9      3
 Irregularity of one pupil                                      8      3
 Nystagmus                                                      7      2
 Visual acuity lost                                             7      2
 Atrophy of disc                                                6      2
 Total blindness                                                5      2
 Paralysis of the fourth nerves                                 1      1


  =Can neurosyphilis exist in the absence of positive findings in the
  spinal fluid?=


=Case 56.= There was no great difficulty in setting up a diagnosis of
general paresis in the case of James Burns, a mechanic of 31 years of
age, who came voluntarily to the Psychopathic Hospital for treatment.
The point in Burns’ case was that the spinal fluid proved entirely
negative in all respects despite the fact that the serum W. R. was
positive, and despite the following facts of history and mental
examination.

The patient claimed syphilitic infection seven years before, namely, at
24 years of age, and also claimed that he had infected his wife, who was
in fact at the time undergoing antisyphilitic treatment. He complained
of insomnia, worry, depression, hypersensitivity to noises (such as
those made by his own children), thoughts of suicide, and amnesia. The
amnesia, however, might be regarded as subjective since our tests failed
to show amnesia. Nor was there any diminution in arithmetical ability.
Despite the patient’s claim that he had been “way off in his way of
thinking,” there appeared to be no delusions. Beyond a certain
flightiness in conversation, we could hardly get any evidence of
psychosis unless of the neurasthenic order.

=Physically=, however, the left pupil failed to react to light though it
was found to react to distance, and the right pupil exhibited a
diminution of its reaction to light. There was no ataxia of gait, yet
there was a complete Romberg reaction. There was a moderate tremor of
the hands and of the tongue. Otherwise there were no reflex disorders
upon systematic examination, nor was there any demonstrable disorder in
the rest of the physical examination.

    1. What is the diagnosis in the case of James Burns? On the whole we
      agree with Nonne, that negative spinal fluid findings (of course,
      in the absence of treatment) preclude the diagnosis of general
      paresis. The symptoms might possibly be explained, however, by
      means of a localized syphilitic involvement of the cerebrum, no
      cells or products of inflammation having penetrated to the spinal
      fluid. According to Head and Fearnsides, this condition may be
      found especially in the anterior or middle fossa. Accordingly,
      going upon these views of Nonne and of Head and Fearnsides, we
      should be entitled to make, perhaps, a diagnosis of cerebral
      syphilis.

    2. What is the significance of the Argyll-Robertson pupil in James
      Burns? Nonne states that if one follows cases with
      Argyll-Robertson pupil over a sufficient period of years, they one
      and all eventuate in active symptoms of cerebrospinal syphilis
      (not necessarily of the cortical type), and this despite the fact
      that the pupillary change may have been present a number of years
      before any other symptom had developed.


  =Neurosyphilis (“DISSEMINATED ENCEPHALITIS”) within seven months of
  initial infection. Autopsy.=


=Case 57.= We borrow the main features of a remarkable case examined at
the Danvers State Hospital clinically by Dr. H. W. Mitchell and reported
elaborately by Dr. A. M. Barrett. This case, whom we shall call John
Summers, acquired syphilis at about the end of the third week in May,
1902, and consulted a physician on June 12, at which time a
characteristic initial lesion of syphilis was plain. Summers was
excessively alcoholic at times and was not seen by a physician again
until July 2, just after an alcoholic debauch. At this time there was
ulceration of the primary lesion, and a papillary eruption had developed
over the arms, chest, abdomen, and legs. Mercurial treatment and mixed
treatment were given. Arthritis occurred but disappeared with increased
dosage.

About six months after infection, the patient developed severe
headaches, hardly controllable by treatment. Amnesia and a certain
stupidity, with neglect of personal habits, and even of eating,
developed, whereupon Summers was admitted to the Danvers Hospital,
December 11, 1902. He weighed 124 pounds, was extremely feeble, with
dull and expressionless face, coarse purposeless movements of arms; left
pupil larger than right; right external strabismus and ocular ptosis;
increased knee-jerks, crossed adductor reflex, coarse tremors of arms
and hands; and extreme clouding of consciousness. It was doubtful
whether the pupils were stiff to light or not.

The patient died on the ninth day, December 18, in a state of coma.
After admission, his stupor had become more marked; there had been
incontinence of urine and fæces, and the patient could be aroused only
by loud tones. Difficulty in swallowing had developed; the right-sided
ptosis had become more marked, and muscular twitchings had developed on
the right side. When the left leg was pinched, there was twitching of
the left leg and arm. There was slight spasticity of the right arm and
leg. An examination upon the day of death definitely showed a lack of
reaction of the pupils to light.

[Illustration:

  1. Exudate in pia mater—mononucleosis.
]

[Illustration:

  2. Superficial (subpial) cellular reaction of neuroglia tissue
    (expanded cell bodies).
]

[Illustration:

  3. Cellular gliosis of deeper layers of cortex. Apparent increase in
    capillary supply, possibly relative to loss of neural elements.
]

 Case 57. Neurosyphilis (“disseminated syphilitic encephalitis” of A. M.
 Barrett), fatal seven months from initial infection. (Photographs by A.
                               M. Barrett.)

Dr. Barrett was able to find in the literature a case of Bechterew which
histologically resembled his own case, but though in the instance
reported by Bechterew the first symptoms developed within the year
following infection, death did not occur until two years later.

In view of a total duration of symptoms clearly not over seven months,
it is interesting to inquire how far microscopic brain changes could
have proceeded. Neither calvarium nor dura mater showed changes. There
was a slight haziness of the pia mater over the convexity, but the pia
mater over the base (especially below the cisterna and from thence
spreading out over the pons and into the fissure of Sylvius) was not
only hazy but definitely thickened and hyperæmic. The thickening was
most marked about the root of the right third nerve (corresponding with
the eye findings in life). There was also a macroscopic thickening of
the left Sylvian artery. Section of the brain showed nothing abnormal
except a small area among the pyramidal fibres of the right side of the
pons, where there was a single hemorrhagic area about 7 mm. in diameter
around which there were small punctiform hemorrhages. (Compare
twitchings of left leg and arm upon stimulation of left leg, and note
also the muscular twitchings and slight spasticity of right leg and arm
noted just before death.) This case was examined and reported upon in
1905. We learn from Dr. Barrett that a re-study of the case with modern
methods has failed to demonstrate a spirochetosis.

The meninges show infiltration and destructive and proliferative changes
of the blood vessels. Condensed extracts from Dr. Barrett’s full report
follow:

    There were local variations in the severity of the meningitis. The
    sulci showed the most marked infiltration. The slighter degrees of
    exudation were made up largely of lymphocytes with a few plasma
    cells, occasionally large mononuclear cells, and rarely a
    polymorphonuclear leukocyte. Where the exudation was more extensive,
    the large mononuclear cells became more common and the
    polymorphonuclear leukocytes increased in number. The large
    mononuclear cells were often phagocytic, containing from one to six
    leukocytes. The exudate was always most abundant about the blood
    vessels. The plasma cells were always most numerous in the
    adventitia of the veins, here greatly outnumbering the leukocytes.
    The polymorphonuclear leukocytes were relatively infrequent except
    where there were necrotic areas, which areas were usually continuous
    with an infiltration of a vessel wall.

    As to vascular changes, the media was not often involved, nor was
    the adventitia so often affected as the intima. Such lesions as
    appeared in the intima and adventitia were infiltrative rather than
    proliferative. The elastica of the blood vessels proved to show but
    slight changes.

    A characteristic change was the endarteritis,—of a focal nature with
    a few large mononuclear and lymphocytic cells pushing the intima
    inward at the edge of a lesion. In the more marked portion of the
    focal process, the thickness of the intima was greatly increased by
    proliferation. Great numbers of large mononuclear cells could be
    seen between the intima and the elastica. About these cells and
    interlacing among the other elements of the proliferating tissue was
    an excess of connective tissue fibres.

    The meningeal veins were more often diseased than the arteries;
    there was adventitial infiltration with lymphoid and plasma cells;
    sometimes the vein walls had become necrotic and infiltrated with
    polymorphonuclear leukocytes.

    It will be remembered that the left Sylvian artery was grossly
    thickened, and microscopic section of this vessel showed a partial
    thrombosis.

    The brain showed diffuse and focal changes. The _diffuse_ process
    was one of nerve cell degeneration and proliferative changes in the
    neuroglia and blood vessels, and no section of the many examined
    proved to be free from such changes, although in the majority of
    instances, these diffuse changes were slight. The cortical layers
    showed more of these diffuse changes than did the white substance.
    Barrett considered that the glial cell changes were more delicate
    indicators of the cortical changes than the nerve cell changes. He
    found rod cells, satellitosis, superficial gliosis, and a large
    gamut of changes in the neuroglia. There were two rather
    characteristic nerve cell changes: a shrinkage change going on to
    almost complete destruction, and a type of cell swelling, also
    apparently proceeding to complete destruction.

[Illustration:

  4. Arteritis of pia mater.
]

[Illustration:

  5. Focal vascular lesions.

  Case 57. Seven months from infection. “Disseminated syphilitic
    encephalitis,” Barrett. (Photographs by Barrett.)
]

[Illustration:

  Paretic neurosyphilis (“general paresis”)—cerebral atrophy, _without_
    meningitis. Therapeutics cannot hope to restore lost tissue.

  Duration. 3 years from beginning of well marked symptoms; 6 years from
    beginning of obvious symptoms; 12 years from a so-called “nervous
    prostration.”
]

    Among _focal_ changes, there were four main types: Areas of
    encephalitis, having the general appearance of granulation tissue,
    areas of simple necrosis or softening, apparently directly related
    to vascular changes near by, hemorrhages, and certain foci regarded
    as gummatous.

    Save for pial infiltration and a few vascular changes, there was
    very little change in the medulla and spinal cord. There was a
    hypertrophic gliosis of the margin of the medulla and cord
    throughout, and a focal lesion of well marked gliosis at one point
    in the bulb. There were no fibre degenerations in the medulla or
    cord, nor were there any coarse fibre degenerations in the cortex
    itself except in one locus, the left gyrus rectus. This case is of
    peculiar value in showing to what extent lesions may proceed in a
    period of six to eight months after primary infection.

Of course the case is in one sense entirely atypical. The lesions were
not confined to the nervous system. Aside from the maculo-papular
eruption and ecchymosis of the skin, there was a diffuse hemorrhage of
the inner half of the conjunctiva of the left eyeball, a small
hemorrhagic focus in the mitral valve, a caseous nodule, one cm. in
diameter, in the apex of the left lung whose tuberculous or syphilitic
nature is left in doubt; a broad milk-colored patch of thickening of the
capsule of the liver. It is to be noted that there were no gross lesions
of the aorta.


  =On the classical assumption that PARETIC NEUROSYPHILIS (“general
  paresis”) is a fatal disease, is there a disease PSEUDOPARETIC
  NEUROSYPHILIS (“pseudoparesis”) which may recover or pursue a long
  course like that of a case of diffuse neurosyphilis (“cerebrospinal
  syphilis”)?=


=Case 58.= Peter Burkhardt had been an efficient highway inspector, but
in his forty-fifth year he had begun to be unable to do his work
satisfactorily. His wife had become somewhat afraid of him. He had had
somnolent spells in his chair and had squandered money. The mental
symptoms had lasted for some six months, but had become more marked
during the month preceding admission. Burkhardt would at times fail to
recognize his friends.

The general =physical condition= of Burkhardt was very good. The pupils
were irregular and reacted sluggishly both to light and to
accommodation. The knee-jerks and ankle-jerks were absent. There were no
other neurological disorders upon systematic examination. There was a
speech defect. =Mentally=, little could be determined except a certain
sluggishness.

History and physical examination at once suggested general paresis. The
serum W. R. was doubtful, but the spinal fluid reaction was positive, as
was the gold sol reaction (which was “paretic”); the globulin and
albumin were greatly increased; there were 48 cells per cmm.
Antisyphilitic treatment, consisting of salvarsan twice a week and
potassium iodid by mouth, was followed by a rapid mental improvement.
After two months, Burkhardt was discharged apparently normal, with all
the blood and spinal fluid tests negative. He has been taken back into
the highway service.

    1. What is the proper definition of pseudoparesis? Fournier termed
      pseudoparesis certain cases that looked like paresis but were not
      syphilitic in origin. Of these cases the most characteristic group
      is that of alcoholic pseudoparesis. It is clear that there will be
      no difficulty in the definition of a disease pseudoparesis whose
      entity is presented in the adjective that precedes the term
      (_e.g._, alcoholic pseudoparesis). According to this usage, a case
      of pseudoparesis would be one in which the symptoms and possibly
      some of the signs somewhat resemble the symptoms of paresis itself
      but for which another etiology could be fairly established.

    2. Are there any cases of syphilitic pseudoparesis? We are of the
      opinion that the term should be dropped. It is true that there are
      cases which clinically look like general paresis and exhibit the
      appropriate laboratory signs of general paresis but seem to differ
      from paresis in their course even when they receive no treatment
      whatever. In the present phase of doubt as to the classification
      of paretic and non-paretic forms of neurosyphilis, it seems to us
      of doubtful utility to characterize a case as pseudo simply
      because it differs in its course, particularly as the literature
      has always duly recognized that a number of cases of general
      paresis have had long courses and sometimes very long remissions.

      There is also another group of cases that have been termed cases
      of pseudoparesis, namely: certain cases of neurosyphilis which
      clinically look like general paresis and seem to be following its
      classical course but are interrupted by treatment. Here again it
      seems to us doubtful whether the designation pseudo should be
      attached to this group of cases, particularly while the whole
      therapeutic question in the paretic group of neurosyphilis cases
      remains _sub judice_. Accordingly we are tempted to include in the
      group of paretic neurosyphilis cases that either get well of
      themselves, or get well under treatment, or pursue a very long
      course, or are subject to very long remissions. But we make this
      decision in terminology without prejudice to the therapeutic
      question and it is open to any critic to throw these cases into an
      atypical non-paretic group of neurosyphilis cases.

    3. How shall we explain the absence of ataxia of case Burkhardt when
      knee-jerks are absent and when, therefore, we are entitled to
      conclude a certain degree of spinal disease? As stated in
      connection with case Sullivan (16), the absence of knee-jerks is
      not a warrant for terming a case—paresis of the tabetic form. The
      fact is that the lesion in paresis tends to be intraspinal, just
      as the higher brain lesions tend to occur within the brain
      substance. The meninges are relatively spared both within the
      cranium and within the spinal canal. The characteristic
      degeneration of posterior nerve roots which we find in tabes
      dorsalis is not necessarily found in general paresis even when
      there are somewhat extensive spinal lesions. Accordingly the
      absence of sensory returns by way of the posterior nerve roots
      which characterizes tabes dorsalis is not necessarily a phenomenon
      of general paresis. The mechanism by which the knee-jerks are lost
      depends upon histological detail. They may be lost when under
      tabetic conditions the posterior roots are severely diseased and
      when under paretic conditions only intraspinal collaterals or a
      small portion of fibres are affected. The whole question hinges
      upon where and to what degree the various reflex arcs are cut in
      the disease. The tabetic phenomena are, as so commonly stated,
      intradural; that is, the sensory or gangliospinal neurones at
      certain levels are affected all the way in from the points at
      which they pierce the dura mater. The affection of these and other
      neurones in general paresis is an intraspinal and parenchymatous
      affection.


  =Neurosyphilis; auditory hallucinations; ideas of persecution;
  attacks of excitement. SYPHILITIC PARANOIA (Kraepelin)?=


=Case 59.= Bridget Curley was a case that was discharged from the
Psychopathic Hospital, recovered, after 26 days in hospital. The
symptoms so resembled those of alcoholic hallucinosis that the diagnosis
was made despite the fact that the patient consistently denied the use
of intoxicants. There was, in fact, no proof that she drank alcohol. The
case was, however, not clearly one of alcoholic hallucinosis or of any
other well-defined form of mental disease. A provisional diagnosis of
manic-depressive psychosis, manic phase, had, in fact, also been made.

The illness had begun with depression and inactivity, Bridget’s friends
accounted for these conditions on the ground that a lover had departed
for Ireland. A few days after the depression began, Bridget became dizzy
and refused to give a boarder his breakfast, stating that she had lost
her memory and had begun to hear bells ringing and people talking. She
then became greatly excited and was brought to hospital, where the
prolonged baths quieted her.

It seems that Bridget had had stomach trouble and headaches at the top
of her head or sometimes in her temples. =Physical examination= showed
the left pupil to be larger than the right, a slight tremor of the lips,
a slight systolic murmur at the apex, slightly irregular pulse, and
moderate edema of ankles. The blood serum was negative to the W. R., but
lumbar puncture was executed and the fluid showed a positive W. R.

The patient was tested by the Binet and other methods, and although 35
years of age, seemed to be by the mental tests hardly over 11 years old.
She was inclined to be feverish, somewhat restive, and pugnacious;
rather slow of speech, sometimes refusing to answer and grimacing. Her
pugnacity was, however, easily controllable, and the excitement was
largely at night. This excitement subsided rapidly in the course of a
few days.

    1. What is the diagnosis in this case? The following diagnoses and
      suggestions for diagnosis were made at the staff meetings:

      Unclassified mania.
      Manic-depressive psychosis, manic phase.
      Toxic delirium.
      Dementia praecox.
      Bacterial infection of the brain.
      Unclassified delirium.
      Acute delirium.
      Infectious psychosis.
      Acute confusional psychosis.
      Psychopathic personality by use of alcohol.
      Mental deficiency with atypical mental state.
      Syphilitic paranoia.

    2. Is this a case of syphilitic paranoia? The so-called syphilitic
      paranoia of Kraepelin is a rare and uncertain type of syphilitic
      mental disease. Delusions and hallucinations are prominent. As a
      rule, the onset is stated to be slow and insidious, or at any rate
      there are a variety of indefinite prodromata. Jealousy is a
      prominent feature, sometimes attended with marked sexual
      excitement. Auditory hallucinations and ideas of persecution are
      particularly in evidence. The most striking feature in Kraepelin’s
      group was a sudden occurrence and equally sudden disappearance of
      violent excitement, with or without external cause. Thus, an
      excitement would be produced by a few words spoken, and
      immediately after, the phase of excitement would pass and the
      patient would become entirely friendly and accessible once more,
      as if nothing had happened. About half of Kraepelin’s cases showed
      a positive serum W. R. He does not report lumbar puncture
      findings, and grounds the existence of disease upon certain
      autopsied cases. The speech and writing disorder of paresis as
      well as the characteristic disorientation for time and muscular
      weakness of general paresis were absent in the group. It appears
      that most cases of the group have hitherto been placed in dementia
      praecox.


  =The clinical symptoms of CHRONIC ALCOHOLISM are sometimes largely
  identical with those of PARETIC NEUROSYPHILIS (“general paresis”):
  differentiation by means of the laboratory findings.=

To demonstrate this proposition, the cases of Francis Murphy (60) and
David Collins (61) are in point, being sharp foils to one another.


=Case 60.= A laboring man about 44 years of age was brought to the
Psychopathic Hospital one summer day, in a stupor. This patient, Francis
Murphy, had been at his regular work as axeman in the Park Service, when
he suddenly fell in a heavy convulsion. He was carried to a general
hospital, still in convulsions, and ether was administered to quiet the
movements. The convulsions shortly ceased, but the patient’s
consciousness failed to clear; hence his transfer to the Psychopathic
Hospital.

Here he remained much disturbed and was placed in a room with a mattress
on the floor. On this mattress he would crouch on all fours for a
considerable time, looking fixedly downward as if at an object on the
floor, unresponsive to questions but compliant with efforts to place him
on his back. He gave the impression of daze and either disorientation or
confusion.

Within twenty-four hours the patient became more tranquil and
consciousness became clearer, but the patient was at a loss to bring to
memory either recent or remote events. However, he replied to questions,
giving some different story each time he was approached. Curiously
enough, the patient seemed very contented and good-natured and would
even laugh foolishly at times, saying that he felt fine and all ready to
go out to work.

The general impression conveyed by Francis Murphy at once suggested the
possibility of neurosyphilis. Convulsions, perhaps initial in middle
age, with a post-convulsive stupor, followed by a partial clearing up,
with persistent amnesia and a suggestion of fabrications with euphoria,
bore out the suggestion.

The =physical examination= strengthened the impression of neurosyphilis.
Well developed and nourished, florid, with a manual tremor and sweating
of the palms, the patient was in general without physical symptoms.
=Neurologically=, however, whereas the left pupil was larger than the
right and reacted properly to light, the right pupil was a bit
contracted, somewhat irregular, and either reacted not at all to light
or very slightly so (reacting perfectly to accommodation). The
knee-jerks could be obtained only with reinforcement, and several other
reflexes could not be elicited (triceps, radial, ulnar, periosteal,
Achilles, umbilical). Moreover, the heel-to-knee test was poorly
performed; some of the common tests phrases were very poorly repeated;
there was marked tremor in writing; and the paragraphia seemed to be not
merely peripheral, for syllables were left out in words and ordinary
words spelled incorrectly (psychographic disturbance).

We do not care here to insist that the right pupil was really an example
of the Argyll-Robertson phenomenon since the slightest tinge of doubt is
important if a positive diagnosis is practically equivalent to asserting
syphilis. Practically, however, the right pupil was regarded as an
Argyll-Robertson pupil under hospital conditions (flash-light reaction).
Argyll-Robertson pupil, areflexia, speech disorder, writing disorder,
memory disorder, conduct disorder, and euphoria, all with a history of
convulsions, certainly warranted the tentative diagnosis of
neurosyphilis.

As usual, resort was made to the W. R. in the serum and in the spinal
fluid. One of the first results to come through from the laboratory was
the absence of globulin, normal albumin, negative gold sol reaction, and
a cell count of two cells per cmm. in the spinal fluid. Later the _W.
R.’s_ were returned _negative_ for blood and spinal fluid.

In the meantime, an illuminating change had occurred in the patient, for
two days later,—three days after the first convulsion in the park,—the
patient had apparently quite recovered; his consciousness became nearly
clear; he could remember every event up to the time of the convulsion,
and his memory came back in appropriate degree for both remote and
recent events.

The patient, it appeared, had for some time been drinking more and more
heavily. In recent days, he had been taking five or six whiskeys and a
half dozen beers daily on the average, and often much more. About ten
years before, the patient narrated, there had been a convulsion at a
ballgame, and this convulsion the patient himself called a “rum fit.”

Here, then, is a case of ALCOHOLIC PSEUDOPARESIS. Without the W. serum
test and without the spinal fluid examination, it is probable that the
diagnosis of general paresis might have clung to the patient for some
time on account of the apparent Argyll-Robertson pupil, which had to be
accepted as such on the flash-light data. In point of fact, in this case
the pupil later reacted more normally to light, and the speech and
writing disorders measurably cleared up.

    1. Can alcohol produce the Argyll-Robertson pupil? The majority of
      neurologists would today answer, Yes.

    2. If in the case of Francis Murphy, the W. R. in the blood had
      happened to be positive on account of a non-neural syphilitic
      infection (spinal fluid negative), would the diagnosis _general
      paresis_ be warranted? Probably the diagnosis _general paresis_
      would have been made. If the patient had been lost to observation,
      he might well have been regarded as an atypical paretic with
      prodromal convulsions.

    3. Would positive globulin and excess albumin in the spinal fluid
      alone or in association with a positive serum W. R. warrant the
      diagnosis _general paresis_ or _neurosyphilis_? The chances are
      that most neurologists would advocate proceeding to treatment in
      any case of positive serum reaction, whether or not there was
      globulin or excess albumin; but the positive globulin and excess
      albumin would probably not warrant the diagnosis _general paresis_
      or _neurosyphilis_ in the absence of excess cells and the
      characteristic gold sol reaction and W. R. in the fluid.

    4. Is the case of Francis Murphy one of alcoholic epilepsy (as
      suggested by Murphy’s own phrase, “rum fits”)? It must be
      remembered that epileptics become alcoholic and that epileptic
      convulsions increase or become more severe with alcoholism. On the
      other hand, the literature indicates that alcoholism can produce
      convulsions, as can many other factors. The literature also
      indicates that there is a condition of epilepsy in which the
      convulsive tendency sets in as a result of alcoholism in a patient
      not previously disposed to epilepsy; it appears also that
      sometimes, though very rarely, the epilepsy continues after
      withdrawal of alcohol, and even after giving up the habit. Francis
      Murphy appears to have had but two spells of convulsions, both of
      them following heavy bouts with alcohol. There is so far, then, no
      warrant for calling Francis Murphy’s case one of alcoholic
      epilepsy.

    5. Does the use of alcohol by a subject destroy the value of the
      W.R.? It has been held by some that alcoholism interferes with the
      accuracy of the W. R. This has not been our experience and for the
      present we are of opinion that the results have the same value in
      alcoholics as in non-alcoholics. The next case (Collins, 61) is
      one in which a positive W. R. occurred in an alcoholic. When
      dealing with paretic neurosyphilis it is especially true that the
      W. R. is disturbed very rarely, if at all, by toxins or drugs,
      except antisyphilitic drugs.


  =Alcoholism may cloud the diagnosis of NEUROSYPHILIS.
  Differentiation by laboratory tests.=


=Case 61.= David Collins was a steamfitter of about 43 years of age,
picked up at 6.45 a.m. in the midst of convulsions and talking
incoherently, in a state apparently of fairly clear consciousness. On
arrival at the hospital, the patient was able to tell how he had always
been a hard drinker, and how during the past week of unemployment he had
taken large quantities of poor whiskey,—perhaps an average of a pint a
day. Collins also told how he had had delirium tremens several times,
but he said the present spell was quite unlike delirium tremens. There
was no disorientation or impairment of memory, and the patient did not
in any wise suggest a mental case a few hours after admission.

It appears, according to Collins, that he had obtained some work the
night before, and had quit work about 6.30, whereupon he stepped into a
barroom, took one drink of whiskey, left the barroom, walked down the
street, and suddenly lost track of the world, coming to consciousness in
a carriage with two policemen, but remaining, as he said, “dopy,”
inattentive, and confused. After a meal, however, the patient began to
feel better and soon felt quite all right.

The =physical examination= was quite negative except that
=neurologically= there was lingual and manual tremor, a speech defect,
apparent only with test phrases, unsteadiness of handwriting, left
knee-jerk greater than right, a left-sided Babinski reflex, and a
difficulty in executing rapid successive movements (dysdiadochokinesis).
This degree of neurological disorder in our experience warrants lumbar
puncture as well as a serum test. The lumbar puncture shortly disclosed
a positive globulin and excess albumin, and the returns from the W. R.’s
were positive for both spinal fluid and blood serum. The data of the
gold sol reaction were not available on account of technical
difficulties. However, it appears that the diagnosis of neurosyphilis
could hardly be avoided in this case.

David Collins differs from Francis Murphy, then, in showing a positive
blood and spinal fluid reaction for syphilis as well as a positive
globulin and excess albumin. As above remarked, it is probable that the
positive globulin and excess albumin would not warrant more than a
suspicion of neurosyphilis taken by themselves.

Unfortunately, we were unable to persuade the patient to submit to
treatment, and from the patient’s point of view possibly his decision,
not to submit to treatment, was a good one since he has had no symptoms
of any sort for a period of 18 months since his episode. However, as
abundantly elsewhere demonstrated, we feel that the patient is wrong,
and that the physicians are right in urging treatment.

    1. Is not the convulsive episode an alcoholic phenomenon in David
      Collins entirely separate from the patient’s general and
      neurosyphilis? Possibly; however, an outbreak of neurological
      symptoms with spontaneous recovery is not only consistent with the
      diagnosis of syphilis, but somewhat characteristic of
      neurosyphilis. We suspect that another attack will occur in David
      Collins.[12] We shall from time to time make use of the social
      service to suggest his going under treatment, and shall employ his
      record of contact with a public institution to drive in our
      suggestion. Still it is clear that there are numerous cases in the
      community that are not accessible to social service initiated from
      a public institution. Accordingly, educational propaganda is
      necessary for salvage of the middle- and upper-class victims of
      syphilis. It is a little unfortunate that the ethics of the
      private practitioner make such salvage of middle- and upper-class
      persons not very likely. Might it not be that an extension of
      state medicine to this field would incidentally increase the
      amount of successful private practice?

    2. What may be the cause of such a convulsive episode as that of
      David Collins? It would appear that the convulsions of general
      paresis and of neurosyphilis in general often occur without gross
      structural lesions of the brain. It may be suggested that vascular
      irritation or parenchymal irritation by spirochetes, acting in
      appropriate parts of the central nervous system, can produce such
      convulsions.

    3. What is the significance of the unilateral phenomenon in David
      Collins (left knee-jerk greater than right; left-sided Babinski)?
      The current explanation of hyperreflexia is that somehow
      inhibitory impulses from upper portions of the nervous system have
      ceased to influence the local arcs that mechanize reactions like
      the knee-jerk and the normal plantar reflex. The phenomena are
      commonly found in cases with pyramidal tract disorder, and in the
      case of David Collins one may suspect, therefore, that there was a
      central disorder affecting the right pyramidal tract above its
      decussation. One might suspect that the convulsions were initiated
      by a lesion (whether gross or microscopic in range) in the right
      side of the cerebrum; but whether in the white matter or in the
      gray matter must be left doubtful. The clearing up of all symptoms
      suggests either that the lesion was microscopic in range or that
      the phenomena were transient and functional.

    4. Can the dysdiadochokinesis be used to indicate cerebellar lesion
      in David Collins? Possibly; but it does not appear that the
      difficulty in executing successive movements was unilateral. It
      seems impossible to bring into close topographical relation the
      basis for the Babinski and left-sided hyperreflexia, and the basis
      for the dysdiadochokinesis. Alcohol is sometimes asserted to exert
      an especial effect upon the cerebellum.

    5. Must we suppose structural lesions, either (a) of the nature of
      cell losses demonstrable microscopically, or (b) of the nature of
      secondary degenerations demonstrable by Weigert myelin sheath
      methods, in the case of David Collins? It appears that we do not
      need to assert the existence of such lesions.

    6. Could the hyperreflexia and the Babinski reaction be due to local
      spinal cord disease? Possibly; but the existence of other
      neurological symptoms (lingual and manual tremor, speech defect to
      test phrases, ataxic handwriting, and dysdiadochokinesis) makes it
      probable that there were lesions, or at any rate disordered
      functions, within the cranium; and there appears to be no basis
      for asserting local spinal cord disease.


  =Differential diagnosis between NEUROSYPHILIS and ACUTE ALCOHOLIC
  PSYCHOSIS.=


=Case 62.= Joseph Buck was a chef of 60 years who came in, seeking
advice because his memory was getting poor; he was unable to remember
names and what he was about to do. He was tremulous and had much pain in
his limbs. He had been drinking heavily for weeks,—probably ten weeks;
in fact, he described himself as having had “the shakes” and as having
lately seen animals and people that were unreal. He had had the shakes
before and the condition had lasted for two to three days after alcohol
was discontinued.

=Physically=, Buck was tall, well developed, although poorly nourished,
with a skin suggesting alcoholism. There was a slight acne over the back
and chest; there was a slight enlargement of the heart, with blood
pressure, systolic, 180, diastolic, 120. There was a corneal opacity of
the left eye, which the patient said was the result of syphilis
following a chancre, which he had acquired at the age of 27. There was
also a ptosis of the upper lid of the left eye. The right pupil was
irregular and reacted to light sluggishly, and with a very small
excursion. The patient was slightly deaf in both ears. The deep reflexes
were all lively and equal. The tremor was most marked in finely
coördinated movements. There was a slight swaying in the Romberg
position but the sign could not be said to be present. The gait was
unsteady. There was a marked tenderness over the nerve trunks.

So far as =mental examination= went, it seemed that the patient’s claim
of amnesia was subjective. There was certainly no more amnesia than a
slight difficulty in recalling details. The diagnosis of alcoholism with
convalescence from delirium tremens would certainly seem to have been
sufficient for the phenomena, and the suggestion of alcoholic neuritis
only confirmed the picture. To be sure, one might expect a diminution or
absence of deep reflexes; still, these reflexes may be overactive in an
irritative stage of the disease.

Naturally, however, the history of syphilis and the pupillary phenomena
and ptosis, made the consideration of neurosyphilis necessary. Both
serum and fluid W. R.’s proved positive; there was an excessive amount
of albumin and globulin, the gold sol reaction was typically “paretic,”
and there were 377 cells per cmm.

The patient improved upon a rest treatment and was given injections of
mercury for his syphilis. After a few months he felt well enough to
return to work, and continued at work throughout a season, receiving
mercurial treatment throughout this time. A spinal fluid examination
fifteen months later showed a weaker gold sol reaction, reduction in the
amount of globulin and albumin, and but 26 cells to the cmm. The W. R.’s
had remained positive.

    1. What are the forms of syphilitic neuritis? According to Nonne,
      syphilitic neuritis and polyneuritis have at last acquired
      standing in neuropathology. The older claims depended upon
      findings on palpation and recovery after antisyphilitic treatment.
      Since the introduction of salvarsan, cases of ophthalmoplegia,
      facial, acoustic, and optic nerve disease, as well as neuritis of
      the extremities, have been reported in large numbers. These
      phenomena are to be regarded as neurorecidives in the modern sense
      of that term. The neurorecidive is not a salvarsan effect, but is
      an effect of the syphilitic process itself, settling in the
      peripheral nerves. Paresthesias are especially prominent in
      peripheral mono- or polyneuritis, and this point is of some value
      in differentiating the syphilitic peripheral neuritis from root
      neuritis. Root neuritis is more often characterized by neuralgic
      attacks. Objective hyperæsthesia of neuromuscular origin is also
      found in these cases, demonstrated by pressure on the nerves. The
      motor phenomena consist in a flaccid paresis or paralysis,
      especially affecting the radial, ulnar, and peroneal nerves. Nonne
      states that it is rare for syphilis to affect a single nerve
      region, and he regards cases in which a single region alone is
      affected as usually due to a local gummatous process.

    2. What is the significance of 377 cells per cmm.? See discussion of
      Washington (Case 66).


  =Differential diagnosis between NEUROSYPHILIS and CHRONIC
  ALCOHOLISM.=


=Case 63.= Albert Fielding, 46, was an insurance broker, who was brought
to the hospital for excessive alcoholism. Indeed, he showed all the
signs, both of chronic and acute intoxication, except that there was no
nerve trunk tenderness. Fielding was very loquacious though his speech
was rather thick. He showed tremor of hands and an alcoholic skin.
Physical and neurological examination proved entirely negative.

Fielding claimed that he had had a nervous breakdown at about 36 years
of age, after disappointment in love. He had the drinking habit and
began to drink more and more. He had now become nervous and tremulous
and had to drink in order to brace himself. After a few days, the
patient began to be much better, having recovered from acute alcoholism.
=Mental examination= now showed good memory with orientation intact.
There was a certain tendency to reminiscence and to somewhat childish
actions. He had attempted to stop drinking but had been unable to quit.
As a matter of fact, his mother and father had been excessive drinkers
and he had inherited the tendency, etc.

The =diagnosis= seemed to be plain. The routine W. R. upon the blood
serum was negative. However, the patient had remarked during the history
taking, that he had had a chancre and secondary symptoms of syphilis.
Accordingly, lumbar puncture was resorted to. The fluid showed a
slightly positive W. R.; the gold sol reaction was of the syphilitic
type; there was a considerable increase in albumin and globulin, and
there were 20 cells per cmm. The diagnosis of neurosyphilis seemed
clear.

=Course=: The patient received six months’ treatment in a sanatorium but
the symptoms remained almost as before, and the patient showed the same
childishness and inability to take care of himself. Since the symptoms
continued six months after the withdrawal of alcohol, it might well be
suspected that the condition was more than a merely alcoholic one.
However, in a number of purely alcoholic cases, such long-standing
effects are found: even as long as six months or longer after the
withdrawal of the alcohol, and one might conclude therefore that
Fielding was actually a victim of alcoholic dementia. The spinal fluid
after these six months (during which period antisyphilitic treatment was
given) showed no change, and the prognosis was offered that the case
would probably develop into one of paresis.

A year later, after six months sanatorial care and six months life in
the community, the patient returned to the Psychopathic Hospital in an
alcoholic condition. The lumbar puncture showed all signs negative
except the W. R. which was slightly positive. The W. R. of the blood was
negative.

In connection with this case, see the case of _paresis sine paresi_
(25).

    1. What is the relation of the syphilitic and alcoholic process in
      Robert Fielding? One does not like to break the so-called rule of
      parsimony in diagnosis, but it would seem that the effects in
      Fielding are the combined effects of syphilis and alcoholism.


  =Differential diagnosis between NEUROSYPHILIS, DIABETIC
  PSEUDOPARESIS and BRAIN TUMOR.=


=Case 64.= A large and imposing person, Calvin Hall, 55, had been
employed as a doorkeeper and guard, in which position he was on duty for
12 to 14 hours daily. Eventually, however, he had begun to have a good
deal of pain in the legs and a few months before observation, one day,
his legs gave way and he fell to the floor. There was, however, no loss
of consciousness, and he was carried to a general hospital. The result
of an examination there was that his family was informed that he had
some nervous trouble.

Hall now began to be melancholy and wept a good deal. His appetite and
sleep remained intact. He felt too weak to walk. At the end of about a
year, he began to improve and again became able to do a little light
work. About a month before coming to the Psychopathic Hospital, about
two years after the onset of symptoms, Hall suddenly began to talk
excessively, in a rambling and rather senseless way. A fortnight later,
he began to suffer from insomnia and restlessness.

Some medical facts were available: It seems that at 25 years this
patient had become infected with syphilis though there had never been
any secondary signs. He was married four years later but there had not
been any children. Moreover, for four years past, the patient had been
treated for glycosuria.

Upon admission, the patient’s sensorium was clear, but his orientation
was only partial. He could give a fair account of his life, but it
appeared that his memory was somewhat impaired. There were auditory
hallucinations (voices of relatives). He often mistook the identity of
persons about him. He talked in a grandiose fashion of his great
strength and especially of a God-given power to read minds. His flow of
thought was rapid, rambling, circumstantial, and with traces of
irrelevance. He was rather continuously busy and at times restive. There
was a good deal of emotional agitation and apprehensiveness, and again
the patient would become suspicious and tearful.

=Physically=, there was a discharging sinus connected with the right
humerus, close to the elbow. The pupils, though equal and regular, were
sluggish in reaction to light. The knee-jerks and ankle-jerks were
absent. There was no Romberg sign but there was some swaying in the
Romberg position. There was a moderate ataxia in walking. Glycosuria to
a moderate degree was determined. There were no casts or albumin in the
urine. The W. R. of the blood and of the spinal fluid was negative. The
albumin of the fluid, however, was considerably increased. X-ray
examination of the skull yielded a suggestion of absorption of the
posterior clinoid processes of the sella turcica. The X-ray examination
of the arm in the region of the sinus showed a chronic osteomyelitis,
possibly syphilitic (or diabetic?).

The diagnostic problems in the case of Calvin Hall are extremely
intricate. There are clinical suggestions of general paresis, not
confirmed by the laboratory findings.

    1. Are we dealing with a case of diabetic pseudoparesis? Is the pain
      in the legs of like origin, and has a neuritic process led to the
      absence of the knee-jerks? The Allen treatment appears to have had
      no beneficial result in this case.

    2. Is there a tumor of the sella region, which could account for the
      mental symptoms and the glycosuria? The spinal fluid albumin might
      be regarded as consistent with a variety of psychoses, including
      that of brain tumor. We have to remember the definite history of
      infection, the sterile marriage and the possibly syphilitic
      osteomyelitis.


  =DIABETES AND NEUROSYPHILIS, relations?=


=Case 65.= Donald Barrie, a man of 61, diabetic for several years, had
begun to worry about the diabetes, feeling that he was about to die, and
had gone so far as to make several threats of suicide. Hence he was
brought to the Psychopathic Hospital for observation.

Barrie was rather well developed and nourished, although he looked far
older than he was. There was a marked arcus senilis; the skin was dry
and rough; the radial and other accessible vessels were markedly
sclerosed; abdomen obese; right testicle very low with thickened and
hard epididymis.

=Neurologically= there was little abnormal to discover. The pupils were
irregular; both reacted fairly well to light. There was a slight tremor
of the extended hands, and still less of the tongue. The voice was
slightly thick and the patient stumbled somewhat on test phrases. Urine:
specific gravity, 1029; sugar; no acetone; no diacetic acid. Sugar 2 to
11 grams for 24 hours on ordinary diet. It proved impossible to get the
patient sugar-free, either by cutting down the carbohydrates or by using
the Allen method.

=Mentally=, the depression with reiteration of wrong-doing and
self-accusation because of the contraction of syphilis, were the
striking features. There was, to be sure, a slight imperfection of
memory for remote events; memory for recent events and knowledge of
current events was very poor. Barrie claimed that his condition was
entirely hopeless, that his memory was exceedingly bad, and that he was
no longer capable of supporting his family.

    1. What shall be said as to diagnosis in a man of 61 with
      glycosuria, depression, amnesia, sluggish pupil, slight tremor,
      slight speech defect, and a history of syphilis? The W. R. of the
      serum proved positive, and also the W. R. of the spinal fluid. The
      gold sol reaction of the fluid was of the syphilitic type. There
      were 112 cells per cmm., there was an excess of albumin, and a
      large amount of globulin. Accordingly, the diagnosis of PARETIC
      NEUROSYPHILIS (“general paresis”), especially in view of the
      laboratory findings, seems necessary.

    2. What is the cause of the glycosuria? First: possibly it has no
      relation with the syphilis; secondly: it may possibly be due to a
      syphilitic involvement of the pancreas; thirdly: it is barely
      possible that it is due to syphilitic disease of the fourth
      ventricle or of the base of the brain, involving the pituitary
      region. Perhaps our case is too complex for analysis. At all
      events, the case brings up the possibility of a syphilitic
      glycosuria.

    3. Can the diabetes in the case of Barrie be explained as
      syphilitic? Warthin of Ann Arbor has recently described somewhat
      remarkable spirochete findings in his autopsy material. The order
      of organic infection according to frequency is: aorta, heart,
      testis, adrenal, pancreas, nervous system, liver, and spleen.
      Warthin has called attention to the relation of pancreatitis and
      spirochetosis to diabetes in a recent review[13] of findings in 41
      autopsied cases from the University Hospital in Michigan. Warthin
      found active luetic lesions in the pancreas in 6 cases.


  =Hemianopsia in a case of neurosyphilis.=


=Case 66.= Lawrence Washington, a colored cabman, 58 years of age, began
to forget addresses given him by his fares. Moreover, he could no longer
see as well as before, especially on looking toward the right side. He
himself states that the visual trouble dated back as long ago as his
39th year, at which time he had a terrific pain in both temples, leading
back from the eyes. Washington thought that his vision had been getting
slowly but steadily worse ever since.

We got the impression that the amnesia claimed by Washington was more or
less subjective and he was found to be well informed. This association
of amnesia and impairment of vision naturally suggests syphilis. The
patient himself stated that he had had a chancre at the age of 18.

We found the W. R. of the serum to be appropriately positive. The W. R.
of the spinal fluid was also positive though weakly so. There was an
excess of albumin; globulin appeared in large amount; the gold sol
reaction was of the syphilitic type; there were 186 cells in the spinal
fluid.

Is this case one of paresis or of some other form of cerebrospinal
syphilis? Let us consider the data of the =physical examination=. On the
whole, the patient was well preserved. There was a slight radial
arteriosclerosis, but on the whole the cardiovascular system was almost
negative. The blood pressure was 100 systolic, 65 diastolic.
=Neurologically= the visual field of the left eye was somewhat limited,
and there was a temporal hemianopsia of the right eye. The
ophthalmoscopic examination showed a disseminated choroiditis on both
sides. The right pupil failed to react to light. The left pupil reacted
slowly. Both pupils reacted properly to accommodation.

The knee-jerks could be obtained only on reinforcement, and when
obtained, the right was apparently more active than the left. The left
Achilles was absent; the right present. There were no other abnormal
reflexes.

The motility of the facial muscles was somewhat impaired.
Finger-to-finger and finger-to-nose tests were rather poorly done. The
muscle sense was good; there was no swaying in Romberg position; and
there was no speech defect.

We are unable to decide whether the case is one of the =parenchymatous=
type (paretic) or of the =meningovascular= type of =neurosyphilis=. It
is certainly rather unusual to find hemianopsia in a paretic.

We have been unable to get definite results from the treatment of this
case, since the patient would not return for months after getting an
injection or two of salvarsan, on the ground that he was improved enough
and did not require further treatment.

    1. What conclusion can be drawn from the 186 cells per cmm. in the
      spinal fluid? Ordinarily this finding would indicate an active
      process. Some writers have claimed that a cell count running above
      100 per cmm. was an indicator of diffuse non-paretic
      neurosyphilis. It does not appear that this claim has been
      substantiated. It is remarkable that this case shows an interval
      of 40 years between infection and the occurrence of definite
      clinical symptoms. With respect to the cell count, both in
      untreated and in treated cases, the following conclusions from a
      recent article (Solomon and Koefod)[14] are in point:

      1. The number of cells found in the fluid of untreated cases
      offers no definite information of prognostic value.

      2. One is not justified in drawing any conclusions as to whether
      the case is cerebrospinal syphilis or general paresis, nor the
      time the process has been active, nor the severity of it, from the
      cell count.

      3. The cell count may vary greatly from month to month, or when
      the interval is but several days, while at other times it may
      remain very nearly the same after an interval of months.

      4. Cases showing natural remissions may show no reduction in the
      cell count, or other spinal fluid findings.

      5. Cases treated with salvarsan, either intraspinously or
      intravenously, tend to show a more or less rapid fall in the cell
      count. This count will, as a rule, remain low during treatment,
      but is likely to rise when treatment has been discontinued, but
      may rise during treatment after having first fallen.

      6. Cases may show remissions during treatment and still have a
      pleocytosis.

      7. Treated cases having the cell count fall to normal may at the
      same time become very much worse and develop more marked paralytic
      symptoms.

      8. In general paresis the cell count in no way parallels the other
      spinal fluid findings.

      9. In cases in which the other tests show an improvement, for
      instance cerebrospinal syphilis, the cell count also readily and
      early drops to normal. At times it may drop to normal before other
      spinal fluid tests become negative; again it may be last to reach
      normal.

      10. The change in cell count seen in syphilitic disease untreated
      is also found in non-syphilitic diseases, as brain tumor.

      11. The cell count offers nothing of prognostic importance in
      syphilis of the nervous system unless accompanied by improvement
      of the other laboratory signs.

      12. The cell count is not an index to the predominance of
      irritative or degenerative changes.


  =Case of CEREBRAL MALARIA and SYPHILIS: simulation of PARETIC
  NEUROSYPHILIS (“general paresis”).=


=Case 67.= Joseph Temple, 45, who had been a sea-going steamboat
steward, was brought to the hospital in a semi-stupor. He was entirely
uncoöperative, often resistive, attempting to bite the physician’s
fingers, and for the most part lying curled up. He was incontinent and
tube-fed. This phase, it seems, had begun the night before entrance to
the hospital. Twenty-four hours later, an extraordinary change was
noted. Temple became alert and attended to his wants, began to eat well,
and began to behave as normally as probably he ever behaved.

He was now able to give a coherent history. It was now January. In the
previous September, he had left for Mexico; he was returning when he
suddenly fell to the deck, unconscious. After this fall, he had not been
well, having had chills and fever. At the Marine Hospital, he had been
diagnosed as suffering from malaria, and was given quinine. He had been
delirious a short time in the hospital, not being able to recognize his
wife, who called. He shortly improved so that his wife was able to take
him home. Nevertheless, headache, gastric distress, and intermittent
vomiting continued. A spell of confusion took place, two days before
admission. The patient tossed about, moaned, and failed to recognize
anyone. Malaria of the æstivo-autumnal type was demonstrated in the
hospital. The temperature always remained at normal. He was somewhat
emaciated and pale. The pupils were small, somewhat unequal, and reacted
though poorly to light and distance. The tendon reflexes were lively.

The W. R. of the serum was positive, and information from the patient’s
physician runs to the effect that there was a syphilitic infection some
seven or eight years ago, followed by secondary symptoms, but the
patient had refused to take any protracted treatment. The spinal fluid
examination was practically negative.

=Mentally=, the patient was euphoric, expansive, boastful, and showed a
marked emotional instability and considerable memory defect.

    1. Can the diagnosis of general paresis be made in Joseph Temple?
      Certainly the acute confusion and the syncope are consistent
      enough with the diagnosis, yet the severe malaria makes it seem
      likely that the phenomena were due to a cerebral attack of
      malaria, and such occurrences are found in the æstivo-autumnal
      form of malaria. Yet malaria would hardly explain the euphoria,
      memory defect, and the pupillary findings, to say nothing of the
      irritability and the active tendon reflexes. Even if we regard the
      active tendon reflexes and the irritability as malarial, the other
      phenomena remain outstanding as exceedingly suspicious of paresis.

      On the other hand, if we try to support forcibly the diagnosis of
      general paresis, we are hardly able to explain the negative
      findings in the spinal fluid.

      In point of fact, a study of the patient’s past life revealed a
      story that the mental traits of euphoria, irritability, and memory
      defect had been characteristic of the patient for many years. In
      fact, there is some question whether the patient is not really to
      be regarded as a moron of high grade.

      Upon this basis, if we regard the confusional phenomena as
      malarial and the persistent mental phenomena as characteristic of
      a moron and somewhat exaggerated by the disease, we have merely to
      explain the suggestive pupils. As to these, it must be remembered
      that though they reacted poorly to light, still they reacted
      somewhat, so it is not a question of explaining an
      Argyll-Robertson pupil, but only an impaired pupillary reaction.
      Of course, some workers are of the opinion that pupillary changes,
      perhaps even the Argyll-Robertson pupils, may occur in syphilitic
      cases that are not neurosyphilitic, or at all events are not
      victims of central neurosyphilis. Finally, we must remember that
      there are cases of neurosyphilis of a vascular type which yield
      negative spinal fluids. The case leaves many questions unanswered.


  =Can paretic and non-paretic neurosyphilis be differentiated by
  means of the gold sol reaction? The gold sol reaction in this case
  was an extremely mild one and would not at all have warranted the
  diagnosis GENERAL PARESIS, yet the discovery of a heavy meningeal
  exudate including an unusually heavy deposit of plasma cells even in
  the spinal pia mater will perhaps warrant us in making a final
  retrospective diagnosis of paretic neurosyphilis. Autopsy.=


=Case 68.= We would like to give the full effect of our surprise at the
outcome of the case of Margaret O’Brien, a school-teacher, 26 years of
age. To be sure, Miss O’Brien developed symptoms at 22 or 23 which we
can now explain consistently with the outcome of the case; for at that
time, she began to complain of severe pain in the head, especially in
the forehead and temples, and also became nervous, unable to remain
quiet, and given to insomnia. She was markedly depressed at the time and
would refuse to talk at times. However, only the headache in this
prodromal period could be regarded as particularly suggestive of
syphilis, and headache in an over-worked school-teacher is not uncommon.

In fact, the picture presented by the patient was one of catatonic
dementia praecox. The patient was admitted to the hospital after a
sudden onset of excitement. At first she was very restless, continually
looking about and getting up and walking away from the examiner, giving
the impression of understanding all questions but preserving an air of
indifference. A few days later, the patient was gotten to answer more
coöperatively. She remarked that the hospital was heaven although in
Boston; that it was summer time (correct) and that her memory was
greatly impaired. The physician was a messenger of God (delusion later
corrected). The patient had not done God’s will; her breath was leaving
her; God’s voice was heard from time to time, and Miss O’Brien had heard
it for a long time. God tells her to do His will. However, as Miss
O’Brien remarked, “I must think all this nonsense, turning against God.”

The patient frequently attitudinized and would remain in an apparently
catatonic condition for many minutes. For the most part, she was
resistive and mute and non-coöperative as to examination. From time to
time, she made impulsive suicidal attempts. So far as a somewhat
inadequate =physical examination= was concerned, nothing abnormal could
be made out; in particular, the pupils reacted normally to light and
were otherwise normal. The routine W. R. of the blood serum, however,
returned positive, and in accordance with the policy of the Psychopathic
Hospital, the patient was subjected to a lumbar puncture. The lumbar
puncture yielded a positive W. R., 109 cells per cmm., a positive
globulin and a considerable excess of albumin, and an exceedingly mild
gold reaction—syphilitic type.

Ten days after admission, the patient had a convulsion. She never
regained consciousness, continued to have convulsions for a few hours,
and died, apparently from paralysis of respiration. The heart continued
to beat for a short period after respiration ceased. The =autopsy= was
consistent with the diagnosis which had been rendered after the
surprising results of the W. R. in the blood and the laboratory findings
in the spinal fluid had been learned. There was a generalized
encephalitis with congestion of all the smaller cerebral vessels and
petechial areas in the meninges and upon the cortical surfaces. We
regard the case as one of syphilitic encephalitis.

The brain weighed 1265 grams, indicating a loss of 79 grams by Tigges’
formula (8 times the body length in centimetres). The pia mater was, in
the gross, quite normal within the cranium; nor were any cells found in
a smear from this pia mater; but the pia mater over the spinal cord was
visibly edematous, and a smear from the spinal pia mater showed great
numbers of lymphocytes and especially of plasma cells—a finding which
was confirmed in stained section, by which a remarkable display of
plasma cells was found plastered somewhat generally over the entire pia
mater of certain segments. The brain substance was softer than normal,
but displayed no differences of consistence. The stripping of the pia
mater of the temporal lobes on both sides yielded the so-called
“decortication” (that is, the adhesion of small bits of brain substance
to the pia mater). The optic nerves were somewhat thinner than normal.
No other gross lesions of the brain were found.

The dura mater, although dense and injected, was not otherwise abnormal.
There was an early visible sclerosis of the middle meningeal arteries,
more marked on the left side.

The cause of death, so far as the autopsy revealed it, was bronchial
pneumonia. There was a diffuse nephritis.

    1. Are the hallucinations in the case of O’Brien characteristic?
      Hallucinations are regarded as playing a minor rôle in general
      paresis. In fact, earlier workers sometimes denied that
      hallucinations occurred at all, and this denial has been made once
      more of late by Plaut,[15] but Kraepelin quotes Obersteiner as
      observing hallucinations in 10%, and regards that figure as
      approximately corresponding with his own experience. Junius and
      Arndt are cited as finding 17% of their cases hallucinated.
      Auditory hallucinations are somewhat more frequent than those of
      vision (alcoholic psychosis must be considered). The visual
      hallucinations of paresis are thought by Kraepelin to be related
      with atrophy of the optic nerves, and he states that they occur by
      preference in patients having such atrophy. Hallucinations though
      not common are more frequent in non-paretic neurosyphilis than in
      paretic neurosyphilis.

    2. What was the cause of death in Margaret O’Brien? The autopsy, as
      above stated, indicated pneumonia. In point of fact, this patient
      developed convulsions and ceased respiration, the heart continuing
      to beat for some time after respiration had ceased. It may be that
      the death should be counted as one of neurosyphilitic seizure.


  =Tonsillar abscess associated with neurosyphilis (Lues Maligna?).=


=Case 69.= Frank Mason, 49 years, a rectifier of spirits, was admitted
to the Psychopathic Hospital in a tremulous, mentally confused,
depressed, and unhappy state. He was particularly concerned because he
could not give an accurate account of his past life and because he found
that he was continually contradicting himself.

Superficial examination shortly discovered the pupils to be much
contracted, irregular, and non-reactive either to light or distance.
Although these pupils showed more than the Argyll-Robertson phenomenon,
yet the suspicion of syphilis was important.

Throat examination showed a large area of ulceration involving the whole
of the right tonsil and extending even to the left side of the median
line so that the whole of the faucial pillar was involved. In the midst
of this ulcerative area was a mass of purulent necrotic tissue, about
which the edges of the ulcer stood out sharply. There was, however, very
little acute reaction about the margin of the area.

The association of pupillary changes (especially stiffness to light),
what looked like tonsillar gumma, and mental disorder (including memory
disturbance) heightened the impression of syphilis.

However, the remainder of the examination was not especially
confirmatory of the diagnosis. The man was well developed and obese,
with a slightly enlarged heart, with sounds of poor quality and the
aortic second sound accentuated. The systolic blood pressure was 130;
the diastolic, 90. There was no disorder of reflexes except that the arm
reflexes were very lively.

After a time, a few facts concerning the patient’s life became
available. Although a rectifier of spirits, Mason could not be found to
have over-indulged in alcohol. It appears that some five months before
his admission to the hospital, a wisdom tooth had been extracted. About
four months before admission, the ulceration of the faucial pillar had
begun, and this ulceration was immediately laid to infection from the
wisdom tooth cavity. Mason then had to discontinue work and a depression
followed. But the account of this depression led us to think that he was
a victim more of natural sadness than psychopathic depression. There was
much worry and insomnia. To meet the insomnia, large amounts of
hypnotics were administered. The sequence of these hypnotics was a
tremendous disturbance and continual crying out by the patient. In fact,
Mason became so excited that he was removed to the Psychopathic Hospital
for temporary care in the condition above mentioned.

We naturally awaited the outcome of the serum W. R. The return was
negative. However, the typical position of the ulcerative lesion and the
non-reacting pupils,—to say nothing of the mental symptoms and the
associated tremors, with incoördination (this incoördination was
non-characteristic and apparently due largely to the tremor),—led to
lumbar puncture.

The spinal fluid yielded a weakly positive W. R. There was a slight
positive albumin, the globulin test was slightly positive, there were 14
cells per cmm., and the gold sol reaction was of the syphilitic type. We
were, then, probably entitled to conclude that syphilis was active not
only in the body at large but also in the nervous system. Looking back
upon the case, we considered that large doses of morphine and hyoscyamus
might well have produced the marked mental confusion and possibly the
tremors that characterized Mason on his arrival at the hospital.

Improvement followed after a few days of rest; the confusion disappeared
and the tremors diminished; the pupils returned to their normal size and
reaction; depression persisted, and the patient was very properly much
concerned about the tonsillar lesion. However, further improvement did
not take place under antisyphilitic treatment and patient died after
several weeks from what was believed to be an embolus from the tonsil.

    1. What was the true interpretation of Frank Mason’s pupillary
      changes? They were probably due to the opiates, despite the fact
      that, taken in association with the gummatous lesion of the
      faucial pillar, we had regarded them as possibly syphilitic.

    2. How shall the negative serum W. R. be explained? Such a reaction
      is consistent with the diagnosis _gumma_. It is, however, a little
      surprising that with active neurosyphilis and a relatively active
      non-nervous syphilitic lesion like that in this case, the serum W.
      R. should have been negative. Possibly a repetition of the test at
      various times would have shown a positive serum W. R. In any
      event, the fluid reaction was positive.

    3. Could the tonsillar ulceration be due to dental infection? The
      chances are against this on account of the interval (2 months)
      between extraction of the wisdom tooth and the ulceration, which
      itself seems to be of a tertiary syphilitic nature. In point of
      fact, the patient admitted a syphilitic infection 21 years
      previously namely, at 28 years of age. At that time he took large
      quantities of mercury and potassium iodid by mouth.

    4. Relation of the case of Frank Mason to the so-called _lues
      maligna_? The case closely resembled the cases reported by Bly.
      Frank Mason showed great destruction of tissue, toxemia, failure
      to react to antisyphilitic treatment. In both of Bly’s cases, the
      tonsil was the starting point of the illness; and in both cases
      there was a trauma of the tonsil or peri-tonsillar structures
      (tonsillectomy and application of caustic). In our case there not
      only had been extraction of a wisdom tooth, but the tonsil had
      been cauterized.


  =Neurosyphilis versus multiple sclerosis.=


=Case 70.= Annie Kelly is a young Irish woman, 21 years of age, who was
perfectly well until three months before her admission to the
Psychopathic Hospital, when suddenly one evening she became very dizzy.
This was followed by a chill and vomiting. The next day she had a sore
throat but was able to be about and do her work. The dizziness, however,
continued and she began to feel rather queer. Gradually it became
difficult for her to walk on account of staggering.

A little later she noticed a weakness of the left side, involving face,
arm, and leg; then she began to find it difficult to talk. Finally the
right leg became weak, making walking practically impossible. All these
symptoms grew worse and the dizziness increased. At times her vision
would be blurred; there were somewhat frequent attacks of diplopia.
Finally she had to take to her bed, and at last she lost control of her
sphincters.

At no time did she suffer any pain. She was taken to a hospital, and
after a time improved somewhat; but she was told she had a brain tumor
and had better be in a large city, where she could have surgical aid if
this became necessary; consequently, she was brought from Montana to
Boston.

On admission to the hospital, the examination disclosed no important
symptoms outside of the nervous and locomotor systems. She was unable to
walk unless assisted. The pupils were large but reacted well to both
light and accommodation, were equal in size, and regular. Slight
nystagmus was present; there was no ptosis or strabismus; vision in the
left eye was poor. The other cranial nerves showed no involvement. The
tendon reflexes were all present and very lively; Babinski, Gordon, and
Oppenheim signs were present on either side. The ataxia was marked,
especially of the lower arms, and she had some difficulty in the
alignment of the fingers. The sense of position of the limbs was very
poor. There was some tremor, which was not of the intention type. The
writing showed some incoördination. The speech showed nothing abnormal.
=Mental examination= disclosed nothing of note objectively, but patient
stated she could not think so clearly as she could formerly.

The =diagnosis= would seem to lie between brain tumor,—which had been
suggested to the patient by her physician,—multiple sclerosis, and
neurosyphilis. The numerous neurological symptoms without any definite
evidence of intracranial pressure were sufficient to rule out for the
moment the consideration of brain tumor. The syndrome of multiple
sclerosis is not complete, but the race, age, and onset, with the
increasing and decreasing intensity of symptoms are very suggestive of
this diagnosis. The symptoms, of course, are all consistent with
neurosyphilis also, and while the patient denied any knowledge of
syphilitic involvement, the examination of the blood and spinal fluid
was made. The W. R. was negative in both the blood serum and spinal
fluid. Further examination of the spinal fluid showed presence of
globulin and an increase in the albumin content, 43 cells per cmm. and a
“paretic” type of gold sol reaction. With the negative W. R. of both
blood serum and spinal fluid, and with so much in favor of MULTIPLE
SCLEROSIS, this diagnosis was made.

    1. What is the relation of multiple sclerosis to syphilis? There is
      no definite relationship between multiple sclerosis and
      syphilis,—that is, multiple sclerosis is not a syphilitic disease;
      but the complete syndrome of multiple sclerosis is often given by
      a syphilitic involvement of the central nervous system (see case
      Lauder, 71).

    2. Is the spinal fluid finding in this case consistent with multiple
      sclerosis? According to Nonne, about 19% of the cases of multiple
      sclerosis show globulin and pleocytosis in the spinal fluid. As a
      rule, the number of cells ranges between 10 and 20 per cmm. and
      the globulin is not present in large amounts. In this case, the
      amount of globulin, which was given as 2+, is only a moderate
      amount,—less than is usually found in cases of general paresis.
      There are not very many cases of multiple sclerosis in the
      literature in which a gold sol reaction has been performed, but in
      the majority of those tested, the reaction is reported as mild.
      However, cases of multiple sclerosis giving a typical paretic
      curve have been described by a number of observers, among whom may
      be mentioned Kaplan and Solomon.

    3. How frequently is it necessary to make a differential diagnosis
      between multiple sclerosis and neurosyphilis? Before the days of
      the W. R. this differentiation was much more difficult than at
      present. But we, however, still have to face a not very rare
      difficulty in separating the two conditions. Syphilis is prone to
      cause small localized lesions in the nervous system. The changes
      in the patient’s condition, with improvements and regressions are
      equally characteristic of both diseases. How closely the
      symptomatology of neurosyphilis may simulate that of typical
      multiple sclerosis is shown in the next case (Lauder, 71). When
      the sclerotic area of multiple sclerosis occurs in appropriate
      parts of the cerebrum, symptoms of mental disturbances will occur.
      In its histological picture multiple sclerosis is at times highly
      suggestive of syphilis, even showing mononucleosis and meningitis.


  =Optic atrophy; nystagmus; spasticity; intention tremor. Diagnosis:
  ?=


=Case 71.= James Lauder began to lose his eyesight at 32 years, and was
shortly determined to be suffering from primary optic atrophy. In the
course of a year, he had become completely blind. No mental symptoms had
developed.

=Physically=, Lauder was in very good condition. =Neurologically=, there
was a complete optic atrophy with paralysis of the internal rectus
muscle, marked nystagmus, and absent pupillary reactions. All the tendon
reflexes were exceedingly lively, though the right arm reflexes were
more lively than the left, and the left leg reflexes more lively than
the right. There was an ankle clonus on both sides. The abdominal and
cremasteric reflexes were lively. There was a slight intention tremor.
There was, however, no ataxia and no speech defect.

=Diagnosis=: The nystagmus, optic atrophy, and the reflex disorder
suggested multiple sclerosis, although the liveliness of the superficial
reflexes, especially the abdominal reflexes, was a point somewhat
against any advanced degree of multiple sclerosis. It would appear that
the absence of pupillary reaction to accommodation is also rather
unusual in multiple sclerosis.

The serum and spinal fluid W. R.’s proved positive. There were 25 cells
per cmm., albumin was in excess, and there was a positive globulin
reaction.

    1. What is the significance of optic atrophy and other optic changes
      with respect to neurosyphilis? Canavan, from our laboratory, has
      reported that she found that 40 of 58 unselected cases of mental
      disease exhibited obvious and undeniably important changes in the
      optic nerve. She found that optic nerve changes were even more
      frequent than chronic spinal cord changes as detectable by the
      same method (Weigert myelin sheath method); there were only 34 of
      the 58 cases which showed chronic spinal cord changes. Eighteen
      cases very probably syphilitic (although the clinical evidence was
      not in all cases supported by the W. R.) failed to show optic
      nerve changes in but three instances. The 15 syphilitic cases that
      did show optic nerve changes showed them in but one eye in three
      cases, in both eyes in 12 cases. Canavan incidentally demonstrated
      a spirochetosis in the pial sheath of the optic nerve in a case of
      neurosyphilis, possibly paretic.

    2. What is the frequency of eye changes in neurosyphilis? Posey and
      Spiller (“The Eye and the Nervous System,” 1906) quote Kéraval as
      finding 42 instances of fundus change in 51 cases of paresis.
      Clifford Allbutt found 41 cases of atrophy in 53 of paresis; other
      authors have found far fewer. Optic atrophy sufficiently marked to
      cause blindness is relatively rare in paresis. Compare table of
      eye changes from Joffroy under Case Falvey (55).

      As for optic atrophy in tabes, Posey and Spiller record statistics
      as so various as to be on the whole unsatisfactory. The highest
      percentages found appear to be those of Mott, 80%, and Gross, 88%.
      It is evident that the standards for measuring optic atrophy must
      differ very much.


  =Atypical case of neurosyphilis. Picture of Huntington’s chorea.=


=Case 72.= Margaret Green, 28, was received at Danvers State Hospital in
an excited and frightened state. She was very talkative and said that
she was being bitten by snakes and serpents. She thought every one
approaching her was the devil, and sprinkled what she called “holy
water” about her for protection. It was clear that she was hallucinated.
She heard her child crying, and she saw a woman carrying it away.

After a few weeks, Mrs. Green grew quiet and more rational except for a
few spells of violence and noise; she gave the impression of a rather
pleasant and agreeable, though somewhat demented, patient. Physically,
beyond a tremor of fingers and tongue and lively knee-jerks and some
evidence of enlargement of the heart, there was nothing to be found.

Margaret Green is still in the Danvers Hospital, being now 48 years of
age. During the twenty years, she has presented,—besides the mental
picture of impairment of memory—occasional spells of confusion, a
variety of delusions based, at least in part, upon auditory and vivid
visual hallucinations, a certain irritability and psychomotor
excitement, and a picture of Huntington’s chorea. The diagnosis of
Huntington’s chorea has always been in doubt by reason of the lack of
any evidences of hereditary taint; it has, however, not been possible to
secure a properly intensive account of her relatives.

It appears that the choreic movements were first observed—in the
hospital at least—about 16 years ago. The patient has always been
decidedly mixed upon dates. From internal evidence derived from her
obviously in part erroneous statements, it may be that the chorea began
at the age of 23. It appears that she had been often termed a victim of
St. Vitus’ dance, and had had to leave her work in the mill on account
of the disease. From one source of information, it would appear that the
patient began to have what was called St. Vitus’ dance when she was 14
or 15 years of age; so far as this informant knew, no other member of
the family had had the affliction.

The first movements observed in the hospital were irregular, jerking
movements, more marked in the left arm but also occurring in the other
extremities, as well as in the face, wherein were produced peculiar
grimaces. The twitching movements would become decidedly worse during
spells of irritability. Observation in the patient’s early thirties left
the question in doubt whether the left pupil reacted to light or not. In
1904, when the patient was 36, both pupils failed to react to light
either directly or consensually. At this time, the jerky movements
continued, especially in the left hand and forearm, the tongue was
tremulous, test phrases were poorly pronounced, the knee-jerks were
exaggerated (especially the left), and both wrist-jerks were
exaggerated. The systematic examination, however, revealed no other
neurological disorder. Within a year, slight spurious ankle clonus
developed on both sides; the eyes, especially the left, gave the
appearance of developing cataracts. A slight consensual light reaction
was demonstrable on the right side, but all light reactions were absent
in the left eye.

At the age of 42, the patient was still disoriented for time, place, and
persons and subject to a deep amnesia; was tidy, tranquil, and of a
pleasant demeanor, but many of her muscles were in continual motion.
There were chewing movements and both hands and feet were rarely still.
There were no longer any spells of irritability or violence; and once
when found crying on the piazza, Mrs. Green, on being asked the reason,
replied that a gray cat had come and looked at her so hard it made her
cry. There were other crying spells at times for equally good reasons,
or for no reason.

More recently, the patient has become fairly well oriented for time and
place, and has acquired a fairly good insight into her condition and a
good memory for past events. She has had occasionally auditory
hallucinations, as of water running. In 1914, it was reported that the
pupils reacted to light, and the rest of the systematic neurological
examination was negative except that the knee-jerks were exaggerated;
and a re-examination in 1916 showed the pupils still reacted to light.
At present, the patient is disoriented for time, stating that her age is
about 25; she is no longer subject to auditory hallucinations; she has a
marked difficulty in enunciation, emphasized by the lack of teeth and in
part due to continual movements of the tongue; the movements appear to
be part of a generalized chorea.

In a systematic review of the Wassermann findings in the hospital
population, the blood of Margaret Green was examined and found to be
positive. Lumbar puncture forthwith performed showed a positive W. R. in
the fluid; there was a positive globulin and an excess of albumin; the
gold sol was characteristic of paresis; there were, however, but three
cells per cmm.

    1. Are the choreiform movements related to the demonstrable syphilis
      of the nervous system? Neither the fluid W. R. nor the gold sol
      reaction should be regarded as necessarily an indicator of tissue
      loss. The fluid W. R. is commonly thought to signify merely that
      the fluid contains substances which are somehow due to the
      presence of spirochetes in some region pretty closely related with
      the fluid. The gold sol reaction, although well established to be
      characteristic of neurosyphilis, is perhaps not so strong an
      evidence of the existence of spirochetes in the region from which
      fluid constituents are derived. There is no pleocytosis. However,
      the positive globulin test and the excess of albumin do indicate a
      certain amount of destructive process somewhere in the neural
      tissues. Are we to suppose that these substances have been
      continually found during the course of this disease? This question
      cannot be answered with the data in hand, and we can only suspect
      that these positive tests for albumin and globulin are an effect
      of tissue destruction caused by neurosyphilis. It must be admitted
      that the argument here is a little tenuous. The lesson is plain:
      that in the present stage of our knowledge the W. R. should not be
      omitted even in cases which present a fairly convincing picture of
      some well-known entity. Thus, a disease, which looks like
      Huntington’s chorea, as well as a disease suggestive of multiple
      sclerosis, requires investigation by the methods of the
      syphilographer.

    2. How shall we explain the changes in pupillary reaction in this
      case? They cannot yet be explained. A few observers have reported
      changes in pupillary reflexes in the direction of normality. In
      our experience such changes have not been noted. It cannot be too
      strongly emphasized that it is very easy to make errors in judging
      pupillary reaction if care is not used. For instance, if the
      patient is accommodating for near vision, light will probably not
      cause contraction. A frequent cause of error in testing the light
      reflex arises from using a weak electric light. An electric
      flash-light is much less efficient than daylight. Probably the
      most satisfactory method is to take the patient to a window, ask
      him to look at a distant object, shade the eye with the hand,
      remove hand, and observe.

    3. What is the chief triad of symptoms in Huntington’s chorea? (1)
      Choreiform movements associated with (2) progressive mental
      enfeeblement, (3) occurring in a patient whose family history
      shows a similar condition in a preceding generation.


  =Differential diagnosis between NEUROSYPHILIS and SENILE
  ARTERIOSCLEROTIC PSYCHOSIS.=


=Case 73.= Marcus Chatterton was a retired sea captain, 75 years of age.
At the age of 71, he had had a seizure with a slight right hemiplegia
and inability to talk. He had been slightly confused for a short time
but had rapidly recovered. During the intervening four years, there had
been three similar attacks, and the last one had caused him to come to
the hospital. He was, in fact, confused upon admission but had become
perfectly clear by the next day. There was a considerable memory defect,
which the patient himself did not entirely appreciate. Possibly his
judgment had been deteriorating slightly. He had been irritable of late
and sometimes sleepless.

=Physical examination= showed a rather well preserved man with but
slight senile changes. The pupils were equal and reacted readily to
light and accommodation. There was no sensory disorder and no
disturbance of coördination. There were no tremors. The systolic blood
pressure was 205, the diastolic 135. The arteries were sclerotic upon
palpation. A sufficient diagnosis would have seemed to be
arteriosclerosis, and the hypothesis of syphilis would hardly have been
raised off-hand by most practitioners. The W. R. of the serum was
negative. What led to lumbar puncture in this case was the fact that the
sea captain’s wife had died 15 years before of general paresis. The
lumbar puncture was rewarding since the W. R. was positive. There was an
increase of albumin and globulin, a “paretic” type of gold sol reaction,
and 56 cells per cmm.

Accordingly, we must regard the condition as one of neurosyphilis.
Perhaps the arteriosclerosis was of syphilitic origin. If this is a case
of general paresis as we suppose, it is one of very long-standing
syphilis.

    1. Do delusions of grandeur in the senile period suggest syphilis?
      Not necessarily; it appears that there is a small group of senile
      cases which might be called cases of senile pseudoparesis in which
      extravagant delusions of grandeur are entertained, and in which
      frontal atrophy is found although entirely without evidence of
      chronic inflammation. It has not been proved that these cases are
      of syphilitic origin. It is suggestive that the site of the most
      extensive lesion is precisely the site of the most extensive
      lesion classically found in paretic neurosyphilis, viz., in the
      frontal regions.

    2. Is neurosyphilis frequently found in both mates? It can hardly be
      said that this is a usual finding. However, it is far from rare,
      and it occurs frequently enough to be used in support of the
      theory that there is a special strain of spirochete that has a
      predilection for nervous tissue. It must be remembered, however,
      that the wives of syphilitics are frequently infected without
      being aware of it. In such cases they receive no treatment and
      consequently have a larger chance of developing neurosyphilis. It
      is a good rule to consider the mate of every syphilitic a
      candidate for neurosyphilis.


  =An atypical case of recurrent dazed states resembling HYSTERICAL
  FUGUES. Probably an instance of NEUROSYPHILIS.=


=Case 74.= Abel Bachmann, a man of 40 years, remains doubtful and
perhaps belongs to the still unresolved group of mental cases due to
syphilis that cannot be placed in any of the well-known categories.
Bachmann had been found by the police, working in front of a cowbarn
without the consent or even the knowledge of the owner. Bachmann had, in
fact, spent the night in the cowbarn and was working with the idea of
paying for his night’s lodgings. The situation struck the police as so
peculiar, and Bachmann was so confused and irresponsive, that he was
brought to the Psychopathic Hospital. The afternoon of his admission,
however, he entirely cleared up and was able to give a good account of
himself.

His story was that he had been worrying a good deal about a divorce
suit, and the morning of his episode he had awakened with peculiar
feelings. He walked from Boston to Cambridge, feeling that he was in a
strange city. He recognized the places he passed, yet they all seemed to
be changed. Upon reaching Harvard Square, he determined to return to
Boston and walked and walked, failing to reach Boston. All day he had
eaten nothing; when night fell he stole into a field and dug out
radishes. A postman stopped and said, “Hello, Bill,” which awakened him
as by an electric shock. A barn presented itself, in which he spent the
night. In the morning, the barn looked different. In fact, his entire
surroundings appeared mysterious. As he felt like working, he went to
work in front of the barn.

It seems that in his life there had been two other episodes of a similar
nature; in fact, Bachmann had been in a state hospital for six weeks
after the first episode. The first episode had lasted a few days only,
and followed worry when he learned that the girl with whom he was in
love was married. The second attack followed the death of his mother,
whereupon he was taken to a state hospital although the total duration
of symptoms was only three days. Bachmann had had a chancre or some
other form of genital disease at 26, and had at that time been treated
with mercury.

Except for irregular and absolutely rigid pupils, reacting neither to
light nor to accommodation, Bachmann showed no physical and especially
no neurological disease whatever. Moreover, the W. R. in the blood serum
was negative.

As to diagnosis, one might consider hysteria, of which, however, there
are no visible stigmata. It would not appear that brain tumor would be
likely to have lasted so long as eight or nine years, even if we should
attempt to make the hypothesis of tumor cover both the non-reacting
pupils and the episodes. Bachmann was non-alcoholic, and there was no
sign of any other form of intoxication. The spinal fluid showed a
negative gold sol reaction, there were no cells in the fluid, there was
no globulin; albumin was normal. However, the W. R. was strongly
positive.

The situation, then, in this case is that we have somewhat peculiar
psychopathic episodes, pupils rigid to light and accommodation, a
positive W. R. in the spinal fluid, and extremely little else to permit
a diagnosis. We are ignorant as to the course and pathology of such
cases. However, we cannot resist the temptation of the diagnosis of
neurosyphilis, although further classification is not ventured.

    1. What is the significance of stiff pupil as an isolated symptom?
      Nonne finds that in the end, after years of observation, the
      Argyll-Robertson pupil turns out to be an advance courier of other
      more functionally serious signs and symptoms of neurosyphilis. We
      can confirm this experience and regard it as an established
      clinical proposition that the Argyll-Robertson pupil cannot be
      neglected. In this connection, refer to the case of alcoholic
      pseudoparesis (Murphy, 60), and also to the case of pineal tumor
      (Donald Falvey, 35). Enthusiastic reports have occasionally been
      made upon apparent restoration of the true syphilitic
      Argyll-Robertson pupil to normal light reaction. The difficulties
      in rendering the symptomatic diagnosis of Argyll-Robertson pupil
      in a given case are so great, and the chances of complication so
      numerous, that we are inclined to attach little significance at
      present to these claims.

    It may not be amiss to mention a somewhat humorous incident familiar
      to some local neurologists. A case was reported by the interne for
      a number of months as a victim of a pupil stiff to light and
      accommodation, and the entirely adequate cause of this phenomenon
      was actually only discovered at autopsy by the triumphant medical
      examiner, who demonstrated that the patient in question was
      possessed of a =glass eye=.


  =TABETIC NEUROSYPHILIS (“tabes dorsalis”) versus PERNICIOUS ANEMIA
  with spinal symptoms.=


=Case 75.= Mrs. Brown was a woman of 56, who for the past eight or ten
years had been complaining of trouble in her legs. As she described it,
at times her legs were so weak she could hardly stand; at other times
there was considerable pain and numbness. She has always been considered
“high strung”; that is, she had a very bad temper and lost control of
herself almost entirely when she became excited. Her legs had been
growing progressively worse, and for about a month prior to admission
she had been unable to stand or walk. She had also lost control of her
bladder. On account of her temper, it had been almost impossible to
nurse her; no nurse would stay with her because of her scolding and
fault-finding. Recently, she had been having fits of the blues.

Her husband, who was seen before Mrs. Brown, was an old gentleman, over
70, who was chiefly remarkable from the fact that he had unequal,
irregular pupils, which reacted neither to light nor accommodation;
there was also a speech defect.

The patient herself proved to be extremely irritable, as had been
stated,—so much so that at times it seemed almost impossible to do
anything for her. She was very querulous, constantly complaining, and
not satisfied with anything that was done. Aside from this, her =mental
examination= proved to be entirely negative; that is, there were no
psychotic symptoms.

The systematic =physical examination= gave the following significant
findings: blood pressure, 160 systolic, 90 diastolic; no evidences,
however, of peripheral arteriosclerosis. Patient was unable to walk or
stand, and had no control over her bladder. The knee-jerks and
ankle-jerks absent on both sides; ataxia in the leg movements; loss of
sense of localization, with no tenderness over the nerve trunks; no
atrophy, paralyses, or muscular asymmetry of the parts. The vibratory
sense was maintained. Subjectively, the patient thought that the
vibratory sense differed in the legs from that in the arms.
Localization, touch, pain, heat, and cold responded to correctly. The
arms showed nothing abnormal; there was no incoördination, dysmetria, or
dysdiadochokinesis. Her pupils were equal, regular, and both reacted
normally to light and accommodation.

=Diagnosis=: The first consideration in the case is naturally tabes
dorsalis, especially when one considers that the husband had signs which
suggested syphilis of the nervous system. The rapid onset of the acute
symptoms in this case, and the absence of the characteristic signs of
pain were at least atypical for this diagnosis, as was the absence of
any pupillary signs. Further, the W. R. was negative in the blood and
spinal fluid; there were no definite signs of inflammatory reaction as
shown by the other spinal fluid tests. These findings made a diagnosis
of tabes entirely improbable. After tabes, the most frequent cause of
the symptoms above enumerated is perhaps to be found in pernicious
anemia. Examination of the blood showed that the patient had 2,500,000
erythrocytes per cmm. The hemoglobin by Tallquist scale was 80%. The
smear was practically negative; no blasts could be seen. Although this
picture is not a typical one for pernicious anemia, at least it is
significant in the low number of red cells to be found, and as no causes
for anemia were to be found, it seemed probable that we were dealing
with a primary anemia. The diagnosis in the case, therefore, is spinal
sclerosis of primary anemia. The mental picture was not uncharacteristic
of PERNICIOUS ANEMIA.

    1. Could the diagnosis be rendered in this case without a lumbar
      puncture? In the first place, the emaciation is not entirely
      characteristic. The pupils react normally to light. Probably such
      a case might well have been regarded as one of tabes dorsalis in
      former days, or even at the present time, if a lumbar puncture had
      not been resorted to.

    2. Could this case possibly have been one of tabes dorsalis with
      negative spinal findings? Such cases have been reported
      frequently, but, unlike the present case, are apt to be of
      long-standing and non-progressive, in which the active
      inflammation is no longer present. The negative findings would not
      be consistent with tabes, in which the symptoms are of short
      duration and of sudden onset.

    3. If the serum W. R. had been positive would the diagnosis have
      been neurosyphilis? We are loath to make the diagnosis of spinal
      syphilis when the spinal fluid is normal. Syphilis may produce a
      marked anemia, however, and thus produce symptoms such as shown by
      Mrs. Brown. It is even possible that such is the explanation of
      this case, taking into consideration the suggestive findings in
      the husband. However, there is insufficient evidence to make such
      an hypothesis rock firm, and we do not more than suggest it.


  =Atypical case of CONGENITAL NEUROSYPHILIS—peculiar mental state.=


=Case 76.= James Seabrook, 20 years of age, obviously showed a number of
signs of congenital syphilis. The =physical examination= disclosed an
old scar and indentation in the right mastoid region, another on the
right side of the neck, another on the posterior surface of the right
forearm, and two on the outer surface of the right upper arm. The
lesions were about the size of half a dollar. There was a saddle-shaped
nose and a perforation of the palate and uvula; there were palpable
cervical and axillary glands, small but numerous. There was a dulness in
the region of the right scapula, and slight dulness on both sides
behind. There were loud whispering and piping râles and bronchial
breathing throughout the chest, more marked on the left; there was much
coughing, and the sputum was at times blood-stained. The pupils were
irregular but reacted perfectly. The left knee-jerk was slightly more
active than the right. The W. R. in blood and fluid was negative; the
gold sol, globulin and albumin tests were negative. There were, however,
56 cells per cmm. in the fluid.

We learned that the patient had had several spells of great excitement,
with pounding on the door and a desire to fight bystanders. There were
spells of headache and vertigo. =Mentally= the tests showed him to be
subnormal.

The =diagnosis= Of CONGENITAL SYPHILIS seems established; possibly the
pulmonary condition is syphilitic. The mental subnormality as well as
the abnormal traits and episodes are probably to be accounted for on the
basis of syphilitic involvement of the brain.

    1. Are the headache and vertigo connected with syphilis? This is
      perhaps suggested by the pleocytosis in the spinal fluid.

    2. How shall we explain the negative W. R.? This patient had
      received, shortly before his entrance to the hospital, salvarsan
      and mercury. Possibly the drug treatment has little or nothing to
      do with the negative W. R.’s since they not infrequently grow
      weaker as congenital syphilitics grow older.

    3. What is the explanation of the spells of excitement? Compare the
      spells of excitement in a form of neurosyphilis described by
      Kraepelin, namely: syphilitic paranoia, discussed in the case of
      Bridget Collins (59).

    4. Is treatment indicated considering the W. R.’s to be negative in
      blood and fluid? Despite the negative W. R.’s in this case
      treatment is strongly indicated on account of the pleocytosis.
      This would seem to indicate that there is an active inflammatory
      process in the cerebrospinal axis, and it is more than probable
      that this process is syphilitic. How much real improvement of the
      symptoms would result from antisyphilitic treatment it is
      impossible to prophesy. Every case is a special problem, and this
      case is very unusual in showing a pleocytosis in the absence of
      other indications of syphilitic nervous disease, _viz._, globulin,
      albumin and W. R.’s.


  =CONGENITAL NEUROSYPHILIS resembling an undifferentiated case of
  FEEBLEMINDEDNESS—actually PARETIC.=


=Case 77.= John Friedreich, a 7–year old boy, was brought to the
Psychopathic Hospital by agents of a charitable society, who found him a
neglected child and quite evidently a subnormal one.

The dominance of syphilis in the situation was clear. The boy’s father
had died but a few months before of syphilitic heart disease, from which
he is said to have suffered for five years. The boy’s mother (the
parents were first cousins) had also been treated for syphilis and was
excessively alcoholic. The first child of this union—a girl—had died at
6 years, of a disease diagnosticated spinal meningitis. The history
indicates that syphilis was acquired after the birth of this first
child; but in any event it is possible that the meningitic condition of
which the first child had died was syphilitic. The second pregnancy
terminated in a stillbirth; the third issued in a girl, who died two
weeks after birth of what was termed “inward convulsions.” The fourth
pregnancy resulted in a miscarriage; the fifth in our patient, John
Friedreich. The sixth pregnancy resulted in a girl, now 5 years of age,
who is apparently normal. (Her W. R. was negative and she shows no
stigmata of syphilis.)

The patient, John Friedreich, at some very early age had a rash on his
body diagnosticated as syphilis. He also had many seizures called
fainting spells. Ever since birth he had been taking mercury pills. He
had not learned to talk until his third year, and was able then to say
only a few disconnected words. In fact, John has never been able to talk
in complete sentences, mumbling much that is quite unintelligible.
However, he walked at 15 months in a normal fashion and nothing peculiar
in his gait was noted until he was 5 years old, when he began walking on
his toes, particularly those of his left foot. Shortly thereafter, the
seemingly inevitable trauma appeared; John fell out of a window and
severely injured his left leg, whereupon the peculiarity of toe-walking
became more pronounced and associated with a limp.

[Illustration:

  Juvenile paresis. 7 years.
]

The patient strikes one =physically= as having the development of a
child of about five years (actual age, 7). There are a few lymph nodes
palpable in the anterior triangles of the neck. The dilated and slightly
unequal pupils react neither to light nor accommodation. There is
practically complete deafness; loud sounds are not at all noticed.

Withal, the child in a general way presents a somewhat attractive
appearance, being very playful and mischievous, lying about on the floor
and playing with whatever comes to hand, talking to himself or making a
few indistinct remarks to the bystanders. He walks awkwardly, on the
toes of the left foot. He pays little or no attention to his toilet and
needs to be dressed and cared for in all ways. He is quick-tempered and
at times very difficult to manage.

There was, of course, little doubt of the =diagnosis= of CONGENITAL
SYPHILIS and of FEEBLEMINDEDNESS. The W. R. was positive both in the
blood and in the spinal fluid. The gold sol reaction of the fluid was of
the “paretic” type; there were 44 cells per cmm. and there was a large
excess of albumin and much globulin.

As to prognosis, there is doubt.

    1. Is, or is not, this a case of juvenile paresis?

    2. Is it, perhaps, a relatively permanent case of feeblemindedness
      due to congenital syphilis? On the whole, on account of the spinal
      fluid symptoms, we should be inclined to give the case a
      relatively poor prognosis, namely, of death in a few years.
      However, we may perchance be later surprised to learn that the
      patient has lived on, at least into early adult age.

      Note: Mercury tablets in some cases of congenital syphilis do not
      seem effective. John Friedreich was treated most intensively by
      syphilographers from birth.

      Dr. W. E. Fernald in a personal communication stated that
      syphilitic cases of feeblemindedness are rather those of the
      imbecile and idiot groups than of the higher levels. This
      statement emphasizes again that the true hereditary cases of
      feeblemindedness are rather those of the higher group, whereas the
      cases in which special causes have operated in the uterus or in
      early life eventuate in idiocy and imbecility. However, such a
      case as that of Friedreich shows that now and then a case of
      feeblemindedness without evidence of neurological disorder and
      looking in almost all respects like an hereditary case may be at
      times produced by syphilis.

    3. How often is the central nervous system involved in hereditary
      syphilis? An interesting table bearing on this point is presented
      by Veeder.[16] The table concerns the lesions in various parts and
      systems of the body in 100 cases of late syphilis. It appears that
      in 47, or approximately one-half of Veeder’s series of 100 late
      cases, the infection developed some form of lesion of the nervous
      system. As Veeder remarks, this result runs counter to the common
      statements of pediatricians, notably of Holt.

                     Bones:
                   Periostitis tibia               4
                   Periostitis skull               1
                   Osteomyelitis                   1

                     Joints:
                   Acute arthritis knee            8
                   Acute arthritis ankle           1

                     Skin:
                   Macular eruption                1
                   Condyloma anus                  3
                   Gummata                         3
                   Alopecia                        3

                     Eye:
                   Interstitial keratitis         24
                   Choroiditis                     1

                     Ulcerations:
                   Nasal                           2
                   Laryngeal                       1
                   Pharyngeal                      1

                     Central Nervous System:
                   Mental deficiency              23
                   Cerebrospinal syphilis         14
                   Hemiplegia                      6
                   Epilepsy                        5
                   Spastic paraplegia              4
                   Chorea                          2
                   Hydrocephalus                   2

                     Miscellaneous Conditions:
                   Ozena                           1
                   Enlarged spleen (only symptom)  1
                   Torticollis                     1
                   Aortitis                        1
                   Obscure abdominal pain          1
                   Obscure pain in legs            2
                   Endarteritis obliterans         1
                   Paroxysmal hemoglobinuria       1
                   Raynaud’s disease               1
                   Hutchinson’s teeth              4


  =Juvenile paretic neurosyphilis. Quadriplegia.=


=Case 78.= Gridley Ringer, 15 years of age, had the facies of a
congenital syphilitic, including Hutchinsonian teeth, rhagades of the
face, and the so-called Olympic brow. No secondary sexual
characteristics had developed. There was a marked speech defect.
=Mentally=, Ringer was a low-grade imbecile. He had been born at full
term, and delivery had been normal. There had never been other
pregnancies. He had never developed normally.

The father admitted syphilis 23 years before, namely, 8 years before the
birth of his son, but the father had been treated for several years and
had been declared cured.

    1. What would be expected in the spinal fluid of this case? Without
      the history, it would perhaps be impossible to say whether the
      case was one of a quiescent imbecility or one of juvenile paresis.
      The spinal fluid of the juvenile paretic gives a picture identical
      with that in the adult. The spinal fluid in this case showed a
      positive W. R. (as did also the serum), a marked increase of
      albumin and globulin, 115 cells per cmm., and a “paretic” gold sol
      reaction. Accordingly, the diagnosis of GENERAL PARESIS was made.

    2. What is the prognosis? The prognosis of juvenile paresis is
      currently regarded as entirely grave. There is probably less hope
      for improvement in juvenile paresis than in the acquired paresis
      of adult life, since it seems to be a general principle that
      congenital syphilis is always more difficult to cure than acquired
      syphilis.

      This case had seizures a few months after initial observation, and
      the seizures were followed by a transient right hemiplegia. This
      right hemiplegia was shortly followed by a left hemiplegia, which
      remained permanently. Moreover, a few weeks later, a right
      hemiplegia again developed, leaving the patient with complete
      paralysis and aphasia. Death followed in six weeks.

    3. What effects were shown in the parents? Following up the parents
      was rewarded by the discovery that the mother was suffering from
      nerve deafness, probably of syphilitic origin, and that the father
      had recently begun to suffer from what he considered rheumatism,
      but which on examination was shown to be tabetic neurosyphilis
      (“tabes dorsalis”). This family again supports the hypothesis that
      there is a strain of spirochetes especially prone to attack the
      nervous system. Here it would seem that the syphilis acquired by
      the father had infected the mother and been transmitted to the
      son. In all three infected by the same strain or strains of
      organisms the nervous system was involved. It is difficult,
      nevertheless, to explain on this hypothesis why in one case the
      disease took the form of tabes dorsalis, in the second, eighth
      nerve involvement and in the third, paresis. This question of
      strains is really more than academic because it enters deeply into
      the question of treatment, as well as that of the suggested
      increased viability of the neural strain.


  =Is there a relation between epilepsy and juvenile neurosyphilis?=


=Case 79.= John Doran fell off the rear of an ice-wagon, at six years of
age, and shortly afterward developed fits. It appears that John was not
unconscious at the time of his fall, but that he complained of headache.
Although the convulsions were fairly frequent at first, it appears that
they later became rare and occurred only when the patient got into a
temper. At the stage of exhaustion after violent excitement, John would
fall.

=Physically=, at 9 years a fair development and nutrition were evident.
There was a great exaggeration of the frontal bosses; the nose could not
be said to be typically saddlebacked, yet there was a suggestion of a
sinking of the bridge. The teeth slightly suggested the Hutchinsonian
type, but only slightly. There was a slight roughening of the tibia, and
there was a slight scar over either knee. The patient graded according
to the Binet scale at 9 years, and he was regarded as definitely
feebleminded.

The family physician states that, according to his information, the
father contracted syphilis when the child was between three and four
months of age, and that the mother also was infected at this time.
However, the child had not been suckled except immediately after birth,
and there had been no evidences, according to the family physician, that
John had acquired syphilis.

Ordinarily, one might content himself regarding the case of John Doran
as one of idiopathic epilepsy with mental defect or deterioration.
However, the frontal bosses, suggestive teeth, the flattened bridge of
the nose, the roughened tibiae, and the old scars, though singly not of
great significance, collectively make one suspicious. Despite the family
physician’s belief that John could not have acquired syphilis from the
parents, the infection seems entirely possible despite the fact that no
symptoms developed early thereafter.

The W. R. in this case proved positive in both blood serum and spinal
fluid.

    1. What is the relation of trauma to this case of JUVENILE
      NEUROSYPHILIS? Probably none.

    2. What would be the effect of treatment? For a number of years John
      Doran was lost sight of. He was, however, treated, according to
      our information, with intraspinous injections of salvarsanized
      serum, whereupon his convulsions shortly ceased. He has been
      recently examined mentally once more, and still grades as
      feebleminded. He still has violent outbreaks of temper.

    3. Is such a case as Doran typical? Shanahan has investigated
      conditions at Craig Colony. There were 22 out of 886 epileptics
      (at Craig Colony) or 2½%, who showed a positive W. R. Nine of
      these cases were regarded by Shanahan as cases of epilepsy
      actually caused by syphilis. Viet had found 7%, and Bratz and Lüth
      5% of constitutional epileptics to be syphilitic, but the data of
      these German authors were obtained before the era of Wassermann
      tests.


  =Adrenal tuberculosis complicating juvenile paretic neurosyphilis
  (“juvenile paresis”). Autopsy.=


=Case 80.= When James Arnold appeared at the Danvers Hospital in his 22d
year, he looked as if he were but 12 or 14 years of age. He was
excessively fat but of fair muscular development. The left eye diverged
outward, and the left pupil was smaller than the right. An odd feature
was a high degree of pigmentation of the skin of the genitalia and the
groins (the axilla, the mammillary areas, and the oral mucosæ were free
from pigmentation). =Physically= speaking, the patient was practically
normal. =Neurologically=, however, there was much of interest, in the
light of which the clinical history was of value.

It seems that after an apparently normal early childhood, the boy had
begun, at the age of 11, to experience difficulty in carrying out
every-day school tasks; and after this his mental capacity had slowly
but progressively deteriorated. The deterioration was not merely
intellectual, but the boy became dishonest and untrustworthy and
developed a number of untidy and uncleanly habits, behaving at the age
of 16, as the parents stated, like a child of six.

In his seventeenth year, the boy had been taken with a severe attack of
what was regarded as an “attack of indigestion.” This attack ushered in
a gradually developing muscular weakness, especially involving the
limbs. By the age of 21 he had become irritable and the paresis was so
extreme that the patient was unable to get in or out of a carriage.

This generalized muscular weakness was plain upon admission to the
hospital though there seemed to be no actual paralysis. The patient was
unable to walk in a straight line and Romberg’s position could not be
maintained. Marked tremor was present in the hands and lips. There was
bilateral impairment of vision and nystagmus. Reflexes and sensations
normal. Speech was markedly affected, all syllables being very much
slurred. School knowledge and memory for both recent and remote events
very poor. The patient’s habits were very untidy. He was very emotional,
easily made to laugh or cry; and in behavior, extremely childish.

Two months after his admission to the hospital, the weakness suddenly
became extreme. He was constantly nauseated, refusing food. The face and
hands were cyanosed and the heart’s action rapid, weak, and irregular.
This attack lasted for a week and was followed by a period of
improvement, during which, however, he still remained very weak and
apathetic.

One month later he again became so feeble that he was unable to raise
himself in bed. He complained persistently of feeling very “sick.” His
temperature was elevated and there occurred the same train of
circulatory symptoms observed previously, _viz._, rapid and tumultuous
action of the heart, with cyanosis of face and extremities. He soon
became unconscious, remaining so until his death, which occurred on the
seventh day of the acute attack.

This case was under observation before the days of the W. R., yet
clinically the case had been diagnosticated JUVENILE PARESIS. There was
no history of the acquisition of syphilis or any likelihood of its
acquisition. Considered clinically, many of the classical features
described by Addison were present, _viz._, marked asthenia and apathy;
severe and frequent gastro-intestinal symptoms (the disease probably
commencing with the attack of so-called “acute indigestion” six years
prior to patient’s death); attacks of extreme cardiac weakness with the
characteristic small, low-pressure pulse. On the other hand,
pigmentation of the skin (usually the most striking clinical feature)
was limited to the external genitalia, these being colored a deep brown.

The most striking feature found at autopsy was a bilateral adrenal
tuberculosis (caseation, giant cells, lymphocytosis, tubercle bacilli).
The thymus gland was persistent (7×5×.5 cm.), whereas the thyroid gland
was smaller than usual. The brain showed macroscopic and microscopic
features entirely consistent with the diagnosis of general paresis,
including lymphocytosis, plasmocytosis, irregular degrees of nerve cell
destruction, and gliosis, with an especially characteristic microscopic
picture in the frontal regions.

It may be of note to consider the degree of change undergone by a brain
in 11 years or more of deterioration, and the following description of
the head findings is therefore included:

    =Head=: Hair abundant, dark. Scalp normal. Calvarium, weight 435
    gm., transparent in bregmatic region only, elsewhere thick and
    dense. The average thickness of the vertical plate of the frontal
    bone is 7 mm. The frontal bone shows a moderate thickening and
    hardening of the inner table with obliteration of diploë. Dura mater
    moderately adherent to the bregmatic region of calvarium.
    Arachnoidal villi moderately developed. Sinuses not remarkable. Pia
    mater shows a moderate focal thickening with opacity, especially
    along sulci. Vessels well injected. =Brain=: Weight, 1200 gm. The
    brain shows marked focal variations in sulcation and consistence.
    Spread on a board, the right hemisphere is obviously somewhat bigger
    than the left. There is a difference of only 0.5 to 0.75 cm. on
    measurement of the greatest circumference of the cerebrum, taken
    from the median line superiorly to the median line inferiorly, but
    the right hemisphere is throughout slightly more convex than the
    left. Both postcentral gyri are much narrowed in their superior
    portions, and the sulci posterior thereto are deeper than the other
    sulci of the hemispheres. The sulci of the orbital surfaces are
    asymmetrical and, on the left side, show a tendency to microgyria.
    The cerebral hemispheres as a whole show a remarkable tendency to
    slight protrusion of the border gyri; especially those of the two
    poles, of the free edges along the great fissure, and most
    strikingly the gyri at the boundary line between the inferior and
    lateral surfaces. This _marginal prominence_ is slight but obvious
    and is emphasized by a slightly paler color in some regions. The
    cerebrum shows a general induration which is greatest in the frontal
    tips and along the inferior borders of the lateral surfaces of the
    hemispheres, especially right. The orbital surfaces are firm,
    especially anteriorly and externally (prefrontal); the tips of the
    temporal lobes are firm, and the superior temporal gyri are firmer
    than adjacent gyri. The postcentral gyri are indurated more than the
    other gyri of the superior surface. The hippocampal gyri are
    likewise firmer than adjacent gyri.

    =Cerebellum and pons=: Weight, 145 gm. The inequality of the two
    hemispheres is more marked than in the case of the cerebrum.

    Greatest lateral diameter; left, 4.5 cm., right, 5.5 cm.

    Anteroposterior diameter adjacent to notch: Left, 5.8 cm., right,
    5.5 cm.

    There is no appreciable difference in depth. The diminution in
    volume appears to be chiefly at the expense of the right clivus. The
    inferior surface is firmer than the superior. The laminæ adjacent to
    the horizontal fissure are firmer than the remainder of the
    cerebellum. The pons is small.

There was also a lateral curvature of the spinal column, as well as
characteristic adhesions between spinal dura and pia mater which are
always suggestive of syphilis. For the rest, there were few findings of
note: some adhesions of the left pleura, hypostatic congestion of the
lungs, tracheitis, and chronic gastritis. There were four lobes of the
right lung but it is doubtful whether this should be regarded as a
stigma.

    1. Can we separate the symptoms of Addison’s disease from those of
      paresis in this case? The extreme cardiac weakness with a
      characteristic, small low-pressure pulse is in point. The asthenia
      and apathy are consistent enough with Addison’s disease as well as
      with paresis itself. It would also be possible to ascribe the
      gastro-intestinal symptoms to either disease.

    2. Of what significance is the persistent thymus? Persistent thymus
      has been observed in a few cases of Addison’s disease, but that it
      plays any part in the symptomatology thereof is a matter of doubt.

    3. How can the obesity be explained? It is of course of note that
      the thyroid gland was small, but microscopically there were no
      peculiar features in this gland.

    4. Was the adrenal tuberculosis actually primary? Minute search
      failed to reveal evidences of tuberculosis elsewhere unless we
      regard the few adhesions binding the lower half of the lung to the
      chest wall as indicative of an old tuberculosis. In particular,
      the mesenteric lymph nodes were normal.


  =Neurosyphilis? Secondary stage of syphilis.=


=Case 81.= Florence Fitzgerald, a woman 25 years of age, applied at the
police station to be taken care of. She said she had been a prostitute
for the last few months, was now ill, and wanted to reform. She appeared
physically ill and was sent to the Psychopathic Hospital, where she
remained at first almost mute, making answers chiefly by nodding the
head. She gave the impression of daze or stupor, and in fact her
condition was at first regarded as catatonic. This reaction, after a few
days, changed and Florence became quite normal, giving a full account of
her condition.

It seems that four months before going to the police station, she
developed a chancre, which was locally treated. A careful physical
examination showed a fine red macular eruption which was without much
question a syphilitic roseola. The spinal fluid yielded a positive W. R.
although other tests of the fluid were negative. Curiously enough, no
physical sign of involvement of the nervous system could be discovered.
We were inclined to regard the mental symptoms as partly due to the
syphilitic intoxication, and partly due to a psychic reaction of the
nature of defense. As for the positive W. R. in the spinal fluid, in
early secondaries various observers differ as to the frequency both of
the W. R. and of other changes, percentages being given that range from
25 to 90%. See case Caperson (45). It is of note that clinically there
were symptoms referable to a syphilitic involvement of the nervous
system; namely, marked headache and malaise. The headaches of the
secondary period are frequently the result of meningeal involvement.


  =TABOPARETIC NEUROSYPHILIS (“taboparesis”); death from TYPHOID
  MENINGITIS. Autopsy.=


=Case 82.= Frederick Estabrook was a salesman, who, be it noted, had
never had typhoid fever or any disease remotely resembling typhoid
fever. He had acquired syphilis at 19; had married at 22; was the father
of two healthy children (no miscarriages); had had a certain disturbance
of bladder and rectum, but remained a successful salesman to the age of
28, when advancing tabes confined him to bed for a time. At 30, mental
signs of PARETIC NEUROSYPHILIS developed, and death followed at 32,
after an acute illness of a week.

The details of the history after the first symptoms at 28 are as
follows:

At twenty-eight patient lost control of limbs and was confined to the
house about two months, under medical care. Three months later he had
regained partial control of his limbs but had lost all control of his
sphincters. After another month he had returned to work, but did not
work steadily and seemed to have lost ambition. In the summer of 1905,
his mind became obviously altered. He grew indolent and extravagant and
given to buying expensive and useless articles. Loss of interest in
things followed, together with loss of memory for recent events, lack of
insight into illness, delusions of persecution by wife, irascibility
followed quickly by crying. Before admission to hospital, he was
euphoric, drawling and tremulous in speech, sprawling in penmanship,
alternately depressed and exalted in manner. Knee-jerks were absent,
gait ataxic, pupils stiff to light.

The family history was negative with respect to insanity. All the family
were reported as nervous. A brother died of peritonitis at twenty-eight,
a sister of pneumonia under twenty. Another brother and sister are
living. Father and mother died of heart trouble at about sixty-seven and
sixty respectively.

The patient was at high school one year and was a fair student.
Considerable tobacco was used, and some alcohol. Intoxication denied.
There was no history of typhoid fever or other acute disease.

The patient on admission was sallow, poorly nourished, and flat-chested,
with a slight lateral curvature. There was slight dulness over right
apex in front and in right upper back. Voice sounds were increased over
right apex in front and over whole right back. The right chest showed
bronchial respiration throughout. The respiration in front of right
chest was of an interrupted character. The liver seemed moderately
enlarged. The urine showed a very faint trace of albumin. There were a
few small nodes in right groin and a scar on dorsum of penis.

=Neurological Examination.= Slight swaying in Romberg position. Slight
tremor of protruded tongue and extended fingers. Pupils irregular, left
slightly larger than right. Left pupil reacted to light consensually,
but not directly. Right pupil reacted very slightly to direct light, not
consensually. Knee-jerks and Achilles jerks absent. Ankle clonus absent,
abdominal and cremasteric reflexes brisk. Sharp and dull points were
recognized in the legs with numerous mistakes. Vocal and facial tremor.
Speech slow and drawling. Test phrases repeated well if care was taken.
Consciousness clear. Orientation perfect. Calculating ability preserved.
Many words omitted in writing. Penmanship clear but shaky.

Hallucinations absent. Memory of recent events poor. Associations of a
logical or defining type. Patient denied various statements in
commitment papers and had little or no insight into the mental side of
his disease—slight euphoria.

After a month’s observation the patient was removed to a quiet ward and
set to work a few days in the scullery. One night he began to yell as if
assaulted and said later that he had an idea that he was going to die.
Before three months had passed he had become untidy, disorderly, and
imperfectly oriented.

The general degeneration continued rapidly. One week before death the
temperature rose to 103 degrees F., and the patient succumbed to what
seemed clinically like a bronchopneumonia. Unconsciousness two days
before death.

Note with respect to history of typhoid.—Inquiries of his physicians,
wife, employer, and brother tend to show conclusively that the patient
never had a disease even remotely resembling typhoid fever.

The =autopsy= findings were as follows:

Acute conditions:

Hypostatic pneumonia, with early serofibrinous pleuritis and without
lymph node swelling; =enlargement of mesenteric lymph nodes=; =acute
cerebrospinal leptomeningitis=; multiple small hemorrhages of spleen.

Other findings:

=Scar of penis=; =sclerosis of aortic arch= (Heller’s type?) and slight
coronary arteriosclerosis; =calvarium= thin and =dense=; =dura mater
thickened= and adherent to calvarium; calcified arachnoidal villi;
=chronic= cerebral and cerebellar =leptomeningitis=; =atrophy of frontal
lobes=; =granular ependymitis=; =sclerosis of posterior columns= of
spinal cord; emaciation; unequal pupils; slight parietal fibrous
endocarditis, slight mitral sclerosis; gastro-intestinal atrophy;
chronic cystitis; chronic abscess of prostate.

    The description of the head findings is as follows:

    Skin exceedingly loose, and the whole skull cap thinned. The diploë
    are absent. Adhesion with dura easily separated. The dura somewhat
    thickened, but not distended. Along the longitudinal sinus extensive
    calcareous granulations adhere to it. The longitudinal sinus does
    not contain blood, and the inner surface is normal in color. The pia
    is extensively thickened and opaque and a general subpial exudate
    exists which is more marked over the vertex where it lifts the pia
    from the brain surface to the extent of three centimeters in
    Rolandic, superior frontal, intraparietal, and mesial precentral
    sulci on each side. The arteries at base are free from atheroma. The
    temporal lobes are much bound down by adhesions, as is the
    cerebellum. Post mortem softening is evident. The hemispheres show
    no asymmetry, but the frontal convolutions are markedly atrophic.
    The corpus callosum is united to the cortex by old adhesions and has
    to be dissected away from it. Lateral ventricles contain some slight
    amount of cloudy fluid, and the pia along the vessels is opaque.
    Some granulations in ependyma. Brain weight, 1305 grams. Pons and
    cerebellum, 195 grams.

    Cord.—Dura much thickened, and the pia corresponds to its appearance
    in brain with a like exudate. Cross sections of cord show sclerosis
    of posterior columns.

    Bacteriologically the _typhoid bacillus_ was cultivated _from the
    meninges and from the swollen mesenteric lymph nodes_. The blood was
    negative; the intestines were negative so far as lesions were
    concerned.

The microscopic examination confirmed the clinical diagnosis of GENERAL
PARESIS and of TABES, since there was not only an extensive chronic
encephalitis, with the usual lymphocytic and plasma cell deposit and
irregular gliosis, but also a well marked posterior column sclerosis,
not unusual save in its extreme degree.

It might be surmised that some difficulty would arise in distinguishing
the effects of paretic meningoencephalitis from those of the more recent
typhoidal process. The well-known tendency of typhoidal processes to
escape polynuclear exudation, at least until frank necrosis has set in,
gave rise to the idea that the two mononuclear pictures—that of general
paresis and that of typhoidal processes—might be confusing.

The picture presented by the meninges was scarcely what might be
expected. Although numerous mononuclear phagocytic cells are everywhere
found, yet the predominant picture is that of a polynuclear exudation.

The polynuclear leucocytes occur in greatest numbers in the tissue
spaces, especially in the meshes of the lumbar arachnoid and in the
spaces of the frontal and paracentral pia mater. In the lumbar region of
the spinal arachnoid wide fields occur in which the cells are almost one
hundred per cent polynuclear leucocytes. In places phagocytic cells
occur, and in a few fields, even in the open tissue spaces, the number
of phagocytic cells may arise to fifty per cent. Edema is a considerable
feature in the meninges. Fibrin is found chiefly in the cerebral
meninges and appears in numerous delicate strands in the tissue spaces.


    Moloch, horrid king, besmeared with blood
    Of human sacrifice, and parents’ tears;
    Though, for the noise of drums and timbrels loud,
    Their children’s cries unheard that passed through fire
    To his grim idol.

                                Paradise Lost, Book I, lines 392–396




                       IV. MEDICOLEGAL AND SOCIAL


  =Neurosyphilis in a public character: eloquence, reformatory
  efforts, notoriety.=


=Case 83.= Major Isaac Thompson, M.D., was a character. He had been
regarded as eccentric for many years prior to his death at 63. In fact,
it seems that there had been more or less definite symptoms and signs
about his fortieth year. The doctor himself had a ready explanation for
his Argyll-Robertson pupils; he explained that he had had a peculiarly
heavy smallpox at about the age of 27 (which would be about 1872).

The doctor had a good secondary education, he had gone through the Civil
War as a hospital steward, went into business after the war, married,
and then went to the medical school, graduating at the age of 34. He
continued in practice for a dozen years, and then gave it up. For years
he had been especially interested in certain literary lines and he had
published any number of pamphlets, all of a somewhat striking
description, often with a political color and intended to stir up reform
measures. The doctor never bore a very good reputation, and years later
it was recalled that certain books disappeared from libraries and their
loss was almost certainly traced to Dr. Thompson. In general, however,
he was considered to be a rather worthy local figure.

It is possible that a fall on the ice in his 61st year actually started
the fatal process, since after that time the patient had difficulty in
walking, and a few months later developed periods of excitement with
peremptory insistence on obedience to his wishes. Whereas formerly the
doctor had finished up one literary piece of work after another, he now
began to do very scattering work. He appeared in public to denounce
certain financial schemes with great force and unusual eloquence. His
eloquence was greatly complimented, and these compliments induced the
doctor to a remarkable crusade against a certain corporation; there was
so much truth mixed with the fiction of his eloquence that he obtained a
considerable following in his campaign. He wanted to start a bureau of
information for the instruction of the public on these matters, and he
planned to put up a building adjoining his own home for the
accommodation of the various clerks and writers in this bureau. However,
before the building had been actually started, an outbreak occurred.

One morning the doctor was very excitable and noisy over the telephone,
ordering typewriters and giving directions to mechanics. He repaired to
Boston in connection with certain resources that he supposed (and gave
others reason to believe) had been supplied by the Government and by a
large newspaper. One evening he returned very late. It appeared that he
had had a fracas at a hotel and had knocked down one or two colored
porters, acting as though drunk. Upon being put to bed, the doctor
talked incessantly of religious matters, proposing to undertake a Sunday
School class. His interlocutor did not exhibit a particular interest in
this scheme, whereupon Dr. Thompson threatened him with violence. Police
and doctors were called in and a constant stream of conversation lasted
for hours. The patient was finally brought to Danvers Hospital upon
representation by physicians, to whom he told that his luck had turned,
that he was about to be made senator from the district, and that he and
Roosevelt were going to break up the trusts, and that, as a matter of
fact, he was a relative of Mr. Roosevelt.

Upon admission, the patient was a well preserved and well groomed man
with gray hair and beard. He was somewhat pallid but his teeth were well
preserved and well cared for, and there was little or no physical change
except a slight hypertension. He claimed that he had suffered from
kidney disease for some years, and there was in fact a trace of albumin
in the urine.

=Neurologically=, the plantar and Achilles reactions could not be
obtained, but there were no other reflex disorders except the bilateral
Argyll-Robertson pupil. The doctor’s explanation for these stiff pupils,
which he described as existing for many years, was frank and
circumstantial, so that the unlikelihood of Argyll-Robertson pupils due
to smallpox was rather frowned upon by him. Without entering upon a
detailed description of the clinical symptoms and course of the disease
which led to death a little over a year after admission, it may be said
that the differential diagnosis lay between the expansive form of
general paresis and a maniacal condition, presumably the maniacal phase
of manic-depressive psychosis. From the data of a special staff meeting
held upon the case, we learn that the diagnosis of manic-depressive
psychosis was entertained more strongly than that of general paresis.
Thus, for general paresis alone was the somewhat gradual onset with
increasing excitement, accompanied by expansive delusions concerning
unlimited finance, personal over-importance, and Argyll-Robertson
pupils. Dismissing the Argyll-Robertson pupils from consideration, the
diagnosticians were led to see in the constant motor activity displayed
in conveying an enormous number of thoughts on paper, inconsistent
talking with digressions, a manic-depressive psychosis. There was no
amnesia and no other sign of mental deterioration. There was a certain
improvement early in the hospital stay of the patient. Consciousness was
clear and orientation perfect. The delusions themselves, though
extravagant, were not inconsistent or fantastic. The hallucinatory
disorder was hardly characteristic either of manic-depressive psychosis
or of paresis.

The patient might be described as “interesting.” A good preliminary
training with years of travel and variety of occupation, furnished him
with a fund of knowledge. An excellent memory, prompt replies and
repartee, endless digressions with voluntary return to the original
topic, caused him to be an amusing and even instructive interlocutor.
However, his commitment and confinement in the institution seemed always
entirely wrong, and he expressed mixed feelings about the family, now
being bitter against them, and again condoning their mistakes. The
patient’s conduct was good and he was tidy in habits, and tried as far
as possible to conform to the requirements of the hospital. The doctor
showed a marked antipathy toward a certain male attendant, who had
removed articles from his clothing upon admission and had reclaimed a
book on rules and regulations. The doctor prepared a list of 327
different acts of abuse, lack of care, and insubordination which he said
he had observed in the hospital.

In the last weeks of the patient’s illness, his ideas became more
expansive and extravagant, dealing with a grapevine system of wireless
communication and delusions of unlimited wealth. He would at times keep
his room flooded with urine and water for the purpose of keeping down
the plague which he said was infecting the hospital. Later he mixed food
with urine and other ingredients, claiming that he was constructing an
elixir of life.

The =autopsy= showed few changes of the calvarium or of the dura mater,
nor was the pia mater more than slightly thickened and milky over the
frontal poles, along the longitudinal fissure and over the sulci. There
were fairly firm adhesions of the pia mater to the dura mater along the
longitudinal fissure and over the frontal poles and at the temporal
tips. The hemispheres were firmly interadherent, and the
cerebello-pontine tissues were covered with a firm leptomeningitis. The
floors of the ventricles were smooth and the basal vessels showed little
beyond a few spots of sclerosis. There was a generalized increase of
consistence. The frontal gyri were rather prominent with wide sulci, but
upon section no very marked atrophy of the gray matter could be shown.
The rest of the brain failed to show any flaring of sulci or any special
evidence of cortical atrophy. The brain weighed 1250 grams; a possible
diminution of 100 grams, considering the patient’s body length. However,
it must be remembered that he was at this time 63 years of age.

=Microscopically=, the diagnosis of GENERAL PARESIS was confirmed on the
basis of plasmocytosis, lymphocytosis, gliotic changes and nerve cell
destruction. There was an unusual variation in the degree of the
destructive process, which picked out, for example, certain regions of
the right side for maximal lesion (cornu ammonis, gyrus rectus, and
superior frontal gyrus).

If the patient’s own estimate of 35 years’ duration for his
Argyll-Robertson pupils can be trusted (and in general his memory was
extremely good), we may well conceive an unusual duration for the
process in his case. There was, however, in the body at large no very
marked degree of changes. There was a slight old tuberculosis. There was
a slight interstitial nephritis, with cardiac hypertrophy and fibrous
myocarditis. There was also a sclerosis of the mitral and aortic valves;
there were chronic changes in the spleen, liver, and bladder; there was
generalized arteriosclerosis of mild degree; there were two round
gastric ulcers near the pylorus. The liver weighed but 800 grams, and
its left lobe was somewhat rough.

This case is placed among the medicolegal and social cases because the
phenomena that ushered in his last illness were mistaken by the local
public for meritorious social reform measures. They were regarded as not
markedly different from the variety of steps taken by the very active
doctor in previous years; indeed the public eloquence that he displayed
a year before his death was quite in line with previous habits, despite
the suspicious over-brilliance of language. It is an important question,
how far the eccentricity and literary overactivity of the latter half of
the doctor’s total life can be explained on the basis of a mild
syphilitic irritation of the nervous system. In this connection we are
tempted to recall the suggestions of Mœbius concerning a portion of the
literary products of Nietzsche. Our doctor was by no means so brilliant
an exemplar of syphilitic literature as was Nietzsche, if we grant the
hypothesis of Mœbius to cover our doctor’s case as well as that of
Nietzsche. In the future, important studies of character change under
the influence of syphilis will doubtless be made. With modern diagnostic
methods, of course, the diagnosis would have been rendered almost at
once in the case of Major Isaac Thompson, M.D., and much of his past
life would have been brought under special review in connection with the
syphilis which doubtless the blood serum or at any rate the
cerebrospinal fluid would have shown.

This case illustrates but one of the many social complications arising
as the result of paresis. When one recalls that the onset is often
insidious and not correctly understood for a period of time, it is
readily seen that many unfortunate acts may be committed by a patient.
As hypersexual desire is not an infrequent early symptom and as judgment
is early disturbed, loose morals may ruin the patient’s reputation. The
poor judgment and expansive delusions often lead to foolish business
deals wherein the patient’s family is left destitute. At other times the
onset is sudden and then the danger of false commands or acts by a
person in a responsible position, as a steamship captain, an engineer or
chauffeur, may lead to loss of life and property.


  =Sudden grandiosity: debts. PARETIC NEUROSYPHILIS (“general
  paresis”): Question of liability.=


=Case 84.= Lester Smith was a salesman, 31 years of age, who, while on a
business trip, accompanied by his wife, suddenly developed grandiose
ideas. He originated a scheme of cornering the phonograph market. His
prospects seemed so certain to him, that he hired an expensive suite of
rooms in a hotel at something over $35 a day. As at the first
presentation of his bill it was found that he had no money to meet these
charges, he was taken into custody and at once transferred to a hospital
for the insane, where it was discovered that he was suffering from
GENERAL PARESIS.

    1. What is the patient’s responsibility for these debts? Legally the
      patient or his estate is responsible for debts accruing from
      services rendered or goods received. As he is adjudged _non compos
      mentis_ contracts entered into would not hold, and he would not be
      considered liable for criminal acts.

Note: This case shows how dangerous paresis may be not only to the life
and usefulness of a patient, but further how it may ruin a family
financially. Mr. Smith’s little escapade used up all the money that he
had been able to save in his life and when he was taken to a hospital
his wife was left destitute.


  =Suicidal attempt (?) by a neurosyphilitic.=


=Case 85.= At first Mrs. Annie Monks, a widowed seamstress, 50 years of
age, did not particularly suggest syphilis. Mrs. Monks was sent to us
from a general hospital. She had been found unconscious in her room,
with gas turned on, and a diagnosis of gas poisoning was made. Mrs.
Monks remained unconscious for 24 hours, and her apparent suicidal
attempt seemed to warrant her being sent to the Psychopathic Hospital.
Mrs. M., however, scoffed at the idea of any attempt at suicide, and
claimed to have had no recollection of any such affair. On the contrary,
she had gone to mass the morning of the day on which she was taken to
the hospital, remembered well enough returning to her room but nothing
of what followed until she woke up.

Mrs. Monks was not coöperative and would reveal few facts about her
history. For years, she had had edema of the feet and palpitation of the
heart (the heart was somewhat enlarged, with a double murmur in the
aortic area, systolic louder, and a blood pressure of 160 systolic and
85 diastolic; clubbed fingers; palpable liver). She had been treated in
the out-patient department of a general hospital for a number of months.
We could obtain no evidence of mental impairment, particularly none of
memory.

Aside from the heart lesions above indicated, the patient was fairly
well nourished, with a slight enlargement of superficial glands, and was
otherwise normal.

=Neurologically=, the slightly irregular pupils reacted poorly to light;
the right knee-jerk could not be obtained, whereas the left knee-jerk
was very active. Systematic examination revealed no other disorder
except that the abdominal reflexes could not be obtained.

Here we have, in a cardiac patient, a possibly or probably accidental
gas poisoning, and little to go upon for a profounder diagnosis than the
sluggish irregular pupils and unilateral absence of knee-jerk.

The routine serum W. R. came through as positive. Following custom, we
examined the spinal fluid, finding the W. R. here again to be moderately
positive (strongly positive to 1 cc., moderately to 0.7 cc., and
negative to 0.5, 0.3, and 0.1 cc.). The gold sol index was 1 2 2 1 0 0 0
0 0 0, which must be interpreted as syphilitic. There were 16 cells to
the cmm., the albumin was 1+, and the globulin stood at 2+.

Here, then, we seem to have evidence of an inflammatory process of the
central nervous system, and it is natural forthwith to be sceptical as
to the accidental nature of the gas poisoning. Perhaps there was an
attempt at suicide based upon a passing impulse, or perhaps there was a
period of confusion in which the cock was not turned off.

In any event, we feel justified in making the diagnosis of cerebrospinal
syphilis on the basis of the neurological and laboratory findings. On
the whole, we are inclined to make a diagnosis of VASCULAR NEUROSYPHILIS
with a moderate involvement of the MENINGES.

    1. What is the outcome in such cases as that of Annie Monks? The
      case somewhat resembled that of Martha Bartlett, who still
      survives. The case of Annie Monks illustrates another outcome. A
      few days after her admission, she became unconscious once more,
      and upon recovery remained very much confused and aphasic,
      moaning, and unable to handle herself well, although without
      definite paralysis. Three weeks later the patient died, although
      in the meantime strenuous antisyphilitic therapy was practised.
      Death was sudden. We thought death due to cerebral embolism.


  =Early delinquency and neurosyphilis in a juvenile.=


=Case 86.= Frank Johnson was 21 years of age when he was taken up by the
police for threatening his sister with a revolver. The police thought he
deserved an examination at the Psychopathic Hospital. The patient
protested that he had threatened his sister only to frighten her
because, he said, she nagged him and made him nervous. In fact, they had
always had trouble as she had always nagged him and they had always
fought together. Moreover, their mother always took the sister’s part.
They had been troubling him for days, and at last Frank could stand it
no longer. His sister had complained of the way he treated her dog.
Moreover, Frank said he had not been feeling well; there had been some
trouble with his stomach; and after one of the nagging attacks, he had
taken out an old empty pistol to scare his mother and sister.

In these cases, it is good practice to consult the sister also. She said
that Frank had always been very difficult to manage, unwilling to work,
preferring to loaf about, spending every obtainable cent; he was once in
a reformatory for several years, but not reformed thereby; recently
given to drinking; at times acting somewhat peculiarly (sitting at the
window with his hat on, refusing to move).

Further =mental examination= of Frank showed that he was properly
oriented and in possession of a good memory, although he was quite
obviously a liar. He lay about in bed at the hospital, saying that he
was too weak to be up. He was a bit dull, at times not readily grasping
ordinary questions.

=Physically=, Johnson was rather thin; the teeth were somewhat
peg-shaped although far from typically Hutchinsonian. The pupils were
unequal and irregular, and failed to react to light or even to
accommodation when tested. The deep reflexes of arms and legs could not
be obtained, though the superficial reflexes were present. For the rest
systematic examination proved negative. Serum W. R. negative.

The first thought in such a case would be that the criminological
diagnosis of delinquency would be sufficient. However, the pupillary
disorder and the areflexia are suggestive despite the negative serum W.
R. Resort was naturally had to lumbar puncture, whereupon a positive W.
R. was found, a characteristically “paretic” gold sol reaction,
globulin, excess albumin, and 134 cells per cmm. In short, it would
appear that we must consider a diagnosis of JUVENILE PARESIS, and, in
point of fact, the patient deteriorated rapidly from this time, becoming
demented at the end of a few months.

    1. How far are the early difficulties of management (leading to a
      reformatory) due to syphilis? We should not dogmatically say that
      there is a relation between the early delinquency and syphilis.
      Still, it is not unusual to find emotional disorder and
      instability as well as delinquency in congenital syphilitics.

    2. What suggestion, if any, should be made to the patient’s
      intelligent and seemingly normal sister, two years older? We
      prevailed upon Miss Johnson to submit to the W. R. of the serum,
      which was found, as in the case of Frank, to be negative. Frank’s
      sister should undoubtedly submit to a lumbar puncture; but in the
      present phase of mental hygiene, she would be difficult to
      persuade.

    3. How is it possible to find such a marked evidence of congenital
      syphilis in a younger sibling with no evidence of syphilis in the
      elder? In the first place, there may be a history of entrance of
      syphilis into the lives of the parents between the pregnancies.
      However, in other instances, there is no evidence of such
      intercurrent syphilis, and contrary to the prevailing opinion it
      is not so infrequent to find congenital syphilis in the younger
      brother or sister of a normal person.

    4. What can be said of treatment in such cases? In the first place
      it is clear that delinquent cases should be tested far earlier for
      the possibility of syphilis. Had this case been examined by a
      neurologist or alienist many years earlier, it is probable that
      the same pupillary signs and the peg-shaped teeth would have been
      found, and that the hypothesis of syphilis might have been raised.
      There is no good evidence as yet that these cases can be markedly
      benefited by treatment.


  =Neurosyphilis in a “defective delinquent.”=


=Case 87.= Vivian Walker, 22 years of age, was arrested on the streets
of Boston for drunkenness. Upon arrival at the jail, she developed a
series of convulsions, each lasting a very brief time, with loss of
consciousness, frothing at the mouth, and jerky movements of the arms
and legs.

The Walker family was known to the police, since there were police
records in two generations on the maternal side. The father was regarded
as of rather low-grade mentality; a sister had committed suicide. Vivian
herself had been irregular at school, was regarded as vicious, and had
been hysterical. She had been committed to a reformatory at the age of
15 years. In the reformatory she had a number of excited outbreaks, with
resentment of discipline, and these outbreaks presented hysterical
traits. After each outbreak Vivian was depressed. It was during her stay
at the reformatory that her sister committed suicide. Vivian attended
the funeral, and the idea of suicide appears to have taken hold of her
mind, as she constantly spoke of suicide, threatened suicide, and made
several attempts. She claimed at this time to see visions and to hear
her sister’s voice. On that ground she had been committed to a hospital
for the insane at 16.

At the hospital there were many fluctuations in mental condition. Vivian
professed discouragement on account of poor home influences, telling how
her mother had often been in prison, allowing Vivian to come under the
influence of bad girls. Now and then Vivian had outbreaks of profanity
and glass-breaking, and she also made at the hospital for the insane
several half-hearted attempts at suicide. At the age of 19 she was
returned to the reformatory, whence she was placed out on probation and
allowed to return home.

However, she was shortly re-committed to the insane hospital in a phase
of excitement, talking continuously of men and sex relations, and also
of imaginary illicit sex relations with any man whom she happened to
see. Again from time to time she made attempts at suicide. However, she
was allowed to go out on visit, returned to her habits, and at the time
of her arrest was living as a prostitute.

After her convulsions in jail, she was admitted to the Psychopathic
Hospital. At first obstinate and stubborn, later she became tractable.
Special mental tests left her in the subnormal class, but we could
hardly class her as feebleminded. We were able to observe her in a
number of seizures, during which she would drop to the floor, apparently
lose consciousness, writhe about, and assume the position of
opisthotonos, the whole attack lasting but a minute or two.

There was pelvic tenderness, with gonococci in the urethral smear.
Salpingectomy had to be performed, but after the operation Vivian
insisted upon getting up and running about on the second day, tearing
the bandages from her abdomen, and infecting the wound. Outbreaks of
excitement also followed the operation.

In the diagnosis of this case, we must probably separate the convulsive
phase from the remainder of the phenomena. The conduct disturbance,
emotional outbreaks, and suicidal attempts date from early youth, and no
doubt the diagnosis defective delinquent would fit Vivian from the
beginning. The hereditary taint is characteristic enough. The sundry
phenomena in the insane hospital, and particularly the hallucinations,
lead one to wonder whether Vivian is not possibly even suffering from
dementia praecox.

As to the convulsions, it would hardly appear that they are typically
epileptic, although certainly epileptoid. Their onset at 22 is somewhat
unusual. Several features of the seizures together with the opisthotonos
and the previous history of hysteria, lead one to think of making the
diagnosis hysteria.

    1. Can cerebrospinal syphilis cause the symptoms? We found the serum
      W. R. to be positive though Vivian denied syphilitic infection.
      (She also denied gonorrhœal infection despite the clinical and
      laboratory findings.) We found that the spinal fluid yielded a
      gold reaction of a typical syphilitic nature, showed an excess of
      albumin, a slight amount of globulin, and 130 cells per cmm. Even
      these findings, however, would perhaps not justify stating that
      the convulsive seizures are of syphilitic nature. The seizures
      disappeared under the administration of antisyphilitic remedies.
      It would seem, therefore, that the seizures should be regarded as
      of syphilitic nature. In any event, the diagnosis of cerebrospinal
      syphilis is justifiable. This syphilis, however, is of an active
      nature and probably of recent production. We should be at a loss
      to explain the earlier mental features in Vivian as syphilitic and
      are therefore fain to associate the two psychoses, PSYCHOPATHIC
      PERSONALITY and DIFFUSE CEREBROSPINAL SYPHILIS.


  =NEUROSYPHILIS (“paresis sine paresi”) in an habitual criminal, a
  forger.=


=Case 88.=[17] —— was brought to the hospital by the police. He was
charged with having forged a check, and on account of the crudeness of
the work his mental condition was suspected.

=Family History.= The paternal grandfather was considered fast, drank a
great deal and was said to be a thief. The father is said to have been
forced to leave the State when a young man in order to avoid the
reformatory. Paternal cousin murdered a man; the sisters of this cousin
said to have been wild and one brother married a prostitute. Nothing
known of maternal relatives.

=Past History.= Medical history is unimportant. He denies syphilis. His
early childhood is of little significance. He was somewhat dull in
school. At about the age of twelve he began to lie and steal, and has
continued this ever since. His attempts have all been very crude, it is
said, and when confronted he would strenuously deny his deeds, even when
the evidence was overwhelming. He forged checks, borrowed money from all
his friends, and charged things at stores to the family. The family paid
the bills for a time, and then later had him sent to a reform school. He
was married at nineteen, but wife has left him and obtained a divorce.
He has been excessively alcoholic for years, and is suspected also of
taking drugs. He was discharged from the navy dishonorably. He later
joined the army and was discharged therefrom on account of “rheumatism,”
according to his account, but in reality deserted. He had finished a
jail sentence of thirteen months for forgery a little over a year before
entrance.

=Physical examination= shows a well developed and nourished man. The
general physical examination is negative. The lungs show nothing
abnormal. The heart is not enlarged, there are no murmurs or
irregularities; blood pressure, 145 systolic. The alimentary system is
negative. No palpable lymph glands. =Neurological examination=: pupils
equal and react to light and accommodation. Extraocular movements well
performed. Tongue projects in the median line, with no tremor. There is
no evidence of facial paresis or weakness of the muscles. The biceps,
triceps, knee-jerks and ankle-jerks are present and equal on the two
sides. There is no Gordon, Babinski or Oppenheim; no ankle clonus. There
is no tremor of the extended hands. No Romberg sign. There is a little
difficulty in the finger-to-finger test. There is no sensory disturbance
either subjective or objective. No tenderness over nerve trunks.

=Mental examination= shows nothing of a psychotic nature. Patient is
well oriented; memory for remote and recent events is well preserved,
school knowledge well retained, grasp on current events good; no
delusions or hallucinations elicited. Patient is not feebleminded,
according to the intelligence tests of Binet and Simon and
Yerkes-Bridges, but shows poor attention and gives evidence of weakness
in volitional spheres; is very suggestible.

To summarize the case, then, we have a man of thirty years of age who
has shown criminalistic and anti-social tendencies since childhood,
whose general physical and neurological examination is negative
(excepting the laboratory tests), whose mental examination shows no
psychotic symptoms, and who seems not feebleminded. In other words, with
the exception of the serological and chemical findings in the blood and
cerebrospinal fluid, there is nothing to suggest that he is more than a
“criminal type.”

Wassermann reaction in blood serum positive.

Wassermann reaction in cerebrospinal fluid positive. Examination of
cerebrospinal fluid: globulin ++, albumin ++, cells 55 per cubic
millimeter; large lymphocytes, 9.1 per cent; small lymphocytes, 90 per
cent; plasma, 90 per cent. Gold sol reaction, 3321000000.

    1. Can the criminalistic tendencies be condoned in this case on the
      ground of neurosyphilis? As a matter of fact the delinquencies in
      this patient reach back to early childhood and as there is no
      evidence of congenital syphilis it cannot be held that syphilis
      had any bearing in the causation of symptoms. Even were the
      delinquencies only of recent date it is doubtful if the court
      would take cognizance of the laboratory findings in the absence of
      definite mental symptoms. In this connection it may be stated that
      the court takes cognizance only of the acts of a patient at time
      of examination, and not of the history or laboratory findings, in
      committing a person. We have had several patients who from
      history, physical signs and laboratory tests made the diagnosis of
      paretic neurosyphilis easy and yet who could not be committed
      because they were mentally clear at the time. Such patients may be
      of grave potential danger to themselves and families, and present
      numerous social problems. See case of Joseph Wilson (95).


  =JUVENILE PARETIC NEUROSYPHILIS (“juvenile paresis”) with initial
  trauma.=


=Case 89.= Margaret Tennyson was a small girl of six years, described as
having been normal until run down by a double-runner sled about 13
months before her arrival at the hospital. The change was stated to be
remarkable. “She was as unlike her own self as darkness and daylight.”
Once fat and sunny, talkative and demonstrative with her toys, now
Margaret had become silent, sullen, worried, and of a violent temper,
stubborn and unmanageable. It does not appear that the patient was
seriously injured by the double-runner, as she was able to walk a short
distance home. Shortly, however, she began to have trouble with her feet
(diagnosed at the time as flat-foot), and thereafter her whole character
and disposition changed. Upon arrival at the hospital, the patient
walked with a typical scissors gait of spastic paraplegia.

=Physical examination= was very difficult through lack of coöperation
and a screaming and kicking resistance upon every attempt. There was a
suggestion of hydrocephalus in the protrusion of the forehead. The
pupils reacted readily to light and accommodation. The knee-jerks were
active, but there was otherwise no disorder of reflexes. The patient had
great difficulty in getting up from the floor, and for the most part
insisted upon lying in ventral decubitus on the floor, crying when
attempt was made to raise her. An attempt was made to test her by the
Binet scale, by which she was found to rate at 2⅘ years although a
portion of this low-rating was thought to be due to a failure of
coöperation.

The =family history= threw little or no light upon the case. The parents
were living and well; a brother of 16 years was at work in the market
district; two of the other siblings are in the first and second grades
at school and regarded as exceptionally bright by their teachers. The
fourth was the patient, Margaret; a fifth had died at 9 weeks of heart
trouble; the sixth, seventh, and eighth, of 3, 1½ years and 3 months
respectively, appeared entirely well. There were no miscarriages or
stillbirths.

[Illustration:

  Juvenile paresis—spastic paraplegia. 5 years.
]

The scissors gait and spasticity seem to point undoubtedly to organic
disease of the nervous system, along with which the mental deterioration
seemed to suggest an active progressive involvement of the cerebrum. The
history seemed to be convincing that the child was not an instance of
congenital feeblemindedness.

A neurologist’s clinical diagnosis would naturally be syphilis. In point
of fact, this diagnosis was borne out by the laboratory tests, which
showed a positive W. R. in the serum and spinal fluid, positive
globulin, a slight excess of albumin, and a syphilitic gold sol
reaction.

    1. What is the significance of the trauma in the case of Margaret
      Tennyson? The trauma seemed to the family the precipitating cause.
      We find cases of general paresis in adults very definitely
      following trauma, yet neurosyphilis, both in adults and in younger
      patients, mainly occurs without trauma. On the whole, in this
      case, it is perhaps safer to regard the trauma as mere
      coincidence. A sister older than Margaret was found upon
      examination to have a positive W. R. The other children could not
      be examined.


  =Traumatic form of PARETIC NEUROSYPHILIS (“general paresis”).=


=Case 90.= The point about Joseph O’Hearn was his entire mental
soundness up to the time of an injury at work, when he was blown through
a double window in an explosion, badly bruising his head. Shortly after
the accident, although not immediately, the patient began to show signs
of mental disorder, doing very foolish things, losing his memory, and
becoming unable to work.

It was eight months after the explosion when O’Hearn, at the age of 36,
was admitted to the hospital with general mental impairment. O’Hearn was
confused and disoriented for time and place, although he seemed to
understand that he was in a hospital. He was given to foolish laughter
and a silly manner. There was considerable emotional disorder; judgment
was clearly impaired, and memory was poor.

=Physically=, there was little to be found except upon =neurological
examination=. The right knee-jerk was greater than the left; the tongue
and fingers showed marked tremor, there was a speech defect and writing
disorder.

On the whole, it seemed impossible not to make the diagnosis GENERAL
PARESIS, especially in view of the laboratory tests, with positive W. R.
in both serum and fluid, a “paretic” type of gold reaction, 59 cells per
cmm., excess albumin, and a large amount of globulin.

    1. What is the relation of the trauma to the paresis? Trauma is
      regarded as a precipitating cause, and Industrial Accident
      Commissions have been known to allow damages in such cases. Mott
      believes that the symptoms of a post-traumatic paresis must not
      develop until after a week’s interval of freedom from symptoms,
      since he believes that time is required to destroy or irritate the
      brain to the point of producing the paretic picture. Our data are
      in agreement with those of Mott. Mott also points out that gumma
      sometimes occurs at the site of the trauma.


  =False claim for compensation in neurosyphilis.=


=Case 91.= The facts in the case of Levi Sussman can be brought out by
the following extracts from a report to the Industrial Board: A claim
was made to the Board that the symptoms had developed after a fall from
a building, some _nine months before hospital observation_. No
connection could be found between this accident and the PARETIC
NEUROSYPHILIS found. We introduce the case to emphasize the possibility
that irrelevant accidents may be regarded by ignorant or unscrupulous
persons as setting up a mental disorder for which damages are claimed.
If symptoms are already in existence before the accident and are not
especially increased thereafter, naturally no damages should be
recovered. Unscrupulous persons may falsify about the pre-traumatic
history and claim the development of symptoms immediately after the
accident. Such claims are beyond question to be viewed with the greatest
suspicion. Some days or weeks should elapse before definite symptoms in
post-traumatic paresis appear. Just how long an interval may elapse
between trauma and paretic symptoms and shall entitle the case to be
regarded as one of traumatic paresis, is perhaps a matter of doubt. It
would seem, however, on general grounds that three months is the longest
period in which the post-traumatic effects are likely to be delayed.

The question of traumatic paresis is of great interest on account of the
war. The great strain under which the men at the front live and the
physical injury due to being “buried” is probably responsible for an
increasing number of cases of neurosyphilis. Such at least is the
impression of Canadian medical officers with whom we have spoken. See
Section VI, Neurosyphilis and the War.


  =Traumatic exacerbation(?) in PARETIC NEUROSYPHILIS (“general
  paresis”).=


=Case 92.= The case of Joseph Larkin was of note from the point of view
of the Industrial Accident Board. This Irish teamster was said to have
been injured in his head two or three months before coming up for
examination at the age of 45. For a week Larkin had had frontal
headaches, had been sleeping poorly, and had been somewhat worried. In
fact, he had stopped work. The W. R. of the serum was positive and a
diagnosis of PARESIS could be made. The case did not come up for
consideration by the Industrial Board until two years after his initial
appearance.

The =physical examination= showed irregular pupils, sluggish pupillary
reactions, Achilles absent, swaying in the Romberg position, enlargement
of the heart to the left, positive W. R. of the blood and of the spinal
fluid.

=Mentally=, the patient’s orientation for place was poor and his memory
defective. Emotionally he was depressed or apathetic and was
apprehensive. His flow of thought was slow, and his insight into his
condition poor.

It is interesting that a variety of causes have been assigned in this
case for the condition: such as, his work, anemia, unhygienic
surroundings, and arteriosclerosis.

This case is not a sharply-defined case of post-traumatic general
paresis, since there had undoubtedly been a variety of mental changes
before the accident. Accordingly, recovery of damages to a full amount
could hardly be expected as in certain cases in which the phenomena of
paresis appear only after the trauma.

[Illustration:

  Post-traumatic cranial gumma—developing 13 months after local injury
    of skull.
]


  =Trauma: syphilitic lesion of skull at site of injury.=


=Case 93.= The medicolegal interest of Richard Marshall is extreme, as
may be seen from the following brief report by the Psychopathic Hospital
to the Industrial Board.

    “As to the case of Richard Marshall, a patient under the provisions
    of the temporary care act from December 1 to December 10, inclusive,
    this case has proved unusually interesting in that the patient has
    proved to be syphilitic by the Wassermann reaction of the blood.
    There is no evidence of syphilis in the examination of the
    cerebrospinal fluid. The X-ray examination of the skull, taken in
    connection with the Wassermann reaction of the blood, warrants the
    diagnosis of syphilitic osteitis of the skull at the site of the old
    injury. We regard his present condition as shown by the X-ray as a
    syphilitic bone condition predisposed to by the injury. We do not
    find that the patient has any features of traumatic neurosis.

    “Mentally, having an actual age of 30, patient grades at 11.2 years.
    It may be that patient has always been a moron. He has earned about
    $8.30 a week.

    “We regard the patient as deserving treatment and feel that
    responsible parties in the case would do well to have such treatment
    instituted.”

The principal symptom of which Marshall complained was headache chiefly
felt in the region of the osteitis. There was marked sensitiveness to
percussion in this area. It is of course difficult to decide whether the
headache was entirely due to the gummatous lesions or whether the trauma
had caused contusions of the brain as well. It is also possible that the
dura underlying this area was involved.


  =OCCUPATION-NEUROSIS in a granite-cutter: SYPHILITIC NEURITIS?=


=Case 94.= David Fitzpatrick was a case referred to the Psychopathic
Hospital by the Industrial Accident Board. He was a granite-cutter of 52
years of age, and had begun to complain of pain in the forearm,
extending back from the elbow, about six months before admission. It
seems that the patient had been growing progressively worse and had
thought he would have to quit work because of difficulty in grasping the
hammer. A physician had told him that he must stop his work at
granite-cutting or else he would entirely lose the use of his arm. He
was in point of fact laid off because of slackness of work and had been
unable to get work again. The pain in the arm, however, had continued
and at times was very severe. Sometimes the pain and the worry led to
insomnia. Fitzpatrick wanted the insurance company to pay certain
accumulated bills, and maintained that he would be able to do work at
$15 a week if work could be found for him. The general situation in this
case can be gathered from the following abstract from the report to the
Industrial Accident Board.

    “Secretary Industrial Accident Board,
      “Dear Sir:
        “_In re_ David Fitzpatrick

    referred to us with a copy of an impartial report filed by the
    Massachusetts General Hospital,—we concur with said impartial report
    that there is now no evidence of paralysis of the arm. We do not
    find that the positive Wassermann reaction, although it indicates a
    history of syphilis, has affected the patient other than possibly to
    have reduced his general mental capacity. Our special tests yielded
    a percentage of 62% of what a patient of his age and station should
    possess. There seems, however, to be no connection between this
    reduction of mental capacity and the difficulty with the arm. We
    cannot connect the history of alcoholism with the arm trouble.

    “There is some evidence that other stone workers have at times shown
    such effects.

    “The patient’s fairly circumstantial account of his difficulty seems
    to point to a degree of myalgia or muscular pain in the region of
    the forearm when held in a certain position and a feeling of
    numbness in the third and fourth fingers. Whether these phenomena
    are due to local pressure upon nerves in the upper part of the
    forearm due to neuritis, or whether we are dealing with a functional
    neuralgic phenomenon is a question.

    “We have applied some special tests for faradic sensibility to all
    the fingers of both hands and have found that the fingers of the
    right hand are still less sensitive than those of the left,
    particularly the thumb and the little finger. This test has not yet
    been applied in a sufficiently large number of cases to prove any
    difficult point, nevertheless the findings are in line with the
    patient’s own circumstantial account of former feelings of numbness
    in the third and fourth fingers of the right hand.

    “Obviously, then, our opinion is that there is still to be found
    some effect of the disease, whatever it was, which caused the
    patient to knock off work. If we had more experience with such cases
    and more data with the new test which we have applied, we should
    perhaps be inclined to admit the diagnosis of _occupation neuritis_
    and to suppose structural alterations in the nerve trunks
    corresponding with the location of the muscular pain and the
    anesthesia of fingers and the dulling of electric sense, but in the
    present stage of our experience, it is probably wiser to call the
    case one of _occupation neurosis_.”

It is clear that the W. R. in this case was of peculiar value in at
least partially clearing up the findings, yet it must be remembered that
it is a principle of the modern administration of industrial accident
boards and similar organizations that it is the employer’s lookout
whether the employee has syphilis. Recovery can be made as if the injury
were due wholly to an accident. It was not possible however definitely
to prove or disprove a relation of syphilis in the form of a syphilitic
neuritis to the condition in this case.

The special tests above referred to are the electric sensory threshold
tests of E. G. Martin.


  =Character change: neurosyphilis.=


=Case 95.= Joseph Wilson offered a very serious social problem. He was
the father of two children, and his wife was pregnant at the time of his
admission to the Psychopathic Hospital. He was a husky-looking man of 33
years of age, but for the past four years he had been deteriorating in
his work; he had been drinking heavily, and finally had stolen to obtain
money for liquor. It was on account of his alcoholism and delinquency,
which were taken as an indication of change of character, that he was
sent to the hospital.

Examination on his arrival disclosed at once that there was more to the
case than alcoholism, for the =neurological examination= showed that the
pupils were irregular, the right being larger than the left, both
reacting sluggishly to light, and there was an inequality in the
reaction of the two eyes, the left being better than the right. The
tendon reflexes were exaggerated, with ankle clonus on both sides, more
marked on the right. There was also a marked speech defect. Otherwise
the =physical examination= showed nothing of importance.

The W. R. of the blood and spinal fluid was strongly positive. The
globulin test was strongly positive, the albumin was markedly increased,
there were 74 cells per cmm., and a gold sol reaction of the “paretic”
type.

A formal =mental examination= did not show very much of consequence; his
memory showed no marked impairment, he was not deluded or hallucinated,
and he had a pretty good insight into his failings. However, he was
somewhat childish, and his irritability was quite marked. Were one to
rely upon the mental signs alone, it is probable that a diagnosis of
chronic alcoholism with deterioration would be made; but in the presence
of the physical findings and the laboratory tests, the diagnosis of
neurosyphilis had to be given. It is obvious that, while the patient was
suffering from a progressive brain disease, and while he did show mental
symptoms, there was not sufficient ground on which to commit him, and
therefore he had to be turned out into the community. As a matter of
fact, he was not prosecuted on account of his theft, because, although
legally responsible, it was felt that his disease was at the basis of
the character change which had led him into difficulties. Further
developments of his relations with society had to be considered,
however. It was possible to get him to discontinue the use of alcohol
altogether, and for nearly a year he has taken no alcoholic liquor and
has been self-supporting. However, his irritability has been very great,
making it very difficult for his wife to live with him, and causing his
sister to break off all relations with him.

Here, then, is a man with a marked CHARACTER CHANGE as the result of
neurosyphilis, so that it is difficult for him to maintain the usual
social relations. It does not seem possible to remove him from the
community.

    1. May one speak of general paresis without mental symptoms? If one
      considers general paresis a mental disease, of course it cannot
      exist without mental symptoms. However, if one considers the
      disease as a chronic syphilitic meningoencephalitis characterized
      by its pathological anatomy, then one may readily speak of general
      paresis although no real evidence of mental symptoms can be
      discovered. It would seem that we must take this attitude with our
      present conception of brain localization, for it is easy to
      conceive of a general paretic process affecting areas which do not
      definitely relate to psychic function. And further, such a process
      may exist but not be of such a grade as to cause mental symptoms.


  =The neurosyphilitic’s family should not be forgotten in diagnosis
  and treatment.=


=Case 96.= The Bornstein family is remarkable. Let us hang the story on
Becky, the mother, an Austrian woman of 43 years, who appears to have
been perfectly well up to within a year. About a year ago, Mrs.
Bornstein began to suffer from severe headaches, which were treated with
apparent success by an osteopath: at all events, Mrs. Bornstein
recovered therefrom in about six months. However, two months later, she
had a convulsion, with foaming at the mouth, blueness of face, and
general muscular stiffening. The convulsion lasted for several minutes.
Again, a fortnight before admission, the patient had five convulsions of
an identical nature in a single night.

Moreover, since the first convulsion, Mrs. Bornstein’s =mental
condition= has altered and become variable, so that at times she is
excited, at times depressed. She would assert inaccurately that there
was some one in the house, and that she had at different times committed
crimes of a heinous nature. Now and then she would seem to see moving
pictures. Her memory was poor and she seemed to believe that events of
five or six years ago had just happened.

The pupils were sluggish, the knee-jerks and ankle-jerks were absent,
there was slight ataxia, and there was speech defect. The suspicion of
neurosyphilis was so strong that it seemed surprising that the W. R. of
the blood serum, even after repeated tests and after the provocative
injection of salvarsan, proved negative. However, the spinal fluid
yielded a positive W. R., and a gold sol reaction of the “paretic” type,
together with 12 cells per cmm., and a marked increase of albumin, with
positive globulin. It would seem warrantable to make a diagnosis at
least of syphilis of the nervous system in this case, but it is a
question whether we should be warranted in making the diagnosis general
paresis.

That the diagnosis is doubtful may perhaps be seen from the variety of
diagnoses in the rest of the family. In the first place, Mrs.
Bornstein’s husband admits syphilitic infection many years before. He
states also that his wife after marriage showed signs of syphilis and
received some treatment, although limited. It is stated also that the
husband himself at this time has a positive W. R. and has stiff pupils
and petit mal attacks. The oldest son, 22 years of age, is confined in
an institution with juvenile paresis. The second son has recently died
at the age of 20 years, receiving a diagnosis of rupture of the aorta. A
third son, 19 years of age, has the appearance of having achondroplasia,
although the proportions of his limbs do not quite correspond with those
of an achondroplast. The fourth son, 17 years of age, is suffering from
caries of the spine. A fifth son, 14 years old, is neurotic and has the
so-called Olympic forehead. The sixth and last son died shortly after
birth of unknown cause.


  =Neurosyphilitic’s normal-looking family proved syphilitic.=


=Case 97.= Walter Heinmas was a draughtsman 33 years of age when he was
brought to the Psychopathic Hospital suffering from mental disease. This
was diagnosed as general paresis, both on account of the clinical
symptomatology and on account of the laboratory findings. In fact, it
was a case of the classical type with marked euphoria and grandiosity.

As is the routine procedure at the Psychopathic Hospital, in the case of
all syphilitic patients, the family was sent for. This consisted of the
wife and two daughters, aged 9 and 7 respectively. The patient denied
any knowledge of a syphilitic infection. The wife, also, gave no history
of any primary, secondary, or tertiary symptoms; there had been no
abortions, miscarriages, or stillbirths; both children had been born at
term and had been entirely healthy. Examination showed that the mother
had no signs referable to syphilis, and that both the children were
mentally well endowed, with good physique and showing no stigmata of
congenital syphilis. Still the W. R. of all three (the mother and the
two children) was positive in the blood serum. These tests were repeated
several times on the children, with and without injections of salvarsan,
and they remained consistently positive.

    1. Are these children to be considered congenital syphilitics
      despite the absence of stigmata or symptoms? We must consider
      these children as congenital syphilitics and candidates for the
      group frequently spoken of as _syphilitis hereditaria tarda_.

    2. What is the frequency of syphilitic involvement in the mate and
      children of paretics? In our series, we have found that about 15%
      of the marriages where one member develops paresis, result in
      sterility; that in 18% abortions, miscarriages and stillbirths
      occur; and that in 15% positive W. R. is obtained. We have adopted
      the motto: “=The families of paretics are the families of
      syphilitics.=”


  =Neurosyphilis: question of marriage.=


=Case 98.= Mr. Jacobs’ wife was admitted to the hospital with a
diagnosis of general paresis. A few weeks after her admission, she died
as a result of her disease. According to our routine, her husband and
the children were examined for evidences of syphilis.

Mr. Jacobs’ blood serum was found on repeated tests to be positive. He
resolutely denied any knowledge of a syphilitic involvement, but it was
later learned from his brother that about two years before his
marriage—that is, more than 25 years before we saw him—he had acquired
syphilis and had had a very small amount of treatment.

Mr. Jacobs was put upon antisyphilitic treatment in the form of
injections of .3 gram of salvarsan every two weeks with occasional
intramuscular injections of mercury salicylate. After seven months of
treatment, the blood serum still remained positive. At about this time,
the patient came to us to ask about getting married again. He said that
he was living with his sister, who kept telling him that he was the
cause of his wife’s death, and this was so unpleasant that he desired to
start a home for himself again!

    1. What advice should be given? It is a general opinion that the
      longer the period after the initial infection, the less the
      chances of infecting a partner. This chance is further reduced
      under antisyphilitic treatment, of which a considerable amount had
      been given in the case of Mr. Jacobs. However, when one considers
      the trickiness of syphilis and the fact that there is some chance
      of infection, which we would apparently overlook if we gave him
      permission to marry at this time, the only possible course was to
      tell the patient that he should not consider marriage until his
      Wassermann had become negative and remained so for some time. The
      children in this case were negative.

    2. What is the physician’s duty to the family of a syphilitic
      patient? It is our firm conviction that it is the duty of every
      physician to his syphilitic patient, to the patient’s family, and
      to the community, to examine the mate and the children for
      evidence of syphilis acquired or congenital and to offer treatment
      if it is found to be needed. This is one of the chief means at our
      disposal today to prevent the late disasters of syphilis, acquired
      or congenital, for by such examinations the syphilitic condition
      is discovered before lesions have occurred which are irreparable.
      We know that the mate and children of a syphilitic patient have
      been exposed to syphilitic involvement, and it is our duty as
      physicians in possession of such knowledge, and as guardians of
      the public health, to investigate such cases, so that if they be
      found to have syphilis, steps may be taken to treat them early.

    3. How much danger is there of causing unhappiness and breaking up
      families by this procedure? This question offers a chance for many
      theoretical answers. The facts are, however, that in doing this as
      a routine for nearly three years and examining several hundred
      families, there has been no instance to our knowledge in which a
      family has been broken up or grave difficulties have been
      encountered by this procedure.

    4. In what percentage are the mates or children of neurosyphilitics
      found to show definite symptoms of syphilis? It is our opinion
      that the situation in regard to neurosyphilitics is the same as
      for syphilitics in general: That the same laws of attenuation of
      virus, and of chance occur here as elsewhere.

Just as this book is going to press, we have learned that the distraught
Mr. Jacobs, still desirous of starting a home for himself and feeling
entirely well, consulted a physician. This physician took a sample of
blood and had it tested at a competent laboratory, which reported the
blood negative.

On the strength of this test, the physician felt himself warranted in
recommending, or at least not advising against, Mr. Jacobs’ marriage,
which has probably now taken place.

Although there is some doubt what ethical relation a state institution
shall maintain with physicians in private practice, we took occasion to
call the attention of our patient’s new counsellor to the fact of the
patient’s neurosyphilis. We noted that the man’s serum had been
constantly positive (Massachusetts State Board of Health findings) in a
score or more of observations. We called attention to the fact that
lumbar puncture had shown positive signs of neurosyphilis, including a
positive W. R., globulin, excess albumin, pleocytosis, and positive gold
sol. These facts, according to a letter received from the private
practitioner in question, have not altered his opinion in the slightest
to the effect that our patient is completely normal and entirely
suitable for marriage. It is clear that he regards the patient as not a
victim of General Paresis.

    5. What is the significance o the negative observation eventually
      obtained in Jacobs’ serum? One’s first thought is to impugn the
      accuracy of the laboratory work, but against this suspicion is the
      excellent reputation of the laboratory in question, and the
      agreement in the majority of its findings with those of the State
      Board of Health. It is more likely, as we assured the private
      practitioner at whose request the observation was made, that this
      negative test was an exceptional and isolated observation such as
      is not infrequent in long series of observations, particularly
      those made under therapeutic conditions. In so important a matter,
      we are inclined to feel that the physician in question should have
      resorted to two more observations at intervals before running
      counter to the position taken by the hospital.


                       ——many a hard assay
       Of dangers, and adversities, and pains.

                       Paradise Regained, Book IV, lines 478–479.




                      V. SOME RESULTS OF TREATMENT


Cases 99–103 show the Variety of Structural Lesions that Treatment has
to face.


  =SPASTIC HEMIPLEGIA in PARETIC NEUROSYPHILIS (“general paresis”),
  showing marked degenerative changes, a condition in which therapy
  could be theoretically of very little avail. Autopsy.=


=Case 99.= James McDevitt arrived at the Danvers Hospital, July 20, 1906
(saying that he came to be “thawed out”), and died less than six months
later: January 12, 1907. He was 34 years of age. He had been a
shoe-worker after leaving school, had worked eight years with the
General Electric Co., and had then become a bartender. He had, however,
stopped work in September, 1905, and we may safely say that mental
symptoms had begun insidiously at about that time. His symptoms, if
there were any, had been masked by a heavy alcoholism, but an obvious
change had appeared in November, 1905. The patient lost ambition, smoked
and loafed about his room, and developed speech disorder. He denied
venereal disease, nor was there any superficial evidence of such.

=Physically=, the patient showed little or no disorder except acne of
the trunk, patches of eczema on the left lower chest, and numerous
brownish scars along both tibiae.

=Neurologically=, the Romberg position was maintained, but the gait was
very unsteady on attempts to walk a straight line; fingers, tongue, and
face were tremulous, and finer movements were performed with marked
incoördination. No direct or consensual light reactions could be
obtained in the pupils, which were dilated and irregular.

The condition of the reflexes is important on account of the autopsy
findings. The abdominal and cremasteric reflexes were prompt, and the
knee-jerks equal and very lively. Achilles and normal plantar reactions
were present; there was no clonus; the arm reflexes were very brisk.


                    =COMMON THERAPEUTIC CONCEPTION=

    [M]VP =  TYPICAL PARESIS

    MV[P] =  TYPICAL CEREBROSPINAL SYPHILIS

    [M]V[P] = TYPICAL SYPHILITIC ARTERIOSCLEROSIS

                        (M = Membranes,   V = Vessels,
                         P = Parenchyma, [] = not involved)

                                                              CHART 21


The =mental symptoms= need not detain us. Consciousness was clear;
orientation for time, place, and to some extent for persons, was
imperfect. Arithmetic had been largely forgotten. Handwriting was
irregular and scrawling, and in places unintelligible. Although the
patient claimed that his memory was intact, it was decidedly imperfect.
He remarked that John D. Rockefeller, a Chicago king, was President; the
General Electric Works had almost 50,000 people at work; and in fact
Lynn was one of the largest cities in the state, having over 12,000
people. The height of patient’s room was estimated at 25 feet. There was
a slight euphoria. There was never any doubt of the =diagnosis= of
PARETIC NEUROSYPHILIS (“general paresis”).

Five months after admission, slight convulsions developed, after which
the patient was more dull and demented; he became bedridden. More
convulsions followed, leaving the right arm and hand useless. There were
clonic spasms of the muscles of both lower legs. Decubitus developed and
death occurred.

We may set the total duration of symptoms in the case of James McDevitt
at a little over a year; nor is there any evidence of previous or
prodromal symptoms beyond a total period of about 15 months, unless we
may regard his leaving the General Electric Works to become a bartender
some nine years before death, as a symptomatic change of character. In
any event, it is of note that the =autopsy= showed singularly few
lesions. Death was due doubtless to complications following decubitus,
and there was a slight acute splenitis. The kidneys showed some
parenchymal change. The aorta showed many patches of sclerosis, with
calcification or ulceration throughout its length. These changes were
not characteristic of syphilitic disease. There was considerable
coronary arteriosclerosis and a slight mitral valvular sclerosis. There
was a brown atrophy of the heart muscle, somewhat surprising in a man of
34 years. The =brain= was practically normal, weighed 1200 grams, and
showed convolutions normal in size, relation, and arrangement. There was
no sclerosis grossly evident in the blood vessels. The pia mater
appeared to contain a considerable excess of clear fluid. The calvarium
was of normal thickness and showed diploë and the dura mater failed to
show adhesions. There were no macroscopic signs of lesion in the spinal
cord.

=Microscopically=, the lymphocytosis, plasmocytosis, and phagocytosis of
the perivascular spaces, (relative?) increase in blood vessels, the
gliosis, and evidence of nerve cell destruction, taken together
warranted the diagnosis of PARETIC NEUROSYPHILIS. It was plain that the
nerve cell destruction was best marked in the _inner layers of the
cortex_. The microscopic study of the spinal cord showed that there was
very possibly a slight sclerosis of the posterior columns in the lumbar
region, but this was so slight that it could hardly be noted in the
myelin sheath stains (Weigert). Very sharply marked, on the other hand,
were the _bilateral pyramidal tract lesions_ in the lumbar and thoracic
regions, less marked at the cervical levels.

Without attempting to analyze carefully all these findings, it is
interesting to note in this case a foil to the usual spinal cord picture
of paretic neurosyphilis. The spinal cord, ordinarily normal, or perhaps
more usually affected by a degree of posterior column sclerosis, in this
case showed such well marked pyramidal tract sclerosis that we may
perhaps place the case in a subordinate group of SPASTIC PARETIC cases
of NEUROSYPHILIS. The source of the pyramidal tract disease lodges,
however, in the cortex cerebri itself, being part and parcel of the
lesions mentioned above as affecting more directly the inner layers of
the cortex. Many of the so-called giant, or Betz, cells had undergone a
complete destruction. It will be remembered that clonic spasms of the
muscles of the legs appeared in the fortnight preceding death, and that
there had been convulsions for about six weeks before death. There was
no evidence at the autopsy why the right arm and hand should have become
useless, whereas the left upper extremity remained normal. This case,
then, forms an exception to the ordinary paretic neurosyphilis group in
that the brunt of the microscopic process was borne by the inner layers
of the cortex. The cells of origin of the pyramidal tract fibres had
been cut in this lesion, and had become subject to partial or complete
destruction. Note, however, that the lesion remained a microscopic one
and that the marked convulsions were not related to gross lesions,
thereby following the rule for paretic seizures.

[Illustration:

  Bilateral pyramidal tract sclerosis, secondary to destruction of large
    motor (Betz) cells of motor (precentral) cerebral cortex—paretic
    neurosyphilis.
]

From the standpoint of possible treatment, it is of course true that few
organs of the body showed grave lesions save in the calcified and
ulcerated aorta, which conceivably might have become quiescent under
appropriate treatment. But, although the brain was almost if not quite
normal in the gross, and although its membranes showed practically no
lesion, treatment would not have been very promising. To be sure, the
exudate might have been cleared away if the spirochetes responsible
therefor had been destroyed by treatment. Yet the destruction of the
giant cells of origin of the pyramidal tract fibres to such an extent as
in this case could hardly have been compensated for by any known
process. So far as we are aware, the destruction of considerable numbers
of the smaller association elements of the brain is subject to the
compensation of other elements of the nervous system, which conceivably
might be re-educated or newly educated to perform certain processes. The
histological picture in a case like that of McDevitt accordingly leads
to the hypothesis that so well marked a spastic paresis, even in the
presence of otherwise favorable signs, would be of especially baneful
portent therapeutically.


  =NEUROSYPHILIS with total duration of symptoms twenty-two days. The
  comparatively MILD BRAIN LESIONS, INFLAMMATORY AND NOT DEGENERATIVE
  in type, suggest the possibility that therapy might have been
  successful. Autopsy.=


=Case 100.= Jacob Methuen, 35, was a carpenter from Newfoundland. He was
working upon a certain Thursday with his brother, who noticed that Jacob
was lifting the tools about in an unusual manner and talking strangely
to his fellow workmen. He fell asleep, going home in the street car, and
said afterward that he felt dazed and peculiar. He talked all kinds of
nonsense to his wife upon arrival. Methuen remained in bed next day,
fancying he was going to die, calling his family together, and saying
good-bye to them. He remained in bed all through the next day, but on
Sunday appeared better,—more active, and in fact quite natural. He slept
only an hour Sunday night, calling to his wife that it was time to get
up. On Monday he began to be irritable to his wife, and accused her of
flirting with his brother and intending to elope with him. He struck his
wife several times, and when two brothers came to watch him, accused
them both of trying to steal his wife, and struck them. Tuesday he
remained in bed until late at night, when he arose and tried to assault
the family.

It seems that another brother of the patient had died but eleven days
before his admission to the hospital and five days before the onset of
Jacob’s symptoms. Since his brother’s death he had been dwelling upon
religious matters, and in fact the day after his brother’s death, he
waked up during the night, saying that he was too happy to sleep, that
he heard the Master’s voice, and at times the devil’s voice; that there
was to be a modern miracle and his spiritual life from now on would be
different.

Eleven days after admission to the hospital, Methuen died, making a
total duration of symptoms, beginning at his brother’s death, of 22
days.


                       =NEUROSYPHILITIC LESIONS=

                   =LESIONS OF THE SECONDARY PERIOD=

               (1) INTERSTITIAL ENCEPHALITIS OR MYELITIS
                     (“meningitis”)
               (2) PARENCHYMATOUS ENCEPHALITIS OR MYELITIS
                     (“encephalitis,” “myelitis”)

                    =LESIONS OF THE TERTIARY PERIOD=

            (1) CHRONIC INTERSTITIAL ENCEPHALITIS OR MYELITIS
                  (“gummatous meningitis”)
            (2) CHRONIC PARENCHYMATOUS ENCEPHALITIS
                  (“dementia paralytica”)
            (3) CHRONIC PARENCHYMATOUS MYELITIS
                  (“tabes dorsalis”)

    “We have shown that the central nervous system is affected by
    syphilis at the same periods and in the same manner as are other
    internal organs. In addition the ‘parasyphilitic’ lesions are also
    of a typically syphilitic nature, being directly comparable to the
    parenchymatous affections found elsewhere in the body. They are
    ‘tertiary’ lesions differing only from the so-called ‘gummatous’
    processes in the central nervous system in that their localization
    is in the parenchyma while that of the latter is in the
    interstitial tissues.”

                                             McIntosh and Fildes, 1914

                                             CHART 22


=Physical examination= showed a man 5′ 9″ tall, weighing 149 pounds,
rather pale and poorly nourished, with a somewhat enlarged heart and no
evidence of venereal disease.

=Neurologically= there was a slight facial and digital tremor, but
otherwise no symptom or reflex disorder except that the tendon reflexes
were generally increased; the knee-jerks especially were very vigorous.
There was no speech defect. His handwriting was fairly legible.

The patient was very noisy and uncontrollable, tearing clothing and
biting, striking the attendants, refusing food, talking rapidly, loudly,
and incoherently. His manner suggested auditory hallucinations but no
positive evidence of these was obtained. His clothes could not be kept
on him. The following is a sample of his reactions: As the examiner
entered, the patient stood stark naked and glaring. He started to talk
as follows: “Methuen,—I, Saviour, come to life and ought to die—— Now I
lay me—— Now I die—— The heart beats—— No, I ain’t going to die—— I am
going out soon. I want my clothes—— You can’t hold me; I am strong.”
(Struggles violently with the attendants.) “I am God. God. I know you,
you can’t fool me.——I am here——I can do you all. How many doctors are
there here?” (Struggles violently. Looks at examiner.) “He is writing
something. Sir, you can’t fool me in a million years. Do you understand
that, doctor? You can’t fool me. Write all the prescriptions you want
to. Ten thousand years; you hear that, doctor? Ten thousand years. You
can’t fool me; ten thousand years. Ten thousand years are but a day for
the spirit of the Lord,” etc., etc.

The excitement continued unabated. The patient became entirely
disoriented, and finally almost unable to move. He lay in bed trying to
talk and muttering broken gibberish, still attempting to struggle to the
extent of his limited strength.

The =autopsy= showed no sign of lesion (brain weight 1380 grams),
unless, perhaps, the occipital regions were slightly firmer than the
rest of the brain. Death was apparently due to a bilateral pneumonia,
bronchial type. There was an acute splenitis. The only chronic lesions
of the body were a bilateral chronic adhesive pleuritis and a slight
sclerosis of the arch of the aorta.

[Illustration:

  Paretic neurosyphilis (“general paresis”) macroscopically normal,
    microscopically characteristic. Treatment does not have to face
    massive destructive processes already complete.
]

=Microscopically= there was a distinct though mild degree of
lymphocytosis of the perivascular spaces in many regions. Somewhat
extended _search failed to reveal plasma cells_, and it is certain that
if plasma cells existed, they must have occurred in very small numbers.

Here, then, was a case of DIFFUSE NEUROSYPHILIS (with brain picture
consistent) with symptoms lasting but 22 days and with an appearance of
acute mania. It is to be noted that this case arrived at the hospital on
the eleventh day of his symptoms. The case occurred long before the
development of the temporary care system in Massachusetts. It is
probable, or at any rate possible, that he would have been brought to
the hospital far earlier, say, upon the sixth day, had the modern
temporary care system been installed at that time. The routine W. R.
examination would then have been made. With more effective hydrotherapy,
it is possible that the patient’s life might have been prolonged and
that treatment might have been effective. So far as we can see, the case
would have been a singularly good one for treatment despite the
practical unmanageability of the case under ordinary home treatment, and
even under hospital conditions where modern hydrotherapeutic appliances
are not available.


  =PARETIC NEUROSYPHILIS showing very MARKED MENINGITIS, suggesting
  that therapy might have produced improvement. Autopsy.=


=Case 101.= We report the case of John Baxter, a boat tender of 48
years, because this particular victim of PARETIC NEUROSYPHILIS seems to
have had the most markedly thickened and altered meninges in our whole
series. Of course, the therapeutic theory upon which we now proceed in
the treatment of non-paretic and possibly even of paretic neurosyphilis
is that, other things being equal, the meningitis can be removed by
treatment, or in the course of treatment, so that the degree of ultimate
recovery rather depends upon the condition of the brain substance itself
than upon the condition of the meninges. Here, at all events, is an
example of the most highly meningitic neurosyphilis that we have seen.

Curiously enough, two of Baxter’s brothers were also patients at the
hospital at which Baxter died, and a number of the other members of the
family are reported as “nervous.” It seems that at 35 Baxter began to
drink heavily and had never given over the habit of alcoholism.

Upon admission to the hospital, in fact, he showed a sufficiently
typical picture of delirium tremens. His consciousness was clouded, he
had vivid visual hallucinations and was very apprehensive.

His heart was enlarged to the left; the pulse, 120, was of increased
tension and irregular; there was peripheral arteriosclerosis; the teeth
were poor; the tongue coated; and the mouth foul. The urine showed a
trace of albumin and rare hyalin casts.

=Neurologically=, the gait was somewhat unsteady, there was an extreme
tremor of the whole body, including the tongue and fingers. The Romberg
sign was negative although there was marked swaying. The pupils were
equal and reacted normally; the knee-jerks were markedly exaggerated,
the arm reflexes somewhat exaggerated. The remainder of the reflexes
upon systematic examination were negative.

[Illustration:

  A high degree of chronic leptomeningitis. Pia mater thick, opaque,
    concealing brain. In paretic neurosyphilis (“general paresis”).
]

Upon arrival, Baxter was put to bed, but he barricaded his door and
fought with the attendants. The tremor increased, the hallucinations
were both visual and auditory. After a few days, Baxter became so weak
that he could not move. He refused to eat for a period of two days,
explaining in whispers that he did not wish to be poisoned; a voice had
told him the food was to be poisoned. The voice was of agreeable tones,
probably belonging to a lady; it did not speak, but sang to him. The
clouding of consciousness failed to clear up, as in delirium tremens, so
that, though patient was admitted March 3d, it was hardly possible to
speak freely with him until more than a month later, April 9th. A
good-natured conversation would run as follows:

 “What is your name?” “Baxter.”
 “First name?”        After long pause, “Don’t know.”
 “John?”              Pause of 7 seconds, “Yes, I think it is.”
 “How old are you?”   “There are legs——there is a body——up to here——”
 “Say the alphabet.”  Term not understood.
 “Say the _a_, _b_,   “Oh yes; a, b, c, d (long pause), e, f; I cannot
   _c_.”                say it, I did not have much education; I am not
                        intelligent.” (In point of fact, the patient had
                        a good grammar-school education, and had long
                        worked as a clerk in a grocery store, with good
                        wages.)
 There was some
   speech defect.

Soon the hallucinatory phase passed, and the patient remained in a
cloudy and disoriented state, inaccessible, rarely speaking, and
gradually failing physically. Death occurred about three months after
admission (pulmonary symptoms).

In estimating the duration of the process in John Baxter, we must take
into account that he left the grocery business and became a hard-working
but poorly-paid boat tender at about 35 years, at the same time that the
alcoholic habit began.

The =autopsy= showed that death was due to bronchopneumonia with
pleurisy. There were in the body a variety of chronic lesions, such as
gastritis, colitis, epididymitis, splenitis, parietal and valvular
endocarditis, prostatitis, chronic appendicitis, and some mesenteric
lymphnoditis. The heart was somewhat hypertrophied. There was a slight
diffuse nephritis with cysts, emaciation, and decubitus. The calvarium
was thick and somewhat dense. The dura was thickened and adherent, and
the pia mater,—as above stated, the most thickened and altered pia mater
in our series,—is described as everywhere thickened, of a brownish gray
and white color, especially over the vascular lines, and as showing
small white areas of deeper thickening scattered over the surface, but
most markedly over the sulci, and not as a rule over the crowns of the
gyri. There were also yellowish brown spots with a suggestion of fibrin
over the lateral aspects of both hemispheres. The vessels at the base
were not remarkable in the gross. The brain weighed 1220 grams, and
appeared to be of darker color than usual.


  =Some cases of PARETIC NEUROSYPHILIS (“general paresis”) have so
  much BRAIN ATROPHY that it is not possible to expect much
  improvement through antisyphilitic therapy.=


=Case 102.= Theodosia Jewett, dead at 58 years, showed the most
remarkably wasted brain in a long series of victims of paretic
neurosyphilis. We present her case to emphasize what therapy must face
in certain instances, but would recall the fact that exceedingly few
such wasted brains have come to our attention in cases dying in the
institutions of Massachusetts.

Mrs. Jewett, a housewife, whose parents died of shock, and one of whose
two brothers also died of shock, was a normal child and schoolgirl, and
worked as dressmaker until she was married, at 24, to a grocer, by whom
she had two children. At the age of 46, Mrs. Jewett began to suffer from
so-called “nervous prostration.” The attack lasted some two years, but
there were no psychotic symptoms beyond worry and insomnia. The
menopause occurred at 52, at which time the first signs of psychosis
appeared, namely, a forgetfulness concerning familiar matters, such as
sewing, cooking, and the like. At 55, this amnesia had become so marked
that Mrs. Jewett could neither write nor tell time. She, however, was a
perfectly quiet and easily manageable patient, often subject to
drowsiness in the day.

Six months before her admission to the hospital, she began to suffer
from insomnia, failed to recognize her surroundings, and had a number of
crying spells. Restlessness had begun a month before admission; auditory
hallucinations developed in the form of imaginary conversations with
dead persons. A certain loquacity set in, and for a week before
admission, Mrs. Jewett became somewhat resistive.

=Physically=, the patient was sallow, poorly nourished, with pale mucous
membranes, peripheral arteriosclerosis, no teeth, muscular feebleness,
tremor of hands and tongue, and active knee-jerks. =Mentally=, the
patient was depressed, talked to herself, assumed a supplicating
position, suddenly altered her attitude, and was very tremulous. Her
talk was low, mumbling, and incoherent, for the most part composed of
answers to her own questions. Sometimes there was a curious difficulty
in speaking, such that the lips moved but no sound emerged; but for the
most part there was no difficulty in uttering words. The patient either
could or would not write. Only when the attention was secured by
speaking to her sharply was she apparently able to understand questions,
and the answers to these sharp questions came spasmodically and as if
interrupting her own thoughts. Nor was it ever possible to obtain a
repetition of the same answer.

The patient died in exhaustion, with pulmonary symptoms three weeks
after admission.

The =autopsy= which was performed 3½ hours after death showed the
following points of interest:

The heart weighed 210 grams. There was marked thickening of the aortic
valve. The coronaries were slightly thickened.

The lungs were slightly adherent to the chest wall at the apices and
posteriorly. The right lung was consolidated in the lower two lobes
posteriorly and the bronchi exuded pus; the left lung was not
remarkable. There was a chronic splenitis.

The liver showed fibrous changes, was a brownish-red in color, mottled
with yellow.

Combined weight of the kidneys 195 grams. The capsules were adherent,
tearing the cortex when stripped.

The diploë were well marked. The dura was not adherent. The pia was
slightly thickened and raised from the cortex by a large amount of
subpial fluid (showing atrophy of the cortex). The pial vessels were
injected, more markedly so on the left side. The arachnoid villi were
reported as moderately developed, especially along the longitudinal
fissure.

The brain was rather soft in all regions. The weight was 1045 grams.
According to Tigges’ formula the weight of the brain should be
approximately 8 times the body length in centimeters. The length in this
case was 158 cm., therefore, according to this formula the weight of the
brain should have been 1464 grams. The difference of more than 400 grams
is evidently a loss to be accounted for by atrophy, a very heavy loss.

[Illustration:

  Perivascular exudate (low power) in atrophic cortex from case of
    general paresis.
]

[Illustration:

  Markedly atrophic cortex, but without local perivascular exudate.
]

    1. Was the “nervous prostration” at 46 of syphilitic origin? One
      cannot give a categorical answer to this question. The high
      incidence of shock in the family suggests poor stock in which a
      psychoneurosis is not an unusual phenomenon. The presence of
      syphilis might act as a debilitating factor or _agent
      provocateur_, if it were not to cause any demonstrable brain
      lesion. As pointed out in the case of Harrison (9), however, it is
      not unusual in neurosyphilis to find a history of symptoms
      occurring years before the final breakdown and symptoms frequently
      not recognized as of neurosyphilitic nature.

    2. Does the fairly long duration of the psychosis (at least 3 years)
      explain the marked atrophy? Cases having symptoms even much longer
      than three years at times show relatively very little atrophy, so
      that this factor in itself cannot be said to explain the
      tremendous destruction of tissue.


  =The THERAPY OF NEUROSYPHILIS has to face not merely variations in
  the degree of brain wasting and in the degree of meningitis, but
  also variations in the topographical distribution of lesions.
  Autopsy.=


=Case 103.= To bring out this point we may instance the case of Alfred
Weed, a victim of PARETIC NEUROSYPHILIS, dying at the age of 48 years
after a course of about seven years. The following is an abstract of the
clinical history:

A. W. suffered from lues some 24 years before his death at Danvers
Insane Hospital in 1907. There is no account of insanity in his family.
The patient had been undergoing mental changes for six years before
death. At the age of 42 began to take interest in socialism and
spiritualism. Would become excited at times and was observed to talk to
himself. At times it seemed that he was reacting to visual
hallucinations. After eight months he became depressed and apprehensive
and developed delusions of poisoning.

On admission to the Danvers Insane Hospital in June, 1902, the subject
was found to be ataxic, falling in the Romberg position. Pupils were
equal but of pin-point size. There was tremor of the facial muscles. The
knee-jerks were absent. Speech was ataxic. Memory defective. Depressed.
Thought he was to be punished. Refused to eat.

Later in the year of admission, patient became more negativistic. He
refused to have his clothes brushed. His answers were slow. Mental
arithmetic was correctly but slowly done. During January, 1903, the
patient was apt to be active and talkative for a time, and then his
attitude would suddenly change to one of silence, resistivement and
untidiness. From time to time he would be querulous and sulky. In
August, 1903, the patient became weaker and could walk with assistance
only. Paralysis developed in the left facialis region and in the left
external rectus. Pupils were still small, but the left had become
smaller than the right. Light reaction tests unsatisfactory. Knee-jerks
could not be obtained.

In December, 1903, the patient was untidy and helpless, lying with his
thighs and legs flexed. The limbs were spastic on passive motion. In
1905, the pain sense of the legs was found lost and the pupils were
small and stiff. The protruded tongue was deflected to the right. The
right labial fold was more prominent than the left. Knee-jerks remained
absent. Ataxia was extreme.

The =Neurological Findings= may be summed up as follows:

   1. Ataxia of the legs.

   2. (Probable) Diminished sensibility in the legs.

   3. Pupils small and stiff. Left smaller than the right.

   4. Paralysis of left facialis.

   5. Paralysis of left external rectus.

   6. Tongue protruded to right.

   7. Right elbow jerk greater than left.

   8. Knee-jerks absent.

The cause of death was bronchopneumonia. The walls and valves of the
heart showed a few chronic changes. There was a marked splenitis and an
atrophy of the liver. The kidneys showed numerous depressed scars. The
arch of the aorta was somewhat sclerotic. The following is a full
description of the head findings which we present by way of comparison
with other cases. Note especially the cerebellar, dentate, and olivary
changes. Note also the fact that palpable sclerosis is demonstrable over
a far larger area than atrophy, so that we may almost safely conclude
that the process of induration sometimes precedes that of atrophy. One
gets the impression from the extent of visible atrophy and tangible
induration in this case, that a possible therapy would have not merely
to clear the perivascular spaces of cells and spirochetes, but would
also need to arrest the indurating and wasting process. Nor could any
therapy deal effectively with the superior frontal and upper central
atrophy of the cerebrum of this case, or with the olivary and cerebellar
lesions.

=Head=: Hair thin at vertex. Scalp normal. Calvarium thin and dense.
Dura mater slightly adherent to calvarium at vertex. Sinuses normal.
Arachnoidal villi well developed. Pia mater of anterior and central
regions contains an excess of fluid. The pial veins well injected.

The =pia mater= exhibits one unusual lesion: Faintly yellowish brown
spots of miliary and slightly larger size are scattered irregularly in
clusters over the vertex. These miliary pial macules are observed
especially over the posterior third of the left superior frontal gyrus
(a group of twelve or more). Two are seen in the pia mater of the right
superior frontal gyrus. One is seen in the upper part of the left post
central gyrus. The upper end of the right postcentral gyrus contains
three macules.

Besides these brownish macules, the pia mater also shows focal white
thickenings which resemble the more frequent appearances of chronic
fibrous leptomeningitis. The white thickenings are of irregular size but
are, as a rule, larger than the macules above mentioned. They occur, as
a rule, over the sulcal veins and are most frequent in the anterior
region.

The vessels at the base are normal. There is no evidence of pial
thickening at the base of the brain. =Brain= weight, 1265 grams. There
is visible atrophy of both superior frontal gyri and of the upper
two-thirds of both central gyri. The extent of palpable sclerosis
surpasses that of visible atrophy. Palpable increase of consistence is
shown by the prefrontal, orbital (more marked on left side), frontal,
central, hippocampal and occipital regions. The temporal cortex is of
normal or slightly reduced consistence.

Section of the cerebral cortex shows everywhere preservation of the
cortical markings. The sclerosed areas show a diminution in depth of the
cortex, which is more marked in the left prefrontal region. The white
matter of the centrum semiovale of the prefrontal and occipital regions
on both sides shows an increase of consistence. The cerebellar cortex
also shows variations in consistence. The clivus and lobus cacuminis and
the posterior half of the inferior surfaces of both cerebellar
hemispheres are firmer than normal. The laminæ of the left clivus are a
trifle narrower than those of the right. There is visible extensive
atrophy of the laminæ on both sides of a fissure in the middle of the
left lobus cacuminis. In the coördinate portion of the right cacumen
there is a similar process which is less marked. The dentate nuclei are
firm. The olives show an increase of consistence, equal on both sides.
The left olive shows on section a crowding together of its folds in the
middle part of the upper limb.

Spinal cord was not remarkable.

=Summary=:

 Adhesive pachymeningitis
 Chronic fibrous leptomeningitis
 Miliary pial macules
 Cerebral atrophy
 Cerebral sclerosis
 Cerebellar atrophy and sclerosis
 Bronchopneumonia
 Chronic splenitis
 Nephritis
 Aortitis


  =It is generally recognized that DIFFUSE NEUROSYPHILIS
  (“cerebrospinal syphilis”) frequently is cured through
  antisyphilitic therapy. Example. Mental improvement, in one month;
  recovery from paralysis, ten months.=


=Case 104.= John Edwards, a man of 28 years, well developed and
nourished, with general enlargement of glands and skin lesions, came to
the hospital in a stuporous condition, with evidences of a complete
hemiplegia.

According to the wife, Edwards had had a chancre of the lip about a year
before, for which he had been treated with an intravenous injection,
presumably of salvarsan, and also presumably with mercury. The lip
lesion had then disappeared. For a month before admission, Edwards had
had headache and dizziness, for which he was given pills and drugs.
There had also been difficulty with speech and numbness of the left arm
as far up as the elbow, but this paresthesia had quickly disappeared.
The hemiplegia was of only a few days’ duration. After a feeling of
nausea and vomiting, the patient had fallen with left-sided paralysis.
Afterwards, he had shown mental peculiarities, eventually becoming
noisy, hard to manage, and appropriate for hospital care.

The =physical examination= showed a variety of increased reflexes,
including ankle clonus on the left side.

The question might arise whether this case was one of hemorrhage or
thrombosis, and the facts about the onset of the hemiplegia are
inadequate for a decision. However, at so early an age, the probability
of syphilis is large and the history of labial chancre was quite
suggestive. If we may conclude neurosyphilis, the diagnosis of
thrombosis rather than rupture of blood vessel is likely. The laboratory
tests bore out the diagnosis since the W. R. of serum and fluid both
proved positive; the gold sol reaction was syphilitic; there were 176
cells per cmm.; there was excess albumin, and a positive globulin
reaction.


                      =NON-PARETIC NEUROSYPHILIS=

                        =DIFFUSE NEUROSYPHILIS,
                   MENINGOVASCULAR PARENCHYMATOUS,
                        CEREBROSPINAL SYPHILIS=

                 CASES SYSTEMATICALLY TREATED       13
                 CLINICAL RECOVERY, C.S.F. NEGATIVE 11
                 UNIMPROVED                          1
                 UNIMPROVED, BUT C.S.F. NEGATIVE     1

                    MASSACHUSETTS COMMISSION ON MENTAL
                                             DISEASES,
                                      _November, 1916_

                                              CHART 23


The outcome in such a case is dubious. If death does not occur soon,
recovery is not impossible under treatment. At all events, a
considerable improvement is likely.

Edwards was given bi-weekly injections of salvarsan, intramuscular
injections of mercury salicylate, and doses of potassium iodid,
averaging 100 grains, three times a day. Under this treatment, he slowly
recovered and became mentally clear after a few weeks. The paralysis
seemed complete and permanent. Even after three or four months, there
was absolutely no change in the condition, and Edwards was quite unable
to move either arm or leg. Meanwhile, the spinal fluid had become
practically negative to all tests.

_Treatment_ was somewhat optimistically continued and was _rewarded at
the end of ten months_ with marked improvement such that the patient was
able to stand on the paralyzed leg and move the arm to a certain degree.
This improvement is still continuing. The spinal fluid and the serum
have remained negative to laboratory tests.

Note: A period of six months is commonly regarded as that period in
which improvement in paralysis is to occur if there is to be any
improvement. There was certainly not the slightest improvement in the
paralysis of this case before eight or nine months of treatment had
elapsed, and it took ten months to secure the marked improvement
mentioned.

    1. What is the significance of the prodromal symptoms? The headache
      and dizziness should have been viewed with great gravity. They are
      characteristic in MENINGOVASCULAR NEUROSYPHILIS.

      Moreover in this case there had also been difficulties with speech
      and other transient symptoms which should have called attention
      far earlier to the possibility of neurosyphilis.

    2. What is the significance of the high cell count: 176 per cubic
      millimeter? Such high cell counts are frequent enough in diffuse
      neurosyphilis, but low cell counts are frequent also. But although
      the high cell count taken alone is of lesser significance, the
      fact that the high cell count in this case is associated with a
      “syphilitic” gold sol reaction is of far greater significance for
      diagnosis. These associated findings are characteristic of
      meningovascular neurosyphilis.

    3. What kind of recovery may be expected in successful examples of
      treatment in meningovascular cases? Recovery with defect. It will
      be noted that ten months elapsed before any marked improvement
      occurred on the paralyzed side. We could not expect a complete
      recovery from this paralysis.

    4. Was inadequacy of treatment following the chancre responsible for
      the early cerebrospinal involvement? In this connection one must
      remember that such neural involvements occur occasionally even
      during active treatment (neurorecidives). The discontinuance of
      treatment after a short period, in this case less than a year, is
      always a risk to say the least. And this is true even though the
      W. R. becomes negative, for trouble of a neurosyphilitic nature
      may occur later; this when both blood and spinal fluid have
      previously been found negative. The old rule of following and
      treating a syphilitic for several years despite the disappearance
      of symptoms is still a good rule.


  =The results of systematic, intensive, intravenous salvarsan therapy
  in atypical neurosyphilis (cases not certainly paretic, tabetic or
  the common types of meningovascular neurosyphilis) may be in our
  experience as good as the results of treatment in common
  meningovascular cases: example.=


=Case 105.= Henri Lepère, a machinist, 48 years of age, came voluntarily
to the Psychopathic Hospital for a gradually failing memory and
inability to work. He had had indigestion for four years (epigastric
distress, nausea, no vomiting). He was still suffering from epigastric
distress and from headaches. At times he had had difficulty in walking.

=Physically=, Lepère looked older than he was; he was very poorly
developed and nourished, and seemed very weak. There was a slight
visceroptosis.

=Neurologically=, there was considerable speech defect, particularly
well marked in test phrases. The pupils were contracted and gave the
Argyll-Robertson reaction. Neurologically there were no other signs.

=Mentally=, there was a depression with worry; but it was a question
whether these phenomena were not entirely natural. The special complaint
was of failing memory.

The Argyll-Robertson pupil also _prima facie_ signifies neurosyphilis.
Lepère, in fact, admitted syphilitic infection at 23. The gastric
symptoms at once suggested tabes. The knee-jerks and ankle-jerks were,
to be sure, preserved; however, this is not very unusual in tabes. The
amnesia and aphasia naturally suggested paresis. Without resort to
laboratory findings, accordingly, the diagnosis of taboparetic
neurosyphilis (“taboparesis”) was suggested.


   =EFFECT OF EARLY TREATMENT ON THE     DEVELOPMENT OF NEUROSYPHILIS=

 TOTAL CASES                                                    4134
 DEVELOPED GENERAL PARESIS                                   198 =  4.8%
 DEVELOPED TABES DORSALIS                                    113 =  2.7%
 DEVELOPED CEREBROSPINAL SYPHILIS                            132 =  3.2%
                                                             ———————————
                                                             443 = 10.5%

                          =EFFECT OF TREATMENT=
                                                              Repeated
                                        None      1 course    energetic
 NUMBER OF CASES                         100         134         924
 DEVELOPED G.P.                       25 = 25%   31 = 23.1%   30 = 3.2%
 DEVELOPED TABES                      11 = 11%   16 = 11.9%   25 = 2.7%
 DEVELOPED C.S.S.                      3 =  3%   21 = 15.6%   71 = 7.6%

                                       Poorly                  Better
                                       treated                 treated
                                       1880–84                 1895–99
 NUMBER OF CASES                         617                    1139
 DEVELOPED G.P.                       60 = 9.7%               37 = 3.2%
 DEVELOPED TABES                      22 = 3.5%               16 = 1.4%
 DEVELOPED C.S.S.                     15 = 2.4%               28 = 2.4%

                                                   MATTAUSCHEK AND PILCZ

                                                                CHART 24


The serum W. R. proved positive, but the spinal fluid W. R. very
slightly so (yielding only moderate reaction with 1 cc., 0.7 and 0.5
cc., and a negative reaction with 0.3 and 0.1 cc.). Globulin was
moderate, and albumin was found in only moderate excess. There were 21
cells per cmm. in the spinal fluid. The gold sol reaction was that which
we regard as typical of syphilis or tabes. If we were to rely upon the
weakness of the fluid W. R. and the nature of the gold sol reaction, we
should be inclined to favor the diagnosis of DIFFUSE NEUROSYPHILIS
(“cerebrospinal syphilis”) rather than resort to the diagnosis of
paretic neurosyphilis.

Salvarsan treatment was attended by the rapid disappearance of headaches
and gastric symptoms and by a rapid gain in weight and feeling of
well-being. Salvarsan was continued twice a week for two months,
whereupon Lepère returned to work. He has been successfully at work now
for seven months without return of symptoms. Four months after beginning
of treatment, the spinal fluid was examined and found entirely negative.
Nevertheless, the serum W. R. has remained positive despite eight months
of salvarsan treatment.

    1. What is the meaning of the titrations in the spinal fluid
      Wassermann reaction? When Plaut originally applied the Wassermann
      reaction to spinal fluids, he used 0.2 of a cc. of spinal fluid.
      With this amount of fluid he found that cases of general paresis
      gave a positive reaction in about 100% of the cases while this
      positive reaction was only given by 40 to 60% of the cases of
      cerebrospinal syphilis and tabes dorsalis, hence he promulgated a
      differential point that a negative reaction in spinal fluid
      indicated that the case was not general paresis. Hauptmann later
      showed that if 1 cc. of spinal fluid were used, a positive
      reaction would occur in practically 100% of the cases of general
      paresis, cerebrospinal syphilis and tabes. Therefore, at present,
      we use the different titers of spinal fluid from which we draw the
      following conclusions: If the reaction in the untreated case is
      negative with 0.1 and 0.3 of a cc. and positive with the 0.5, 0.7
      and 1 cc. dilutions as in the case of Lepère, we are probably
      dealing with non-paretic neurosyphilis. With this method of
      titration we are also better able to watch the progress of
      treatment as the dilutions of 0.1 and 0.3 cc. become negative
      first.

    2. How soon can one expect improvement after commencement of
      salvarsan therapy in cases of diffuse neurosyphilis? The time
      relation of results in treatment varies with each individual case.
      In the case of Lepère gastric symptoms that had been present for a
      number of months disappeared as if by magic after the first
      injection of salvarsan. As a rule, it is true that the more acute
      the symptoms the quicker their disappearance but this does not
      hold for all cases, as in this particular instance the
      long-standing symptoms disappeared very rapidly. The symptoms
      often disappear very much more rapidly than the laboratory, tests
      change.

    3. How can the mental symptoms (depression and failing memory) of
      which patient complained be explained? In the first place, as has
      been stated, it is doubtful if these are more than subjective and
      the result of the patient’s feeling of discomfort and pain.
      However, it is also possible that there may be intracranial
      involvement of the meninges or of the brain itself. And, if such
      were the case, the improvement might be the result of the
      treatment.


  =The Argyll-Robertson pupil should not be used as a basis for a
  necessarily bad prognosis if treatment can be given.=


=Case 106.= Frederick Stone was a business man of large interests. He
had been in the hands of physicians for several years for a variety of
disorders such as renal, respiratory, cardiovascular, and so on. No
suspicion of syphilis had apparently been uttered by the physicians
despite the fact that Mr. Stone readily stated that he had had a chancre
thirty years before, and that he had received several years’ treatment
of mercury and potassium iodid by mouth.

It appeared that a few years ago he had begun to have trouble with his
nose, which was cauterized and operatively interfered with without
satisfactory results. This nasal condition had later been diagnosticated
as gummatous, and had improved considerably under a mild antisyphilitic
treatment. However, this nasal condition had been considered and treated
quite separately from the remainder of Mr. Stone’s troubles.

What brought him to attention was a sudden diplopia with ptosis. There
was a paralysis of the external rectus of the left eye, as well as a
drooping of the lid on this side. The left eye was much inflamed. The
diplopia greatly bothered the patient, and there was also considerable
pain in the left frontal region, confined chiefly to the distribution of
the first division of the trigeminal nerve. According to the patient
this headache was periodic. There was considerable tenderness to
pinprick over the area and a diminution of sensory discrimination of
fine touch. Both the pupils failed to react to light.

The remainder of the neurological symptomatic examination was
surprisingly clear of disorder, nor was there anything in the history
suggestive of tabes. There was ozena as well as evidence of the
operative work upon nares and throat. Possibly the arteries were
slightly hardened; blood pressure was 165 systolic. There was a large
trace of albumin, and there were numerous hyalin casts in the urine.


                         =PARETIC NEUROSYPHILIS=

                           =(GENERAL PARESIS)=

 Cases systematically treated                                 50

 CLINICAL REMISSIONS                                          34     68%
   C.S.F. ALTERED TO NEGATIVE                              4      8%
   C.S.F. ALTERED TO WEAKER                               16     32%
   C.S.F. UNALTERED                                       14     28%

 CLINICALLY UNIMPROVED                                        16     32%
   C.S.F. WEAKER                                           7     14%
   C.S.F. UNALTERED                                        9     18%

                             MASSACHUSETTS COMMISSION ON MENTAL DISEASES
                                                          NOVEMBER, 1916

                                                                CHART 25


=Mentally=, there was a degree of depression and worry hardly out of
keeping with the general situation. Despite the preservation of memory,
Mr. Stone failed to do rather simple arithmetical calculations; this was
the more remarkable as in his business he had to handle figures a great
deal and had been doing so until recently. There was a slight tremor in
his writing, as well as a certain difficulty in enunciating test
phrases. Insomnia, irritability, and a feeling of nervousness and of
being tired out, completed the picture.

A suggestion for diagnosis would be classically offered by the
Argyll-Robertson pupils. Should not a patient with the Argyll-Robertson
pupils have either tabes or paresis? However, in favor of tabes, besides
the pupil, are to be counted merely the troubles with the eyes. In the
direction of paresis we have to consider speech defect, to say nothing
of less definite symptoms such as insomnia and increased irritability.

We are inclined to think, however, that the disease in this case is
meningovascular. This diagnosis is suggested by the cranial nerve
palsies and by the headache. Headache is much more rarely a phenomenon
in the paretic type of neurosyphilis than in the meningovascular type.

In point of fact, the spinal fluid phenomena bore out the diagnosis of
MENINGOVASCULAR NEUROSYPHILIS inasmuch as the globulin, albumin,
cellular content, gold sol, and W. R.’s were all weakly positive.

    1. How far can we regard the cardiorenal defects as syphilitic?
      Perhaps we may do so on the general principle of parsimony in
      scientific interpretation.

The diagnostic lumbar puncture led to an extremely severe exacerbation
of the pains on the left side of the head. In fact, these pains could
not be held in check by the exhibition of pyramidon. Mr. Stone regarded
the pain as due to the lumbar puncture. However, there was no
improvement in the pain in the prone position,—a feature characteristic
of lumbar puncture pains. Upon administration of salvarsan, this local
pain rapidly disappeared. In fact, there was a startling improvement;
the ocular palsies disappeared in a few weeks, although these palsies
had been present for several months before the administration of
salvarsan. The blood pressure was reduced; the urine became negative.
Perhaps the most startling feature of all (although of this we are not
sure) was that the patient states he was accepted by a life insurance
company although he had been twice refused previously.

=Note= in this case the 30–year interval between infection and
generalized neurosyphilitic involvement. Note also the amenability of
the process despite this duration. We are perhaps entitled also to note
that a neurological examination careful enough to detect an
Argyll-Robertson pupil should have been made by a number of examiners
long before the particular crisis which we have sketched. It is also
permissible to note that the rhinological work should not have been
carried out independently of all other medical work.

    2. What are the untoward results of lumbar puncture? It is true that
      there is always a possibility of setting up a septic meningitis by
      lumbar puncture, but this is a very remote possibility and with
      any reasonable care it is not to be considered. Lumbar puncture
      also has a considerable danger in cases of increased intracranial
      pressure. In cases of brain tumor where the tumor is located in
      the posterior fossa, sudden death may occur from withdrawal of
      spinal fluid. This is supposed to be due to the medulla being
      pressed down into the foramen magnum and causing paralysis of
      respiration. Therefore lumbar puncture should never be performed
      except with the greatest caution in a case in which brain tumor is
      suspected.

      However, aside from these remote serious consequences which play
      very little rôle in the ordinary procedure of lumbar puncture,
      certain unpleasant symptoms do frequently arise. These symptoms
      are chiefly headache and nausea, but, however, may go as far as
      vomiting. These symptoms occur almost entirely in the cases in
      which there is no abnormal condition producing increased spinal
      fluid pressure. Such unpleasant symptoms may last as long as four
      or five days; as a rule, however, last only for a period of a day
      or two.

    3. What is the treatment of discomfort following lumbar puncture? It
      is a rule well worth observing that the patient after lumbar
      puncture should remain flat on his back without a pillow for 24
      hours in order to avoid any unpleasant symptoms. If any symptoms
      do occur, it will be almost certainly when the patient arises, and
      in nearly every instance they will be overcome if the patient
      again assumes the prone position. Raising the foot of the bed so
      as to lower the head also helps. Veronal or bromides may be given
      but as a rule are not very satisfactory.

    4. How permanent is the improvement obtained in the case of Mr.
      Stone likely to be? As a matter of fact, the patient discontinued
      treatment as soon as he felt well again, but after two months the
      pain returned to be again quickly dispelled by salvarsan. This
      improvement must be considered as only temporary. Under continued
      treatment there may be no further relapse. There is, however,
      evidence that much damage has been done to the body by the
      spirochetes, much of which is irreparable. It is even possible
      that further disintegration might occur even while undergoing
      treatment. Still treatment offers much in such a case and is to be
      highly recommended.


  =In DIFFUSE NEUROSYPHILIS, rendering the spinal fluid negative by
  treatment may mean neither cure nor disappearance of symptoms.=


=Case 107.= Greta Meyer, a widow, 51 years of age, came voluntarily to
the hospital, seeking medical aid for a marked depression. She was also
suffering from a right hemiplegia. It appeared, according to Mrs. Meyer,
that she was married at 16, and lived with her husband until 29,
whereupon she left him on account of his alcoholism, his abuse of her,
and the discovery through his physician that he was suffering from
venereal disease. She had had two healthy children and there never had
been miscarriages or stillbirths. Six years after the separation, namely
at 35 years of age, and 16 years before resort to the Psychopathic
Hospital, Mrs. Meyer developed certain red areas on her hand, and
learned at a hospital that these were due to syphilis. She kept up
treatment for these lesions for a year, until she seemed perfectly well.

She had, in fact, remained perfectly well for some 14 years, until at
49, a small tumor had appeared on the right side of the forehead, near
the hair line. This tumor was firm and not sore. Medical treatment
reduced it, leaving, however, a depression in the bone. One day, about a
month after the appearance of the tumor, the patient lay down for a nap,
and upon awaking found she could only with difficulty move her right arm
and leg. Her face was not affected; she was not in pain; and there was
no disorder of speech. In a few days she got much better and she had
been improving for some time past through the administration of further
medicine.

However, since the onset of the hemiplegia Mrs. Meyer had been very
despondent. There had been ups and downs but she had rarely felt well.
The depression was a mild one and in point of fact may perhaps be
regarded as non-psychopathic, since at her age with her disability,
there might well be a degree of sadness and unhappiness concerning the
future. =Mentally=, there was no other disorder of note, and in
particular no disorder of memory.


                         =METHODS OF TREATMENT=

        I. BY MOUTH.
           1. MERCURY
           2. IODIDES
           3. ARSENIC

       II. INTRAMUSCULAR INJECTIONS
           1. MERCURY
           2. SALVARSAN, NEOSALVARSAN, OTHER ARSENIC PREPARATIONS
           3. SODIUM NUCLEINATE
           4. ANTIMONY

      III. INTRAVENOUS
           1. MERCURY
           2. MERCURIALIZED SERUM
           3. SALVARSAN, NEOSALVARSAN, ARSENIC
           4. IODIDES

       IV. SPINAL INTRADURAL
           1. SALVARSANIZED SERUM (IN VIVO—SWIFT-ELLIS)
           2. SALVARSANIZED SERUM (IN VITRO—MARINESCO-OGILVIE)
           3. MERCURIALIZED SERUM (BYRNES)

        V. CEREBRAL SUBDURAL AND INTRAVENTRICULAR
           1. SALVARSANIZED SERUM (IN VIVO)
           2. SALVARSANIZED SERUM (IN VITRO)
           3. MERCURIALIZED SERUM

                                                              CHART 26


=Physically=, the patient showed a right-sided hemiplegia with excessive
right knee-jerk, but without Babinski or other abnormal reflex
phenomena. The extraocular movements were somewhat restricted in range
but there was neither strabismus nor nystagmus.

The question arose whether the hemiplegia was of hemorrhagic or
thrombotic origin. After all, at 51 years, hemiplegia is rather unlikely
to be of a non-syphilitic arteriosclerotic origin; moreover, we had a
clear history of syphilis. The serum W. R. proved positive as well as
the spinal fluid W. R. The finding of 77 cells per cmm., excess albumin,
and positive globulin test, taken in connection with the entire picture
seems to warrant a diagnosis of CEREBROSPINAL SYPHILIS. If we proceed on
statistical grounds, it might be regarded as more probable that the
hemiplegia is THROMBOTIC in origin rather than hemorrhagic. It appears
that syphilitic cerebral thrombosis rather characteristically occurs
without preliminary symptoms, despite the fact that many cases do show
headache, dizziness, and restlessness as prodromal symptoms.

    1. What is the treatment indicated in the case of Mrs. Meyer?

       It would appear that little or nothing can be done for the
      hemiplegia unless the claims of Franz with respect to
      reëstablishment of a degree of function in certain hemiplegics are
      substantiated. However, the indication of meningitic process as
      shown by the spinal fluid, suggests that the case is not a purely
      vascular one but may be regarded as meningovascular. (Possibly,
      also, we should regard the left frontal depression and scar as
      indicative of a non-parenchymatous and non-vascular process.)
      Accordingly, antisyphilitic treatment should be theoretically of
      some value.

      In point of fact, the patient was given injections of mercury
      salicylate, mercury by mouth, and potassium iodid. Her
      psychopathic depression under this treatment, supported by proper
      hygiene and rest, diminished. However, six months later, the
      patient slipped on a wet floor and fell. Though the impact seemed
      hardly sufficient to cause a fracture, the pelvis was somewhat
      severely fractured. Very probably there was a syphilitic
      rarefaction of the bone. Six months later the patient’s depression
      was still in evidence, though somewhat less than upon admission.
      The blood serum remained positive but the spinal fluid had become
      entirely negative, both in respect to the W. R. and in respect to
      the other findings.

    2. How may one explain the continuance of the depression after the
      spinal fluid had become entirely negative under treatment? It may
      be that while the active process had been stopped, as seems
      probable from the negative spinal fluid, that a permanent
      destruction of brain tissue may account for the depression. We
      recognize this readily in instances of vascular disturbance where
      (as also in this case) the active process being stopped, a
      residual defect remains.

    3. Should treatment have been discontinued on reduction of the
      gumma? It cannot be too often emphasized that the disappearance of
      symptoms in cases of syphilis can not be considered as evidence of
      cure. The neurologist and psychiatrist see only too often cases of
      neurosyphilis occurring in patients who have been declared cured
      at some time previous because the symptoms then present had
      cleared up and remain in abeyance for years.


  =Contrary to various warnings, arteriosclerosis by no means
  absolutely contraindicates intensive salvarsan therapy.=


=Case 108.= Victor Friedberg, 42 years of age, gave the following
history. He acquired syphilis at 22 years. He had “adequate” medical
treatment for two years with inunctions of mercury and mercury by mouth
and potassium iodid. The only secondary symptoms were skin lesions of
the legs; these disappeared upon treatment. Married, Friedberg has one
child, apparently normal. There had been no miscarriages or stillbirths.

At about 34 years, there began to be shooting pains in the legs,
occurring at first about once in three months, but later much more
frequently. These pains were severe, lightning in character, lasting
several days at a time, at which period his head would feel heavy; but
there were no disturbances, crises, or difficulty in locomotion.

At 36 years of age, Friedberg waked up with pain one night, and found he
was unable to move his left leg or hand, and he felt his mouth drawn to
the left. Upon trying to get out of bed, he fell to the floor. In five
hours, however, he was entirely recovered, able to get up and walk
about, and to use his left arm quite normally. He went to sleep, but
upon waking up after an hour, discovered that his left side was again
paralyzed. After two weeks in a hospital, he was able to walk with a
crutch. The arm remained helpless for about a year. Both arm and leg
improved slowly for two years, after which time his condition had
remained stationary. For four years past, there had been no more pain,
but at 42—about two years before admission—the pains returned in his
legs, back, and side. At that time he received four injections of
salvarsan, mercury tablets, and potassium iodid. Three weeks before
admission to the hospital, Friedberg again began having headaches, very
much worse than formerly. At first these headaches were frontal, then
occipital, and there was a feeling as if something were growling inside
of the head. There was a feeling of pressure in front on the head and at
the base of the nose.

=Physically=, Friedberg appeared somewhat older than his assigned age.
There was a degree of general peripheral arteriosclerosis, but in
general the physical examination was negative. _Neurologically_, there
was a left hemiplegia with appropriate increase of the reflexes on that
side, spasticity, Babinski reflex, and an Oppenheim; the pupils reacted
properly; there was no Romberg reaction.

_Mentally_, Friedberg was entirely negative.

The W. R. of the blood serum was doubtful, as was that of the spinal
fluid. There were but two cells per cmm. and there was neither globulin
nor excess albumin in the spinal fluid.

The =differential diagnosis= might lie between cerebral hemorrhage and
syphilitic thrombosis. Thrombosis is much more common as a result of
syphilis than is hemorrhage. The occurrence of the thrombosis during
sleep without premonitory symptoms is also characteristic in syphilis.
Possibly there was a low-grade spinal meningitis at the bottom of the
lancinating pains. Whether the headache is an arteriosclerotic effect or
due to a meningitis not shown in the cerebrospinal fluid is doubtful.
However, the absence of inflammatory products in the cerebrospinal fluid
rather indicates that the headache is of arteriosclerotic origin.
Autopsies, however, warn us that we may have a localized meningitis in
various parts of the cranial cavity without the determination of any
inflammatory products in the spinal fluid.

    1. How shall we explain the doubtful (slightly positive) W. R. in
      the spinal fluid if the case is one of VASCULAR BRAIN SYPHILIS?
      The finding is not unusual in these cases. The W. R. producing
      body is recognized to be of a separate nature from the globulin
      and albumin bodies, and is probably also separate from the gold
      sol reaction producing bodies.

Treatment: The theory of treatment is that any spirochetes that may be
still active in the body should be destroyed. Accordingly, although
salvarsan can certainly have no effect in reproducing nerve tissue, it
nevertheless seems indicated. It is frequently stated, however, that
salvarsan is dangerous in cases of this group. We have not found this
statement correct. In this case, there was a symptomatic improvement, as
far as pain and discomfort went, under salvarsan and iodids.

    2. What precautions should be taken in intensive salvarsan treatment
      of syphilitic arteriosclerosis? Treatment should be begun with
      very small doses of salvarsan, that is, about 0.1 of a gram and
      then the amount slowly increased. The injection should be given
      slowly so as not to put too great a load upon the cardiovascular
      system.

    3. What rôle does the mental attitude of the patient play in a case
      like that of Friedberg? It was quite evident that Friedberg was
      neurotic and that he had a syphilophobia. Consequently some of the
      symptomatic improvement may have been more results of assurances
      offered by the physician and knowledge that he was being treated,
      than results of salvarsan. In some cases mental anguish suffered
      by the patient is of more importance than the actual symptoms of
      the disease and this point must be always borne in mind in
      handling syphilitic patients.


  =Symptoms of intracranial pressure cured by antisyphilitic
  treatment.=


=Case 109.= Mrs. Annie Rivers, a housewife 36 years of age, sought
advice and treatment for severe convulsions which she had had during a
period of several weeks. She left the hospital before being properly
examined, and had several more convulsions, after which she was brought
back in a state of marked confusion. The confusion shortly disappeared
almost completely, and a good history was obtained.

It appears that the patient led a normal life and had had six children,
the last of whom was born about four months before her coming to the
hospital. The first symptoms appeared about a month after the birth of
the child, when, one afternoon, Mrs. Rivers suddenly fell unconscious
while ironing. She remained unconscious for nearly three hours. During
this attack there were no convulsive movements or tongue-biting; and
after the spell, she felt neither lame nor sore, but merely tired. This
was Mrs. Rivers’ statement; but her daughter stated that the patient
really did have convulsive movements. A week later came a second
convulsion, followed by daze and stupor. This second attack lasted two
hours.

About a week before entrance, the patient had remained in bed on account
of dull grinding pain in the left side of the head, below the ear, and
upon this day the patient vomited twice. In addition to the dull
grinding pain, there were pains referred to the ear itself and to the
left side of the head, especially over the left eye; there were no pains
on the right side of the head. The next day the patient was better, but
the day thereafter again remained in bed. The only other symptoms were
cold feelings at times and bright spots in the field of vision.

No =mental symptoms= were observed in Mrs. Rivers except a bit of
depression after her hasty retreat from the hospital the first time.
Upon her second admission, however, after a week or ten days’ residence,
apathy developed together with considerable amnesia for the same facts
she had quite readily remembered a few days previously. Along with the
apathy and amnesia developed considerable headache; and there were
attacks of vomiting.


               =UNTOWARD SYMPTOMS OF THERAPEUTIC AGENTS=

                             =A. SALVARSAN=

                      CYANOSIS      MALAISE
                      RAPID PULSE
                      PERSPIRATION
                      RESPIRATORY DIFFICULTIES
                      FEVER
                      NAUSEA, VOMITING, DIARRHOEA
                      DERMATOSES
                      EDEMA
                      KIDNEY IRRITATION
                      LIVER IRRITATION
                      INTENSIFICATION OF SYMPTOMS
                      COLLAPSE

                              =B. MERCURY=

         SALIVATION
           FETID BREATH
           EXCESS FLOW OF SALIVA
           TENDERNESS OF TEETH—LOOSENING AND FALLING OUT
           SPONGY GUMS—EROSION
           METALLIC TASTE
           NECROSIS OF BONES OF JAW
           SORENESS OF PARETIC AND MAXILLARY GLANDS
           SWELLING AND EROSION OF TONGUE AND MUCOUS MEMBRANES
         GASTRO-INTESTINAL SYMPTOMS
         ANEMIA
         PAIN IN JOINTS
         NEPHRITIS

                              =C. IODINE=

            SKIN LESIONS
            METALLIC TASTE
            SALIVATION
            CORYZA
            URTICARIA (EVEN TO GRADE OF ANGIONEUROTIC EDEMA)
            PAINS
            CONSTIPATION
            INVOLVEMENT OF JOINTS
            FEVER
            SOFTENING AND BLEEDING OF GUMS
            EROSION OF MUCOUS MEMBRANES
            GASTRO-INTESTINAL SYMPTOMS
            ANOREXIA
            WEAKNESS

                                                              CHART 27


On the =physical= side, it is interesting to note that the
ophthalmoscopic examination upon Mrs. Rivers’ first admission to the
hospital was entirely negative, whereas a week later, pronounced
difficulty with vision appeared so that in a few days she was able to
make out only very large type. The fundi now showed hazy and indistinct
disc outlines, with small yellowish areas of fatty degeneration above
the disc, reduction of arterial calibre, and dilated and somewhat
tortuous veins (no projection of papillæ), so that the ophthalmological
diagnosis was chronic neuritis.

The physical examination otherwise was mostly negative. The skin
presented irregular areas covered with silvery scales over the arms and
chest, back, abdomen, and legs (the patient had had psoriasis several
years before). Both pupils reacted to light and distance, though the
right was slightly larger than the left and somewhat irregular. There
was a slight tremor of the tongue and extended fingers. The reflexes
were active, especially the knee-jerks; no abdominal reflexes could be
obtained. The serum W. R. was positive, but the spinal fluid W. R. was
negative. The spinal fluid showed but 3 cells per cmm., but there was a
positive globulin test and an excess of albumin.

=Diagnosis=: After the symptoms had fully developed, it became clear
from the optic neuritis, headaches, and vomiting that a condition of
intracranial pressure existed. In view of the positive serum W. R., it
is natural to conceive that the agent producing the intracranial
pressure was a gumma.

It is, of course, possible that a marked degree of meningitis might be
so localized as to produce the same symptoms. The diagnostician would
crave a pleocytosis of the spinal fluid if a diagnosis of meningitis is
to be made; and there was no such pleocytosis. On the whole, we do not
feel that it is possible to make a diagnosis either of MENINGITIS or of
GUMMA.

=Treatment=: Treatment, however, caused a disappearance of all symptoms.
The treatment consisted of but one injection of 0.3 gram of salvarsan,
followed by a few injections of mercury; whereupon Mrs. Rivers became
much brighter, recovered her vision, lost her headaches, ceased to have
convulsions or vomiting spells.

    1. Is salvarsan contraindicated in cases with involvement of the
      optic or auditory nerves? Such a contraindication exists according
      to prevailing opinion. In this particular case, a hemorrhagic
      retinitis occurred after the injection of salvarsan, but this
      retinitis disappeared along with the other symptoms. On the whole
      we believe that in many cases of optic or auditory nerve
      involvement salvarsan should be used. However, one should never
      lose sight of the possibility of untoward results and should
      advise such treatment only when other treatment seems inefficient.


  =TABETIC NEUROSYPHILIS (“tabes dorsalis”) may show very marked
  improvement as a result of intraspinous therapy.=


=Case 110.= Mr. McKenzie[18] was a retired merchant of 42 years whose
complaint was that he tired very easily, could not make his legs go
where he wished, was unsteady and felt a numbness in his legs. These
symptoms had been in progress for a few months only when the examination
was made. This disclosed Argyll-Robertson pupils, absent knee-jerks and
ankle-jerks, Romberg sign, unsteady gait, moderate ataxia and dysmetria.
The W. R. was negative in the blood serum but positive in the spinal
fluid with 0.2 cc., and there were 107 cells per cmm. With the symptoms
and signs it was therefore easy to make the diagnosis of TABETIC
NEUROSYPHILIS (“tabes dorsalis”).

The patient was given five intraspinous injections of mercuric chloride
in blood serum (mercurialized serum) according to the method of Byrnes.
The dose was 0.001 gm. of mercury. Two weeks after the first injection
the cell count was 58 cells per cmm., the Wassermann was positive only
with 0.4 cc. After the fourth injection there were but 18 cells and the
Wassermann reaction was negative even with 1½ cc. of spinal fluids. The
symptoms had improved to such a degree that the patient had no complaint
whatsoever and considered himself cured.

    1. What are the unpleasant results of intraspinous therapy?
      Frequently there is an exacerbation of symptoms and pain may be
      quite severe after intraspinous injections. This, however, lasts
      only a short period, that is, as a rule less than 24 hours. There
      may be other symptoms of cord irritation as retention of urine or
      lack of sphincter control. A rise of temperature is not unusual.


  =Treatment may alter the W. R. to negative in blood and spinal fluid
  in TABES DORSALIS.=


=Case 111.= Ivan Rokicki was a baker, 43 years of age, who came
complaining of exceedingly severe attacks of abdominal pain with
vomiting. He described these attacks as having occurred periodically for
a number of years, lasting sometimes as long as a week, during which
time Rokicki could not eat or get relief short of large doses of
morphine.

Upon his arrival, Rokicki was seen in one of his attacks; he was curled
up with excruciating pain, and the abdomen was rigid, though it was
impossible to produce additional pain by external pressure. There was
spasmodic vomiting, frequently followed by slight relief from the pain,
which however shortly recurred and caused the patient to cry out in his
suffering. The condition was controlled by opiates but lasted a full
week. The leucocytes remained normal and there was no rise of
temperature. The attack ceased spontaneously.

Save for the pain, Rokicki’s =mental examination= proved entirely
negative. =Physically=, Rokicki was fairly well developed and nourished.
His pupils were slightly irregular: the left markedly larger than the
right; both pupils failed to react to light, and the left pupil also
failed to react in accommodation. There were no other reflex disorders
evident to systematic examination, nor was there sensory disturbance or
speech defect. The heart seemed somewhat enlarged but there were no
murmurs; blood pressure: systolic 150; diastolic 110.

The correct symptomatic diagnosis in Rokicki’s case proved to be gastric
crises, and this diagnosis must perforce be the first to entertain in
view of the chronicity, the periodicity, the non-relation to diet, and
the spontaneous cessation of the seizures. The observation of
Argyll-Robertson pupils was naturally held to substantiate the diagnosis
of TABES DORSALIS.

The possibility of abdominal inflammation could be shortly dismissed on
account of the absence of tenderness (the rigidity in this case was not
accompanied by tenderness), fever, and other characteristic signs. There
was no diarrhoea, such as is found in lead colic, and there was no other
sign of plumbism. Jaundice was absent and there was no special radiation
of pain from the abdomen. One had to think of gastric ulcer and
hyperchlorhydria, and possibly malaria or gastroenteritis.

The pupillary reactions pointed to a syphilitic condition despite the
fact that the lack of reaction to accommodation (over and above the
Argyll-Robertson phenomenon) in the right pupil is not entirely typical.
Accordingly, although there was no areflexia, Romberg sign, or ataxia,
resort was had to the W. R. This however proved negative, in blood and
spinal fluid; nor was there any globulin or excess albumin; there were 5
cells to the cmm., in the spinal fluid.

We are left, accordingly, with characteristic gastric crises;
Argyll-Robertson pupils, slightly irregular; and a somewhat enlarged
heart.

Upon investigation, it appeared, however, that a year before the attack
above described, the patient had been examined and both blood and spinal
fluid found positive to the W. R. At that time, treatment, consisting of
intravenous injections of salvarsan and intraspinous injections of
salvarsanized serum (Swift-Ellis), had been instituted. Whereupon the
laboratory tests had become negative, as above stated, and there had
been no alleviation of the symptoms.

    1. How can Rokicki’s normal deep leg reflexes be explained? The
      abolition of the deep reflexes is of course due to lesions
      properly localized. It is probable that this particular case of
      tabes dorsalis is more truly “dorsal” than most cases; for most
      cases exhibit lesions involving regions lower than the dorsal.
      Both in these dorsal cases and in certain rare cases of cervical
      tabes, the deep leg reflexes are preserved. (See cases Green (30)
      and Halleck (31).)

    2. What is the mechanism by which a characteristic gastric crisis is
      produced? The mechanism is unknown. Some endeavors have been made
      to meet gastric crises by surgery of the posterior roots, on the
      assumption that the irritation causing the pain was located either
      in the posterior ganglion or in the passage of the nerve through
      the meninges. In only a few instances, however, has the result
      been what was desired. In many instances the gastric crises and
      pain continued uninterrupted and in addition came discomfort due
      to the lack of sensation in the part supplied by the severed
      nerve. At present this treatment is seldom carried out.

    3. Should antisyphilitic treatment be continued in such a case? As
      far as our present knowledge of syphilis goes one would hesitate
      to suggest further antisyphilitic treatment, feeling that the
      active process had been entirely stopped as suggested by the
      absence of any positive findings either in the blood serum or in
      the spinal fluid. We should perhaps conclude that there was no
      more activity in this case and that the crises were due to the
      changes that had already taken place in the nerve tissue and which
      could no longer be changed.


  =The literature is in doubt concerning (in fact is preponderantly
  against) the success of treatment in PARETIC NEUROSYPHILIS (“general
  paresis”). Our experience has yielded a number of apparently
  successful results through systematic intensive intravenous
  salvarsan therapy. Example.=


=Case 112.= Albert Forest had always been a successful salesman, but in
the middle of March, in his 46th year, he was arrested for grabbing a
purse from a woman in front of a theatre and running down the street
with it. In court, Forest acted strangely and he was sent to the
Psychopathic Hospital for observation. Upon investigation, it appeared
that his wife thought he had been showing mental changes for about a
year. For example, he would embrace his wife on a street car, or refuse
to pay her fare. He once attempted to hit his son on the head with a
red-hot poker. Now and then he would become sleepy and stupid. He looked
rather older than his age and had a coarse tremor of the hands.
Otherwise, no change could be detected in the physical examination,
either neurologically or otherwise. As for the manual tremor, Forest’s
wife gave a history of considerable alcoholic indulgence on his part.

For several days, nothing abnormal could be detected in the man; and in
particular, his memory for both remote and recent events was very good
and his knowledge of current events was good. Simple arithmetic was easy
to him.

One evening his temperature was found to be 104° F. and no cause could
be discerned for this. The next morning, Forest was discovered in a
stupor, with a complete right hemiplegia. The Babinski reflex, the
Oppenheim reflex, and ankle clonus had appeared on the right side, and
the right arm was spastic.

However, all symptoms of this paralysis had disappeared by four o’clock
in the afternoon, and the paralytic phenomena were replaced with
violence. The patient fought with the attendants and for some time
remained extremely difficult to manage, being confused and subject to
outbreaks of violence with destruction of furniture and other property
about the ward.

=Diagnosis.= At first we were naturally inclined to dismiss the case
with a diagnosis of alcoholism. The transient hemiplegia at once raised
a considerable question of brain syphilis or of brain tumor.

The W. R. of the serum was doubtful. The spinal fluid yielded, besides
marked excess of albumin and much globulin, also a “paretic” gold sol
reaction and 75 cells per cmm. The W. R. was positive.

=Treatment.= The patient was given injections of salvarsan, 0.6 gram,
twice a week, with potassium iodid. After a few weeks improvement
followed, and after several months all the laboratory tests became
negative, the patient was apparently perfectly normal mentally and was
discharged from the hospital, and has remained well for 18 months
without further treatment. The serum W. R. has continued to be negative.

    1. What is the significance of the so-called “doubtful” W. R.? Where
      there is not a complete uniformity the results of the strong and
      weak antigens (see appendix on technique of Wassermann reaction)
      the result is reported as doubtful. In the majority of instances
      repetitions will give a strong positive reaction.

    2. Is the case of Forest to be regarded as one of general paresis?
      Sometimes such cases are termed in the literature _syphilitic
      pseudoparesis_ (see case Burkhardt (58)). The differential
      diagnosis of this group is entirely therapeutic. There are,
      unhappily, no laboratory tests which will suffice in the present
      stage of knowledge to differentiate a case of so-called
      pseudoparesis from general paresis. We are inclined to term the
      case one of GENERAL PARESIS, with recovery, or, at all events,
      with remission.


  =The literature is in doubt concerning (in fact is preponderantly
  against) the success of treatment in PARETIC NEUROSYPHILIS (“general
  paresis”). Our experience has yielded a number of apparently
  successful results through systematic intensive intravenous
  salvarsan therapy. Example.=


=Case 113.= We present the case of Gussie Silverman, a housewife, 35
years of age, among other reasons, for its social interest. The case is,
on the whole, sufficiently typical of GENERAL PARESIS. =Physically=, for
example, the pupils failed to react to light and accommodation and were
unequal, the right being larger than the left. The knee-jerks were
sluggish though equal. The ankle-jerks could not be obtained. The
abdominal reflexes were not obtained. Otherwise, there was no reflex
disorder.

From the =laboratory= point of view, the W. R. was positive in the blood
and in the spinal fluid. There were 80 cells per cmm. and there were an
appropriate globulin and albumin reactions. Mrs. Silverman was rather
poorly nourished and had a slight edema of the ankles.

=Mentally=, she was found on admission to be markedly depressed. It
appeared that during a recent pregnancy, terminated by the birth of a
7–months child, she had fainted several times a day, that since the
confinement she had been very nervous, that she had been asking her
husband not to send her away, that she had refused to leave the house,
that she had become excited even to the point of injuring herself,
especially at night, and that she would go so far as to scratch her
husband, shortly afterward being very sorry for her performances. Before
this last pregnancy there had been four others and the resulting
children were all apparently in good health. Except for the fainting
spells during the pregnancy, it would not appear that the story just
told is at all characteristic of paresis.

However, in the hospital Mrs. Silverman could hardly be got to answer
questions, continually saying, “You know what it is; I don’t have to
tell you.” She claimed so marked a degree of confusion as not to know
where she was and what she was doing. She would beg despondently that
something be done for her, and iterate and re-iterate these claims.
There appeared to be a marked degree of amnesia. Some one, she felt, had
controlled her thoughts and made her do things she did not want to do
and say things she did not want to say, things she did not know she was
about to say. She said, “I feel like jumping around. I couldn’t believe
myself as if I am me. Some one is making me jump around. I used to hear
him talking. I don’t know who it is. I used to keep my eyes open and I
couldn’t move. I feel only I would like to talk, and talk, and talk, and
talk all the time. It seems to me that some one talks in me. I couldn’t
sleep for five minutes. My God, I wish I could sleep! I used to feel
something in my heart. I used to faint. It seems to me I used to see a
funny thing. What it was I can’t tell. It used to talk to me, make me
get out of bed, throw me about, make me do things. O, I don’t know what
it was.”

These not entirely characteristic mental symptoms, together with the
suggestive physical signs and the laboratory examination, caused
treatment to be instituted; under which treatment (intravenous
injections of salvarsan) she improved rapidly. Mental symptoms
disappeared under the administration of 12 injections of salvarsan
within two months. Moreover, the spinal fluid became entirely negative.
Two and a half years have now elapsed since her discharge and she has
shown no return of symptoms. The serum W. R. has always remained
negative although there has been no treatment since leaving the
hospital. There has, however, been no change in the reflexes, which
remain as on admission. The 7–months baby has continued to be perfectly
healthy. Its W. R. is negative, as are the W. R.’s of the husband and
the other three children. It must seem surprising that a healthy child
could have been born from a mother with generalized syphilis as in this
case. However, perhaps there are more instances than we imagine like the
case of baby Silverman.

    1. May a patient be considered permanently cured although there has
      been no recurrence of symptoms for 2½ years and although the
      Wassermann has remained negative? One would hesitate to give a
      definite statement that the patient was cured until more time had
      elapsed. It is quite possible that spirochetes may be lurking in
      some portion of the body without causing the production of
      symptoms or Wassermann bodies and yet ready to break out at any
      time. This hypothesis has added weight from the recent work of
      Warthin already quoted. We advise examination of this patient at
      intervals of not longer than six months for a good many years.

    2. Should the course under treatment cause us to change the
      diagnosis? It has often been stated that a differential point
      between cerebrospinal syphilis and general paresis is the reaction
      to treatment, that is, that a case which recovers could not be
      general paresis. Head and Fearnsides state that if six months
      after beginning of treatment the spinal fluid has become negative,
      the case should be considered as one of cerebrospinal syphilis and
      not general paresis. We do not feel ready to concur in this view
      as we know of no similar logic in medicine. We have many cases in
      which a spinal fluid has remained positive for six months and
      later become negative, so that where the symptoms shown are those
      of paretic neurosyphilis, we are inclined to consider the case
      such until such time as more definite evidence checked by post
      mortem examination causes us to change this point of view.

    3. Do the reflexes change under treatment? The signs of spasticity
      often do disappear under treatment and also when there is no
      treatment. A few instances have been reported in the literature
      where Argyll-Robertson pupils are said to have altered to normal.
      It has never been our good fortune to see such a change nor have
      we seen an absent knee-jerk become normal, as has also been
      reported, except where it is the result of pyramidal tract disease
      superimposed upon the posterior column sclerosis causing a return
      of reflex. This, of course, is not to be considered as a return of
      the normal. (See Case 1.)


  =Some RESULTS of systematic intravenous salvarsan therapy are
  PARTIAL (_e.g._, clinical recovery and persistence of positive
  laboratory tests).=


=Case 114.= Walter Henry was an undertaker in a small town. He was
married and the father of two healthy children. In May, 1914, he began
to lose his appetite. He felt restless and seemed to be losing his grip,
and in August he repaired to a sanatorium, where he remained for two
months. Shortly after leaving the sanatorium, he fainted one day, while
digging a grave, during a spell of great heat. Since that time there had
been numerous “weak spells,” with headaches and general debility,
insomnia, and loss of weight.

In February, 1916, Mr. Henry came to the hospital for advice, but the
trip from a distant part of the state was apparently such a strain for
him that shortly after admission he collapsed. There were no convulsive
movements in this collapse, but the patient was confused and his
breathing was rapid and stertorous. The semi-stupor lasted for about 48
hours. Upon recovery from the stupor, Henry was found entirely
disoriented, much confused, and laboring under the belief that he was
digging a grave. After a time he again fell into a stupor and his
temperature rose to 103° F.

The emaciation of this man was striking and unusual, but systematic
=physical examination= showed no special disease. =Neurologically=,
there were marked tremors, and there were purposeless movements of the
arms. There was a marked speech defect. The pupils were dilated,
regular, and equal, and reacted, though slightly, to light. Nothing
abnormal was noted upon systematic examination of the reflexes.

The W. R. was strongly positive in the blood and in the spinal fluid;
the gold sol reaction was typically “paretic”; there were 16 cells per
cmm., globulin was present, and albumin was greatly increased.

The =diagnosis= GENERAL PARESIS was accordingly made, and treatment
instituted. Intravenous injections of arsenobenzol, at first, and later
of diarsenol, were given, as a rule twice a week (usual dose, 0.6 of a
gram). Mercurial injections and potassium iodid were also given. This
treatment was continued as the patient began to improve. The improvement
was of such a degree that at the end of four months, Mr. Henry returned
to his home and his work. He had had 30 intravenous injections of
salvarsan substitutes. Despite the treatment and the clinical
improvement, the laboratory tests remained essentially unchanged. The W.
R.’s of the blood and spinal fluid remained strongly positive, as well
as also the globulin and albumin; the gold sol reaction was still
“paretic”; the cells stood at one per cmm. The patient has continued
antisyphilitic treatment since leaving the hospital, and has remained
apparently well, with good insight into his condition.

    1. What is the significance of a temperature of 103° in a paretic
      without signs of infection and a normal leucocyte count?
      Temperatures of this type are not infrequent in the course of
      general paresis. They are usually spoken of as “paretic
      temperatures.” Their meaning is not understood, but they are often
      stated to be due to a disturbance of the heat-regulating
      mechanism. Such temperatures may remain elevated for a
      considerable period of time, but the elevation may be very
      transitory. At times they vary, like septic temperatures.

    2. What can be argued from the fact that the cell count became
      normal? If thorough antisyphilitic treatment is vigorously given,
      it will be found that in the vast majority of cases of
      neurosyphilis the cell count will return to normal. It matters not
      whether the treatment be intravenous or subdural. It is very
      difficult, however, to obtain this result in general paresis by
      the use of mercury alone. It cannot, however, be urged that this
      finding has any great prognostic significance as it occurs in the
      cases which do poorly as well as in those which recover
      symptomatically.

    3. Is it safe to give large doses of salvarsan to a patient in a
      stupor? It is not a good plan to give a large dose to such a
      patient on account of the danger of sudden death. This is probably
      due as much to the strain put on the heart as it is to any effect
      on the nervous system, or specific arsenic effect. In this
      particular instance, a dose of 0.15 gm. was the initial injection
      and this was increased five centigrams per injection.


  =IMPROVEMENT IN PARETIC NEUROSYPHILIS (“general paresis”) may become
  evident only after several months of intensive treatment.=


=Case 115.= Henry Ryan was a shipping clerk, 54 years of age, who was
brought to the hospital following a convulsion. For a few months
preceding this period, Mr. Ryan had been failing in his abilities. He
had been very forgetful, showed no energy, and had become very
irritable. He also complained of insomnia and of feeling nervous.

On admission to the hospital, the most striking feature in the mental
situation was that he claimed that he had not slept a wink for three
months, and each day he would solemnly affirm that he had not slept at
all the preceding night, although the records might show that he had
slept eight hours. Argument was of no avail against this conviction. In
addition, his memory was very poor; he showed little knowledge of
current events, and had no ability with arithmetical problems.

=Neurologically= viewed, the points of chief significance were
contracted immobile pupils and a speech defect, especially noticeable on
the repetition of test phrases. The whole picture was suggestive of
general paresis, and this diagnosis was confirmed by the laboratory
findings. It was found that the W. R. was positive in the blood and
spinal fluid, that there was a pleocytosis, positive globulin reaction,
excess of albumin, and a “paretic” gold sol reaction. Consequently, the
diagnosis of GENERAL PARESIS seemed justified, although the patient
denied any knowledge of a syphilitic infection.

Treatment in this case consisted of intravenous injections of salvarsan,
diarsenol, or arsenobenzol, whichever drug was most easily obtainable,
given twice a week in doses of 0.6 gram each. In addition, he was given
occasional injections of mercury salicylate as well as potassium iodid
by mouth. Once or twice a week, 40 to 60 cc. of spinal fluid were
withdrawn. Under this treatment for a period of three months, the
patient showed no improvement whatsoever, either in his mental condition
or in the laboratory findings. However, treatment was faithfully
persevered in, and shortly after the three months, improvement began to
be noticed. At first, the patient began to admit that possibly he may
have slept a few winks some time during the previous six months, for he
said he realized it was not possible for a man to live without sleep for
that period. Then he began to admit that he might have slept a few hours
during the night, and later that he was sleeping pretty fairly. His
memory also showed improvement. His general attitude showed alertness,
and he began to interest himself in his surroundings and in the events
of the world, and finally he gained complete insight into his condition.

In the meantime, that is after three months of treatment, the laboratory
findings began to grow weaker. The gold sol reaction was the first to
decrease in strength, and after four months of treatment, it vacillated
between negative and a mildly positive “syphilitic” reaction. Then the
globulin and albumin became less in amount, and the W. R. began dropping
off in the 0.1 and 0.3 cc. dilutions. As is usually true in those cases
of neurosyphilis that receive adequate treatment, the cell count early
dropped to normal. The W. R. in the blood serum, however, remained
positive.

As the patient’s condition seemed so much better, he was allowed to
leave the hospital at the end of five months. He took things easily for
the following seven months, and then, after being out of employment for
the period of a year, as his health continued good, he decided to return
to work. Before doing so, he entered the hospital again for a lumbar
puncture. At this time, it was found that the cell count was normal,
there was a very faint trace of globulin, possibly a slight increase
above normal albumin content, and a very mild gold reaction. The W. R.
in the spinal fluid was negative including the 1.0 cc. dilution; the
blood serum remained positive.

The patient then returned to his old position and has done
satisfactorily for the past six months. During this entire time, he has
been coming to the hospital for treatment: during the major portion of
the time, about once in two weeks; of late, once in four weeks.

The significant point in this case is that improvement did not show
itself until after more than three months of intensive treatment, and
then the improvement was synchronous with a weakening of the spinal
fluid tests.

It is further significant that his mental and physical condition was
good before the tests had reached anything like normal; and that under
treatment, these tests continued to grow weaker and weaker, until at the
end of a year, they were practically negative.

The case further illustrates the enormous number of injections of
salvarsan preparations that may be given to a patient without causing
any appreciable damage to the general health or to the kidney function.
Mr. Ryan has had more than 60 injections.

    1. How soon after treatment is instituted does improvement usually
      occur in paretic neurosyphilis? In our experience improvement
      usually shows itself in from two or three months of treatment.
      Occasionally the improvement may be very marked shortly after
      treatment is commenced, that is, after three or four injections of
      salvarsan. This is not, however, the rule and as in the case of
      Ryan, it may be only after more than three months that improvement
      is seen. This means that in the treatment of these cases patience
      must be exercised and much work done.

    2. What is the point of withdrawing large amounts of spinal fluid as
      in the case of Henry Ryan? It has been stated that the withdrawal
      of 40 or more cc., of spinal fluid while the patient is under
      treatment has the effect of reducing the intraspinous and
      intracranial pressure and thereby allowing the drug to diffuse
      into the nervous tissue better than it would do under ordinary
      conditions. How much truth there is in this contention it is
      difficult to say and there is as yet no experimental evidence to
      confirm this contention. As a matter of fact, the spinal fluid in
      cases of paresis is usually under increased pressure and it is at
      least plausible to conceive that a reduction of this pressure may
      give some symptomatic relief.


  =Evidence of the activity of syphilis outside the central nervous
  system may be seen in cases of neurosyphilis despite intensive
  treatment.=


=Case 116.= William Rosetti was a speculator, 43 years of age, when he
was brought to the Psychopathic Hospital on account of an outbreak in
which he smashed a showcase at the store where his sweetheart was
employed; he caused so much commotion that he was arrested.

On admission, he was very excited, talking loudly and at length. For
some days it was very difficult to manage him, he was so active. At any
moment, he would insist upon undressing and taking physical culture
exercises. He was very euphoric and expansive, and had no insight into
his condition.

=Physically=, he was a powerfully-built man and in very good physical
condition except for an iritis and moderate thickening of the peripheral
arteries. The =neurological signs= of importance were Argyll-Robertson
pupils, and absent knee-jerks and ankle-jerks. With these findings in
mind, a tentative diagnosis of GENERAL PARESIS was made, and this was
substantiated by the laboratory tests, which gave positive W. R.’s in
blood and spinal fluid, globulin, excessive albumin, slight pleocytosis,
and a “paretic” gold sol reaction.

When the patient’s mental condition was somewhat better, he gave a
history of syphilitic infection 15 years before, for which he had had
almost continuous treatment. As a matter of fact, treatment had been
pretty strenuous because he had recurring skin lesions and iritis. It
was practically impossible to get the skin lesions to heal with mercury,
and it was not until salvarsan was introduced that a good result was
obtained in this respect. After one or two injections of this drug, the
skin lesion disappeared and has never returned. However, at least once a
year, he has had attacks of iritis, and for this reason was still being
treated for syphilis at the outbreak of his psychosis.

He was at once placed on more strenuous antisyphilitic treatment in the
form of diarsenol, semi-weekly, aided by mercury injections. After a few
months of this treatment, his mental condition improved so much that he
seemed to be entirely normal. Treatment was continued, however, without
any abatement, and it was of great interest to note at the end of five
months of such treatment that, although mentally he seemed entirely
well, he had an attack of iritis, which was considered as a sign of
active syphilis. This would appear to indicate the great difficulty of
getting results in certain cases of syphilis with any drugs at our
command at present, as in the iritis we are dealing with a condition
which as a rule reacts fairly readily to antisyphilitic remedies.

    1. Are there different strains of spirochetes showing various
      degrees of malignancy? This question has been discussed at length
      in the literature but there is no satisfactory answer at the
      present time. We must always consider the reaction of the organism
      and the host; and it is true in syphilis, as in every other
      disease, that in some individuals it is more difficult to get any
      therapeutic results than in others.

    2. Was the failure to obtain results by long years of treatment due
      to “drug fastness” of the spirochetes? It has been held that the
      organism of syphilis will develop an immunity after a time to
      mercury and arsenic preparations. This led Fournier to recommend
      intermittent treatment as more efficient than continuous
      treatment. Noguchi has shown that in test tube experiments, the
      spirochetes develop a tolerance to increasing doses of arsenic. It
      must be emphasized, however, that this finding has not been
      established for the conditions _in vivo_. Another explanation of
      the failure of treatment in certain instances has been offered by
      McDonagh, who describes a life cycle of the organism of syphilis
      under the name of _cytorrhyctes luis_, of which he believes the
      spirochete to be merely one form, the other forms not being
      affected by arsenic or mercury.


  =Some results of systematic intravenous salvarsan therapy in PARETIC
  NEUROSYPHILIS (“general paresis”) are partial in the sense that with
  clinical recovery the laboratory tests remain partially or less
  strongly positive.=


=Case 117.= Annie Martin was a charwoman, 37 years of age. She had
applied for relief at a general hospital, to which she was admitted on
the suspicion of nephritis; but upon admission she became markedly
excited and noisy, and spoke of seeing angels and hearing God speak to
her. As the attendants were unable to quiet her, she was promptly
transferred to the Psychopathic Hospital. She maintained that she had
been sent to the Psychopathic Hospital through the spite of the general
hospital doctors, and she claimed that other people were also attempting
to work her harm for the purpose of taking her children from her. Visual
and auditory hallucinations were marked, as was the patient’s loquacity,
irritability, and flight of ideas. However, she seemed entirely oriented
and her memory appeared to be intact. She was able to explain somewhat
clearly her supposed condition. The voices told her that somebody was
after her and that her soul belonged to the devil; that she was to be
married but that her soul was to be damned. These voices probably
belonged to priests. She was under the impression that she was going to
be sent to an electric chair and said, “I think I am coming to the end
and I want a pair of rosary beads before the end comes.”

This patient’s pupils were markedly unequal and entirely stiff to light
and accommodation. =Neurologically=, however, there were no other
symptoms. There was a slight trace of albumin in the urine and there
were no casts.

The psychiatric =diagnosis= in this case would off-hand undoubtedly be
dementia praecox. Yet the stiff pupils are almost proof positive of
neurosyphilis. If further proof were necessary, it is found in the
laboratory tests, which showed a positive W. R. of the serum and fluid,
with a “paretic” gold sol reaction; there were 22 cells per cmm., there
was excess albumin, and a positive globulin reaction.

Under intensive antisyphilitic treatment, there was a slow improvement.
After several months, the patient was entirely free from mental
symptoms; the spinal fluid tests became entirely negative except that
the gold sol reaction has remained strongly positive.

    1. Should treatment be continued in the case of Annie Martin in
      spite of the clinical recovery and the negative tests except the
      gold sol? We would again emphasize that it is unreasonable to
      suppose that a long-standing case of syphilis can be cured in a
      period of a few months of treatment and while the tests may become
      negative, it would seem foolhardy to stop treatment on this
      account. We do know that in many cases a Wassermann reaction
      remaining negative for many months may again become positive,
      indicating that the negative reaction did not mean cure but rather
      the absence of the Wassermann bodies in the circulation at the
      time the test was made.

    2. What is the significance of the paretic gold sol reaction when
      the other tests have become negative? As previously stated, the
      gold reducing substance in the spinal fluid seems to be different
      from the substances which give the other pathological reactions.
      We should feel in this case that the process which was producing
      these gold reducing bodies had not been stopped, in other words,
      cure was not complete.

    3. Should one make a diagnosis on the “paretic” gold sol reaction
      alone? The so-called paretic gold sol curve is not always
      indicative of general paresis or even of syphilis but may occur in
      non-syphilitic conditions as brain tumor, multiple sclerosis, etc.
      In our experience we have seen no case of _untreated_
      neurosyphilis in which the gold sol alone was positive, that is,
      in cases in which therapy has not changed the findings in the
      spinal fluid. In our experience the gold sol reaction has been
      fortified by one or several of the other tests as the W. R.,
      globulin test, pleocytosis.


  =Some effects of systematic intravenous salvarsan therapy in PARETIC
  NEUROSYPHILIS (“general paresis”) are limited to the laboratory
  findings without clinical improvement.=

Two examples of such limitation are offered: William Roberts (118) and
John Silver (119).


=Case 118.= A bank teller, William Roberts, 39, was sent to the
Psychopathic Hospital for a depression so marked that he had become
entirely unable to work or care for himself. The story was that some
money had been left him by his uncle, that Roberts could not prove his
right to the money, and that depression, insomnia, and occasional
periods of confusion had followed during a period of about five months.

On admission, Roberts appeared wholly disoriented and unable even to
give his correct age. Attention could not be held, and the patient would
slide off into statements like: “Oh, I made a mistake, I fooled a lot of
people, I have a terrible disease, they are going to get it, they are
going to get me,” etc., etc. There was great difficulty in thinking, and
a marked reaction of fear. This cluster of phenomena certainly suggested
very strongly the diagnosis of manic-depressive psychosis.

=Neurologically=, Roberts proved quite negative except that the tendon
reflexes were very active and the pupils reacted somewhat sluggishly to
light. The blood serum W. R. was negative. No history of syphilis could
be obtained; nevertheless, Roberts kept dropping remarks about the
terrible disease from which he was suffering. It seemed best to proceed
to lumbar puncture, and the spinal fluid disclosed a positive W. R.,
globulin, increased albumin, pleocytosis, and “paretic” gold sol
reaction.

The =diagnosis= of GENERAL PARESIS was accordingly made. During the next
year and a half, no improvement was made; a slight speech defect was
developed, and tremors of the hand and tongue appeared.

The effect of treatment is particularly instructive. Only after 18
months in the hospital was intensive antisyphilitic treatment
instituted; but after a few months of this treatment the W. R. of the
spinal fluid had become negative, the cells normal in number, globulin
absent, albumin present only in normal amount. Only the gold sol
reaction remained positive. It is still of a paretic type. Treatment,
however, did not succeed in altering the patient’s mental condition in
the slightest. At the end of many months of treatment, we still confront
a man showing marked psychic symptoms and a “paretic” gold sol reaction
without other laboratory signs.

    1. What is the significance of the practically negative tests in
      this case without clinical improvement? One must believe that the
      tests became negative as the result of treatment, and that this
      change in the tests was due to the clearing up of some
      inflammatory reactions which were present. This may mean that the
      syphilis had been reduced to inactivity or latency if not cured,
      or at least that there was no activity sufficient to cause a
      positive W. R. in the blood serum, whereas whatever activity was
      present in the brain was in such a region that it did not cause
      any reacting substances to be cast into the spinal fluid. This
      would not mean that there would necessarily be any return of
      function already lost, because this may be considered as a
      permanent loss which cannot be compensated for. As to these tests,
      we now feel that the case should remain stationary; that is, that
      no new symptoms will be added. However, we believe that it is
      somewhat premature with our present knowledge to make this claim
      very forcibly, and would rather suggest that this case be
      considered as demonstrating an interesting fact, the meaning of
      which can be learned only after a period of years.

    2. Why does the gold sol reaction remain strongly positive when all
      the other tests become negative? As already pointed out, above
      (Case Martin (117)) there is no known rule about the disappearance
      of one or other of the abnormal findings in spinal fluid under
      treatment, and we can at present offer no explanation of this
      phenomenon. It does, however, illustrate how careful we must be in
      drawing any conclusions from tests in cases that are being
      treated.


  =Diminution in the spinal fluid tests may occur in treated cases of
  neurosyphilis without clinical improvement.=


=Case 119.= John Silver, a man 29 years of age, presented classical
symptoms of GENERAL PARESIS: He had a convulsion shortly before his
admission to the Psychopathic Hospital, his memory was poor, he was only
partially oriented, he was very euphoric and expansive—thought he had
millions, that he was the Czar of Russia, and so on. His tendon reflexes
were very much increased and there was a marked speech defect. The W. R.
of both blood and spinal fluid were strongly positive; the spinal fluid
showed globulin, increased albumin, pleocytosis, and a “paretic” gold
sol reaction. There was, therefore, no question about the diagnosis, and
the patient was at once put under antisyphilitic treatment. This was
continued for five months; slowly the intensity of the reactions in the
spinal fluid diminished. At the end of the five months, there was the
very slightest possible trace of globulin, with a doubtful increase in
albumin, one cell per cmm., and a mild syphilitic gold sol reaction. The
W. R.’s in the blood and spinal fluid, however, remained strongly
positive. There was no mental improvement coincident with the weakening
of the spinal fluid tests, and at the end of the five months, the
patient had a series of convulsions in which he died.

This case is given as a contrast to Case Henry (114) in which clinical
improvement occurred without diminution in laboratory tests; in the case
of John Silver, marked diminution in the intensity of these tests had no
prognostic significance. This was in keeping with the condition as shown
in Case Roberts (118) where, while the gold sol was the only test to
remain positive, the patient did not improve mentally.

    1. What is the explanation of the lessening of the pathological
      elements in the spinal fluid under treatment? We have seen that
      the various findings may occur independently of one another, and
      we must admit that we do not know definitely what it signifies, or
      why one may be present or absent. It has been held by Head and
      Fearnsides that the findings in the spinal fluid represent
      conditions in the spinal cord and spinal meninges, or at the base
      of the brain only, and not conditions elsewhere. This is in
      keeping with our finding that the gold sol reaction in the spinal
      fluid post mortem very often differs from that in the ventricular
      fluids or cerebral, subdural, and subpial fluids. And further, we
      have found that during life the findings in paresis in the spinal
      fluid may differ markedly from those in the third ventricle, and
      that the change in the fluid in these two areas under treatment
      may not occur simultaneously.


  =Systematic intensive treatment of PARETIC NEUROSYPHILIS (“general
  paresis”), including intraventricular injections of salvarsan, may
  entirely fail.=


=Case 120.= James McGinnis, aged 39, came to the hospital on a
stretcher, semi-conscious, moaning, unable to reply to questions; there
were signs of a right hemiplegia.

The next day, McGinnis cleared a little and became able to utter a few
words. His wife said that he had been entirely well up to four years
ago. At that time he was struck in the eye by the head of a hammer that
flew off the handle. Diplopia had developed, but disappeared.

Only two years later did a marked change appear. McGinnis became
careless as to personal appearance. Seemed absent-minded, apathetic and
drowsy; he would fall asleep in his chair or while at work. He lost his
position and became apprehensive, making not very strenuous efforts to
find work, and finally consulted a physician. The physician told him
that he had a sluggish liver and gave him calomel.

Six months later, McGinnis was restored to his position as foreman, and
his work remained satisfactory for some six months. Then (about six
months before coming to hospital), his speech became slow and somewhat
unintelligible. He quit work, saying that his speech was going from him
and that he might be considered to be drunk. His memory grew rapidly
worse. There was improvement after a vacation and he returned to work,
but continued to be ataxic, complained of vertigo, and fell down several
times, though without loss of consciousness. On the very day of his
admission to the hospital, in attempting to get out of bed, he fell, and
psychotic symptoms at once appeared. There was slight improvement again
with entire disappearance of all paralysis after a few days, a slow
clearing up of the speech disturbance, and a certain return of memory.

=Physically=, there was little to note. =Neurologically=, the left pupil
failed to react to light. The tendon reflexes were all very active, and
more active on the left side. Other abnormal reflexes were absent.
Improvement continued for a number of weeks, but the patient never
recovered from his speech defect, and his memory remained impaired.
Irritable at times, McGinnis was for the most part very happy and sure
he would get well. The W. R. of the blood serum was negative, but the
spinal fluid reaction was strongly positive, even down to 0.1 cc. The
globulin and albumin amounts were excessive. There was a “paretic” gold
sol reaction. There were 7 cells per cmm. The diagnosis of GENERAL
PARESIS was made.

Intravenous injections of salvarsan, arsenobenzol or diarsenol were
made, and intramuscular injections of mercury, and potassium iodid by
mouth were given. No real improvement occurred after a certain initial
betterment; the spinal fluid yielded no changes. Diarsenolized serum
according to the Swift-Ellis technique was then injected into the third
ventricle. Under this treatment also there was no change for the better
over a period of several months. The patient died suddenly after a
series of convulsions, apparently from paralysis of respiration.

    1. What are the causes of hemiplegia and confusion or
      unconsciousness? We must consider epilepsy, brain tumor, cerebral
      thrombosis, cerebral hemorrhage, multiple sclerosis, cerebral
      spinal syphilis, and general paresis.


  =MILD TREATMENT, often thought “adequate,” MAY FAIL, WHEN INTENSIVE
  TREATMENT PROVES SUCCESSFUL.=


=Case 121.= Arthur Bright, a printer, had acquired syphilis in his 49th
year, some six months before examination. He had been treated during
these six months by three injections of salvarsan, injections of
mercury, and mercury by mouth. He had been apparently cured until about
a month before admission. He had fallen without warning from his chair
in a convulsion accompanied by unconsciousness, which lasted about two
hours. The patient had since been feeling rather peculiar. For instance,
time seemed to flow too rapidly. Sometimes the patient had had
difficulty in talking.

=Physically=, nothing abnormal could be found either in general
condition or =neurologically=. The patient was, however, incontinent.
=Mentally=, he was apathetic and unalert, even paying no attention to
his outside physician when he came to visit him.

The =diagnosis= of cerebrospinal syphilis already suggested by his
history was confirmed by the laboratory tests, which showed a positive
serum and spinal fluid W. R., paretic gold sol reaction, 41 cells per
cmm., an excess of albumin, and a positive globulin test.

    1. What is the prognosis in cerebrospinal syphilis in the early
      secondary stage? The prognosis appears very good provided that
      intensive treatment be given and provided that no vascular insult
      or other focal destructive lesion occurs before treatment has had
      time to do its work.

    2. Why did not the “effective” (?) treatment for the syphilis,
      dating from the primary lesion, succeed in staving off the
      cerebrospinal syphilis? It remains a question whether the
      treatment by three injections of salvarsan was efficient in this
      particular case. Of course, it may prove true that no treatment
      whatever in the present stage of knowledge will stave off
      cerebrospinal symptoms in certain cases.

=Treatment=: Bright was given intravenous injections of diarsenol twice
a week, with occasional injections of mercury salicylate. After two
weeks, the patient seemed markedly improved, and continued to improve
rapidly. He was symptomatically well at six weeks. The spinal fluid had
then become negative, although the serum W. R. had remained positive.

After discharge from the hospital, Bright returned to his work, but
continued to take the diarsenol treatment weekly, and two months later
the serum W. R. became negative.

Small injections of diarsenol at intervals of a month were continued,
and Bright remained perfectly well for four months, when a peculiar
seizure developed and lasted for several hours. This seizure consisted
in a sort of somnambulism in which Bright stood up at a table, making
marks on paper, and could not be persuaded to desist. After this
seizure, Bright re-entered the hospital, again showed no mental or
physical symptoms and no abnormalities of blood or spinal fluid.

    3. What is the explanation of this seizure? It is possibly due to a
      small vascular insult, for which potassium iodid may be suggested
      with precautions as to hygiene and continued observation. He has
      since remained entirely well.


  =Another example where MILD MEASURES (though conceived to be
  “adequate”) SEEMED TO BE LEADING TO FAILURE; INTENSIVE THERAPY
  SUCCESSFUL.=


=Case 122.= Levi Morovitz, a waiter, 39 years of age, came to the
hospital with evidences of an old left hemiplegia, including the left
side of the face (there was a left-sided Babinski, Gordon, and
Oppenheim, and all the reflexes were fairly active; sluggish pupil
reactions, Rombergism, and speech defect). Morovitz was much depressed,
very slow in thinking processes, had a marked memory disturbance in
general and apparently much deterioration mentally.

A history was obtained to the effect that Morovitz had acquired syphilis
at about 33, but that he had received practically continuous treatment
ever since at a dispensary. He had, in fact, received four injections of
salvarsan a year before coming to the hospital. Of late, Morovitz had
become much more cheerful and talkative, imagining he could do great
things if he had money. He had begun to eat very rapidly and to be very
nervous. His feet had begun to drag; a distinct speech defect developed,
but from this he had recovered. About six weeks before entrance,
Morovitz had a shock, which left him with the left hemiplegia above
mentioned and with considerable headache.

Even while the preliminary examination was being performed, Morovitz
developed a minor seizure without loss of consciousness. First came
severe pain over the frontal region, which grew in severity so that the
patient held his head in his hands. A bit later, twitching movements
began in the thumb and in the fingers of the left hand, and the small
muscles of the extensor group of the thumb and third finger showed
contractions. These contractions grew more general and the excursions of
the fingers greater, until finally every finger of the left hand became
involved, whereupon movements of the same sort, though of smaller
amplitude, began in the other hand. Finally the left arm began to jerk
with alternate contractions of the biceps and triceps. The whole seizure
lasted more than five minutes. During the seizure there was dizziness
and pain in the head, chiefly on the right side.

=Diagnosis=: The attention is at once arrested by the data of the
seizures described. It appeared that we had to assume an irritation of
the right side of the brain, possibly due to vascular disease, or to
brain tumor, or perhaps to syphilis. The shock with residual hemiplegia
would be consistent enough with any of these diagnoses. However, the
history seemed somewhat long for brain tumor. Nor were there any
definite symptoms of intracranial pressure. “Adequate” treatment
unfortunately does not rule out syphilis. The comparatively early age
(39) of the patient makes it difficult to explain the vascular disease
except on the basis of syphilis. Add to the hemiplegia the euphoria and
grandiose ideas of a year’s duration, and we arrive at a diagnosis of
neurosyphilis, probably PARETIC NEUROSYPHILIS.

The laboratory tests showed the W. R. of the serum and spinal fluid
positive, 80 cells per cmm. in the fluid, large amounts of globulin and
albumin, and a “paretic” type of gold sol reaction.

To be sure the Jacksonian seizure is not especially characteristic of
paretic neurosyphilis, and even suggests a local irritation in the motor
area, such as a localized meningitis, possibly of a diffuse gummatous
nature.

This patient was put on intensive antisyphilitic treatment, namely,
salvarsan twice a week and injections of mercury. He recovered rapidly.
After a few months he left the hospital, and after treatment had
continued for a year, he resumed his work by which time both blood and
spinal fluid had become negative.

It must be recalled that this patient had from the time of his infection
what has been considered good antisyphilitic therapy, in spite of which
he developed after a period of years, the symptoms and signs of
neurosyphilis in its most dangerous form. The conclusion must be drawn
that however good such treatment is for the majority of cases, it was
insufficient for Morovitz. That the early failure to cure was not due to
any “drug fastness” of the spirochete or to any peculiarity of strain is
proved by the result of more vigorous antisyphilitic treatment which
caused an apparent if not a real cure. With our modern methods of
treatment checked by Wassermann reactions and spinal fluid examinations,
treatment is given according to the _needs of the individual patient_
rather than according to general preconceptions. We have reason to
believe that under these conditions there will be fewer cases developing
late symptoms on account of insufficient treatment given even to
patients who are willing to co-operate to the last degree.

The fact that Morovitz had no apparent symptoms for several years led to
rather desultory treatment chiefly in the form of mercury by mouth.
Previous to the time when the W. R. and lumbar puncture were available,
the physician had no exact means of determining cure except the
non-appearance of symptoms. But a period of years of quiescence before
the outbreak of symptoms referable to the involvement of the nervous
system is characteristic of syphilis. With this knowledge in mind it is
evident that today the care of a syphilitic patient must be guided, in
part at least, by examinations of the spinal fluid and W. R.


  =Salvarsan treatment may even occasionally be of value in simple
  FEEBLEMINDEDNESS due to congenital syphilis.=


=Case 123.= The somewhat unattractive Robert Matthews was brought, at 5
years of age, to the hospital for backwardness of mind. It appears that
the patient was born at term, with instruments, that he began to talk at
a year, and to walk at 13 months, but that in point of fact, he had not
talked intelligibly to date. Robert had never played with other children
and is regarded by his parents as backward. In fact, Robert’s sister—a
year his junior—is much brighter. Robert had had scarlet fever but
without sequelae.

Examination by the Binet scale showed that, although he is actually 5½
years, he graded by the Binet scale at 4 and was regarded as
feebleminded.

The =physical examination= showed a general adenopathy and prominent
frontal bosses. In the study of the family history in the search for an
etiology for the evident feeblemindedness, little or none could be
found. There were no miscarriages or stillbirths; the parents were
living and well. There was only the one sister above-mentioned, who is
brighter than Robert.

The advantage of a routine W. R. is here well shown, for the W. R. in
the serum was positive.

    1. What is the prognosis of cases of syphilitic feeblemindedness? It
      would appear that every case is an individual problem.

    2. What is the effect of treatment? Robert Matthews was given
      mercury protoiodid ⅛ gr., three times a day, by mouth, for three
      months. The protoiodid was followed by ten injections of
      salvarsan, average: 0.15 gram, during six months. At the end of
      this period, the W. R. in the blood had become negative. A
      re-examination by the Binet scale, when Robert was 6–5⁄12 years of
      age, showed him to grade at 5⅖, so that one might conclude that
      Robert had shown more mental progress in a year than he had
      previously.

Note: The patient’s sister, 4 years of age, is attractive and bright,
measuring beyond her actual age according to the intelligence tests.
However, the girl was found to have a positive W. R. It may be that
Robert and his sister illustrate the hypothesis of Mott: that the
syphilitic virus becomes less potent as the years go on, and that the
younger children in the family are less affected than the older.
However, in our series, there are a number of instances in which this
hypothesis is not substantiated.

    3. What is the share of syphilis in the production of
      feeblemindedness? The percentage of syphilitic cases found in
      institutions is not high. A variety of cases have been proved to
      be congenitally syphilitic in the absence of a positive serum W.
      R.

Fernald[19] has charted a comparison of cases diagnosticated “moron”
(that is, feeblemindedness proper, in the narrower English sense) and
“imbecile.” Fernald says that the morons have, as a group, many more bad
family histories than have the imbeciles, to quote—“Only 70% of the
[imbecile] group have bad family histories. This at first seems
surprising, but when we consider that more of our syphilitic, traumatic,
and sporadic cases tend toward the lower end of the feebleminded group,
and when we remember that with such cases there is often a seemingly
normal family tree, the drop in the curve appears logical.”

The situation with the idiots, of whom only 38 came into Fernald’s
study, was similar; 12 out of 38, or 32%, of idiots, had good family
histories. On these figures, how unfortunate it would be to dub
feeblemindedness hereditary! It is true, however, that 68–70% of the
idiots and imbeciles, judging by W. E. Fernald’s intensive study, do
have bad family histories.

Goddard[20] states that of all the causes of feeblemindedness, there is
perhaps none for which there is less evidence than syphilis. Goddard
found syphilis in 27 of his intensively charted cases of
feeblemindedness, that is, in 9% of all his charts. He finds the
majority of the syphilis cases occurring in relatives of the
feebleminded to be in the hereditary group; for example, of 164 charts
in the hereditary group, 17, or 10%, showed syphilis. In 34 charts in a
group termed “probably hereditary” 3, or 9%, showed syphilis. Of 37
charts in the group termed “neuropathic” 4, or 11%, showed syphilis,
whereas in 57 “accident” and 8 “no cause” groups, there were but 2 (4%),
and one, or 13%, showing syphilis. However, Goddard concedes that much
more careful studies are necessary if we are to give an exact evaluation
of syphilogenic feeblemindedness.

The first ten of the Waverley Anatomical Series are shortly to be
described in a forthcoming publication.[21] Of these ten cases, four
showed some slight evidence of chronic inflammatory changes, indicating
the possibility of a syphilitic or similar infectious condition. These
cases, be it remembered, were not cases of juvenile paresis, but cases
of what, for the lack of a better name, may be called “ordinary”
feeblemindedness.

If all or any of these processes are syphilitic, the syphilis is
virtually extinct. The cases had not been treated for syphilis and were
not regarded as syphilitic, though several of them showed a few stigmata
somewhat suggestive of syphilis. The anatomical conclusion at this time
is still doubtful.

As in the text case, the hypothesis of syphilis as a direct cause for
simple feeblemindedness must be entertained for a few cases. In any
event, it would not seem logical to let any institution for the
feebleminded run without a Wassermann analysis of the population. In
addition to the Wassermann data from the blood serum, osteological data
from the X-ray have proved of occasional value for syphilis diagnosis in
this as in other groups.


      “Within the gates of Hell sat Sin and Death.”

                                  Paradise Lost, Book X, Line 230.




                     VI. NEUROSYPHILIS AND THE WAR


Although the American toll of war syphilis has not yet begun and
although the crop of neurosyphilis due to war infections may not arrive
until the mid or late twenties of the century (witness German experience
in the eighties of the last century), it seems proper here to give a
number of abstracts _re_ neurosyphilis as it has developed in the war.
Available reports from English, French, and German sources have been
levied upon for the years 1914–16.

It is clear that all the armies have had their share of
neurosyphilitics, some clearly diseased before enlistment, some
developing symptoms as a result of training, stress, or shock, others
hastened or made worse by war conditions.

There are important questions of pension, retirement, and compensation
for neurosyphilitics. No previous war has had the benefit of the
Wassermann reaction and other exact tests bearing upon the nature,
progress, and curability of neurosyphilis.

That we shall have our fill of pension and other problems can already be
seen from continental reports. Thibierge,[22] for example, states that
syphilis has become a real epidemic among the French soldiers and
mobilized munition workers.

Hecht[23] of Austria claims that no less than an equivalent of 60 army
divisions have been temporarily withdrawn from fighting on the Teutonic
side for venereal diseases. He commends Neisser’s idea that salvarsan
and mercury should be given in the trenches. While hundreds or thousands
of Austrians are sick with syphilis, sound and healthy men are being
shot down in their stead. The diagnosis of syphilis, according to Hecht,
ought to be a signal for sending the men to the front. He makes even the
somewhat bizarre suggestion that special companies of syphilitics should
be formed, for convenience of treatment, on the firing line.

Not only is the syphilis problem in the army of importance to the
military authorities, but also to the civil population, and perhaps to
them a greater problem. With the great increase of venereal disease that
is the result of the conditions of army life in war time, there will be
a considerable percentage of cases developing neurosyphilis a number of
years after discharge from the army, but caused by the infection
acquired during service. In addition many men will bring the disease
back to America in an infectious stage and spread it. We would advocate
that the names of all soldiers who had acquired syphilis and were not
considered cured at time of discharge should be given to health
organizations in their home states that they may be given further care.

These practical and several theoretical questions are raised by the
following fourteen cases which we have condensed from their sources.


  =A tabetic lieutenant “shell-shocked” into paresis? Case from Donath
  of Vienna.=


=Case A.=[24] An apparently competent German professor in an
intermediate school, a lieutenant of infantry reserves, 33 years old, on
the 17th August, 1914, was stunned for a while by the shock of a
cannon-firing 25 feet away. Urination became difficult. Headaches and
limb pains ensued, with paralysis of fingers, gastric troubles,
forgetfulness especially for names, insomnia, and general scattering of
mental faculties.

=Neurologically=, the pupils were irregular, left larger than right;
Argyll-Robertson reaction. Right knee-jerk livelier than left. Achilles
reactions absent. Slow and dissociated pain reactions in feet, lower
thighs and lower quarter of upper thighs, with hypalgesia or analgesia.
Station good; gait steady. Mentally depressed, slow of thought. Speech
poor and of indistinct construction (mild dementia). Calculation ability
poor. No pleasure in work.

Wassermann reaction of serum weakly positive.

It seems that for a year the patient had been subject to spells of
anger. He was irritated by his wife who had been nervous since an
earthquake.

_On the occasion of the earthquake_, 1911, the patient himself had had a
spell of _difficulty with urination_. The spell had lasted two or three
months. The patient had had a chancre in 1902, “cured” in four or five
weeks with xeroform. In 1908, when about to marry, he had had six
mercurial inunctions.

    1. Is this a case of traumatic paresis? From the somewhat meagre
      account it would appear that Donath’s lieutenant should rather be
      termed “shell-shock paresis,” in the sense of a paretic
      neurosyphilis liberated by shell-shock (using shell-shock in the
      sense of a shock _without_ direct brain injury).

    2. What compensation is due such a man as Donath’s lieutenant? The
      ordinary principles applicable to traumatic paresis are not here
      in point, since no symptoms pointing to trauma of brain ever
      supervened. See discussion under Case G.

    3. How frequent is paresis in armies? R. L. Richards in White and
      Jelliffe’s Treatment of Nervous and Mental Diseases writes as
      follows (of course concerning peace times):

      “The French estimate that paresis cases are 7 per cent of all
      their military cases. The German estimate is 6.6 per cent. In our
      own army at the Government Hospital for the Insane, of 490 cases
      of mental diseases among officers and enlisted men, 37, or 7 per
      cent, were paresis. During the Russo-Japanese War, in the Russian
      Psychiatric Hospital at Harbin, the percentage of paresis was 5.6
      per cent among the cases developing at the front.”


  =A French soldier “shell-shocked” (also burial) into incipient tabes
  dorsalis? Case from Duco and Blum of Paris.=


=Case B.=[25] A French soldier was buried by effects of shell explosion
September 8th, 1914. He sustained no wound or fracture.

Incontinence of urine developed. Anesthesia of penis and scrotum.
Reflexes absent; pupils sluggish. Wassermann reactions suspicious.

The diagnosis =tabes dorsalis incipiens= was made (hematomyelia of conus
terminalis eliminated).

The patient was estimated to be “40% incapacitated,” according to the
French “_échelle de gravité_” of conditions. A full pension would not be
justified in the opinion of the French authors.

    1. Is there evidence of an increase or exacerbation of tabes
      dorsalis in the war? Birnbaum,[26] reviewing German war neurology,
      quotes Weygandt as believing that the war has probably had to do
      with the production of both tabes and paresis in many instances.
      Other cases, however, have merely been made worse by the war
      stress. Thirdly, there are cases in which the war stress has done
      no harm whatever. Westphal has seen both tabes and paresis develop
      in men who had never before shown any mental or physical symptoms
      whatever, and accordingly, Westphal must be counted among those
      who regard war stress as a liberating factor for these diseases.
      Redlich and Donath are cited in the same connection. (The case of
      Donath is the case presented above as Case A.)

      A very interesting claim was made by Cimbal to the effect that he
      found many examples of paresis developing in the early period of
      the war, particularly in November and December, 1914. Later,
      according to Cimbal, cerebrospinal syphilis and tabes became more
      prevalent.


  =Neurosyphilis in a German recruit, possibly AGGRAVATED ON military
  SERVICE. Pension not allowable. Case from Weygandt.=


=Case C.=[27] A German, long alcoholic and thought to be weakminded,
volunteered, but shortly had to be released from service. He began to be
forgetful and obstinate, cried, and even appeared to be subject to
hallucinations. The pupils were unequal and sluggish. The uvula hung to
the right. The left knee-jerk was lively, right weak. Fine tremors of
hands. Hypalgesia of backs of hands. Stumbling speech. Attention poor.

It appeared that he had been infected with syphilis in 1881 and in 1903
had had an ulcer of the left leg.

The military commission denied that his service had brought about the
disease. In the phrase of the Canadian Pension Board the German
commission would probably have rendered a report “aggravated on
service,” not “by service.” (See Canadian cases D, E, and F.)

    1. Has paresis increased in the war? Both French and German figures
      controvert the claim. Marie, for example, found not a single
      paretic amongst the skull injury cases at the Salpétrière. Most
      authors are found demonstrating cases which they clearly regard as
      in some way produced or unfavorably influenced by the war. There
      seems, therefore, to be a little inconsistency between the general
      statement that paresis has not increased in the war and the
      somewhat frequent cases described as occurring in and modified by
      the war. However, Bonhoeffer, on the basis of nine months’ war
      experience, also holds it to be probable that paresis is no more
      frequent in the field than in the home population.

    2. Is the old syphilitic especially liable to break down under war
      conditions? According to Richards, Shaikewicz says that in the
      Russo-Japanese war paresis was noted especially among the officers
      and non-commissioned officers, and that it was undoubtedly
      hastened in its development by war conditions. Steida says that
      while ordinarily we find paresis developing twelve to twenty years
      after the primary sore of syphilis, in these cases it developed in
      five to ten years after the primary sore. Some of the cases
      progressed with unusual rapidity. It was also noticed that among
      soldiers from the front, under treatment, evidences of syphilis
      were present in 20%, while among the other soldiers under
      treatment, evidences of syphilis were present in 1.6%. Undoubtedly
      the old syphilitic is especially liable to break down under war
      conditions.

      But, on the whole, the German authors in this war find no evidence
      favoring Steida’s claim of the hastened post-infective outbreak.

      3. How did it come about that the efficient German system
      permitted this alcoholic and weakminded syphilitic to enter the
      army? As will be seen, he was a volunteer. In general, the German
      system has been supplied with army surgeons who have been trained,
      not by brief and “brush-up” courses, but by longer periods,
      sometimes two years in duration.


  =Syphilis contracted before enlistment, “AGGRAVATED BY SERVICE.”
  Canadian case, courtesy of Dr. J. L. Todd, Canadian Board of Pension
  Commissioners.=


=Case D.= A laboring man, 42, who always strenuously denied syphilitic
infection, proceeded to France eight months after enlistment. He had not
been in France three weeks when he dropped unconscious. He regained
consciousness, but remained stupid, dull in expression, and with memory
impaired. His speech was also impaired. There was dizziness and a
right-sided hemiplegia.

He was confined to bed four months and was then “boarded” for discharge.

=Physically=, his heart was slightly enlarged both right and left;
sounds irregular; extra systoles; aortic systolic murmur transmitted to
neck; blood pressure 140:40. Precordial pain, dyspnea.

=Neurologically=, there was a partial spastic paralysis of the right
thigh which could be abducted, could be flexed to 120°, and showed some
power in the quadriceps. There was also a spastic paralysis of the right
arm, but the shoulder girdle movements were not impaired. There was a
slight weakness on the right side of the face. There was no anesthesia
anywhere.

The deep reflexes were increased on the right side, Babinski on right,
flexor contractures of right hand, extensor contractures of right leg,
abdominal and epigastric reflexes absent, pupils active, tongue
protruded in straight line.

Fluid: slight increase in protein. W. R.+++

The Board of Pension Commissioners ruled that the condition had been
aggravated _by_ service. (See Case E, “aggravated _on_ service.”)

    1. In view of the fact that the majority of the cases here
      abstracted happen to be in common soldiers, is there any evidence
      bearing on relative incidence in officers and men? Quoting R. L.
      Richards:

      “The percentage of paresis cases among officers alone is variously
      estimated from 50 per cent in the German army (Stier) to 58.9 per
      cent in the Austrian army (Drastich). Since paresis is a disease
      of more advanced life, it is but natural that the percentage of
      paresis among officers, non-commissioned officers, and older
      soldiers should be higher than among the whole military body,
      where the average age is, as we have seen, well below thirty
      years. Hence the above figures do not mean a greater prevalence of
      syphilis among those classes, but that we have no means of knowing
      how many of the others develop paresis. If anything it shows that
      these ‘soldiers by calling,’ have a more stable mental make-up,
      since they succumb chiefly to an exogenous toxin.”

      Rayneau at the 19th Congress of French Alienists and Neurologists
      at Nantes in 1909, discussing the insane of the army from a
      medicolegal point of view, states that the most frequent mental
      disease amongst officers and soldiers is general paresis. At
      least, this disease is the most frequent basis of invaliding,
      retirement, or placing in the inactive list. He states that French
      and foreign statistics are at one upon this matter, quoting
      Christian as finding 32% among the soldiers interned at Charenton;
      Gamier at Dijon, 59%; Meilhon at Quimper, 42% and Talon at
      Marseilles, 33.8%. Grilli found 31 of 40 officers interned in
      Florence, Sienna and Milan victims of general paresis. Stier’s
      German statistics indicate about 50%. Rayneau himself found 16 of
      20 officers paretic and 17 out of 27 subalterns and _gendarmes_.

      The Neurological Society of Paris held a conference December 15,
      1916, with the chiefs of the neurological and psychiatric military
      centres of France, and discussed a variety of questions concerning
      invaliding, incapacity, and compensation in neuroses and psychoses
      of war. Dupré dealt especially with the psychoses of war as caused
      by trauma, strain, infection, and intoxication. General paresis is
      regarded by Dupré as the most important of the dementias found in
      the army. The medicolegal point of view is, of course, that
      general paresis is necessarily related to an old syphilis, but its
      late development leads to misinterpretations as to its probable
      cause, both by the family and friends and even by magistrates. The
      war acts in the French nomenclature as an _agent revélateur_ or as
      an _agent accélérateur_. Although its cause is prior and exterior
      to the war, general paresis in a majority of cases is brought out
      (_revélé_) by the lack of adaptability of the general paretic to
      the novelty and difficulties of his surroundings and duties in
      war. Trauma, strain, and alcohol in a certain number of cases
      accelerate the progress of a general paresis. The aggravation of
      paresis is produced by these same factors, but especially by
      violent cerebral trauma. According to Dupré, the Val-de-Grace
      statistics show that the number of paretics has not been increased
      by the war. Medicolegally, the victim of general paresis, like the
      victim of traumatic or infectious chronic mental disorder, may be
      assigned an incapacity of from 50 to 100%, and these patients are
      invalided under _Réforme No. 1_,—a permanent invaliding.

      Lépine of Lyons also discusses the compensation question in
      general paresis. Lépine thinks that, although syphilis is
      indispensable in paresis, yet the truth is that syphilis plus
      something else unknown to us is responsible for general paresis.
      This something else is neither a special kind of virus nor is it a
      particular kind of prepared soil alone. Trauma, physical,
      intellectual, and moral strain, and insomnia are the factors to
      which he calls special attention as adjuncts in the production of
      general paresis. As to the responsibility of the State for the
      production of general paresis, according to Lépine, the maximal
      responsibility should be 40% on account of the very considerable
      predisposition to paresis created by pre-existent syphilis.

      Marie remarked that, although there had been thousands of head
      cases at the Salpétrière, there had not been a single case of
      general paresis. Dupré agreed with Marie that trauma was not a
      frequent etiological factor; strain and alcohol were more
      important. The Society agreed that in exceptional cases, where an
      encephalic trauma could be regarded as accelerating or aggravating
      the disease, the degree of incapacity might be set at from 10 to
      30 per cent.


  =Syphilis contracted before enlistment, “AGGRAVATED ON SERVICE.”
  Canadian case, courtesy of Dr. J. L. Todd, Canadian Board of Pension
  Commissioners.=


=Case E.= A laboring man, 44, acquired syphilis at a time unknown. Ten
months after enlistment this man developed symptoms on the firing line.
He was inattentive, irrational, incoherent. The diagnosis was then
“mania.”

There were, however, scars at angle of mouth and on lower lip. Occipital
glands were palpable, fine tremor of hands. The W. R. was +++.

Later the patient became violent, destructive, untidy, disoriented.
Auditory hallucinations are recorded.

He was “boarded” for discharge five months after the first symptoms. The
board agreed that these symptoms would have appeared in civil life. In
view of a difference of opinion as to the part played by stress of
service, his condition was set down as “_aggravated on service_” (not,
it will be noted, _by_ service, see Case D).

    1. Under what conditions should pensions be awarded for disability
      resulting from venereal diseases? According to a personal
      communication from Dr. J. L. Todd, Chairman of the Board of
      Pension Commissioners for Canada, pensions are awarded for all
      disabilities appearing _during_ service, unless they can be shown
      certainly to be due to the men’s own fault and negligence. It
      would appear that _during_ service covers both aggravations _by_
      and _on_ service. There remains some doubt as to whether
      contraction of venereal disease constitutes negligence.

    2. What have been conditions in the small inactive American army of
      the past? Richards has made a study of statistics at the
      Government Hospital for the Insane, Washington.

      “The leading features of this mental disease were well exemplified
      in our cases the past year. They formed 7.5 per cent of the total
      number. They averaged forty years of age, and Ziehen says 80 per
      cent of all cases are in the fourth or fifth decade of life. They
      averaged ten and a half years’ service, which would indicate that
      the military life was their calling. Only one had any serious
      hereditary defect. Stigmata of degeneration were infrequent,
      averaging only two for each case. 66 per cent had good schooling,
      considering their opportunities. Physical signs were frequent in
      each case. Only one showed normal light reaction. Ziehen says the
      light reaction is retained in only 20 per cent of the cases.
      Patellar reflex was absent in one case and normal or exaggerated
      in five. The speech defect was slight in four cases. Other
      physical signs were present in the usual proportions. Memory
      defects existed in all the cases. In four the onset was with
      excitement. One began with a character change as the most marked
      feature. In only two were the transfer diagnoses correct. One,
      beginning as a quiet dementia, was diagnosticated paralysis
      agitans, because of a marked tremor. One was excited and euphoric
      and was called a manic-depressive psychosis. One with an obscure
      onset was diagnosticated as a neurasthenic. The other one was
      first observed in this hospital. The physical signs should have
      led to a correct diagnosis in each of these cases.”


  =Duration of neurosyphilitic process important _re_ compensation.
  Canadian case, courtesy of Dr. C. B. Farrar, Psychiatrist, Military
  Hospitals Commission.=


=Case F.= A Canadian of 36 enlisted in 1915, served in England, and was
returned to Canada in February, 1917, clearly suffering from some form
of neurosyphilis (W. R. positive in serum and fluid, globulin,
pleocytosis 108).

There is no record of any disability or symptom of nervous or mental
disease at enlistment. The first symptoms were noted by the patient in
May, 1916, six months or more after enlistment. The case was reviewed at
a Canadian Special Hospital, October 11, 1916, by a board of examiners.
This board reported that:

“The condition could only come from syphilitic infection of three years’
standing” (a decision bearing on compensation); but the general
diagnosis remained:

“Cerebrospinal lues, =aggravated by service=.”

The picture which the medical board regarded as of at least three years’
standing was as follows:

History of incontinence, shooting pains, attacks of syncope, general
weakness, facial tremor, exaggerated knee-jerks, pupils react with small
excursion. Speech and writing disorder, perception dull, lapses of
attention, memory defect, defective insight into nature of disorder,
emotional apathy.

    1. Was the conclusion “aggravated by service” sound? On humanitarian
      grounds the victim is naturally conceded the benefit of the doubt.
      But it is questionable how scientifically sound the conclusion
      really was.

    2. Could the condition come only from syphilitic infection of at
      least three years’ standing? Hardly any single symptom in this
      case need be of so long a standing; yet the combination of
      symptoms seems by very weight of numbers to justify the conclusion
      of the medical board.


  =Can PARETIC NEUROSYPHILIS (“general paresis”) be lighted up by the
  stress of military service without injury or disease? A possible
  example from P. Marie, Chatelin and Patrikios of Paris.=


=Case G.= In apparently good health a French soldier repaired to the
colors, in August, 1914, being then 23 years old.

Two years later, August, 1916, symptoms appeared: speech disorder with
stammering, change of character (had become easily excitable), stumbling
gait. He became more and more preoccupied with his own affairs, grew
worse, and was sent to hospital in October, 1916.

He was then foolish and overhappy, especially when interviewed. There
was marked rapid tremor of face and tongue. Speech hesitant, monotonous,
and stammering to the point of unintelligibility. His memory, at first
preserved, became impaired so that half of a test phrase was forgotten.
Simple addition was impossible and fantastic sums would be given instead
of right answers; handwriting tremulous, letters often missed, others
irregular, unequal, and misshapen.

Excitable from onset, the patient now became at times suddenly violent,
striking his wife without provocation. After visit at home, he would
forget to return to hospital. Often he would leave hospital without
permission (of course the more surprising in a disciplined soldier).

No delusions were found.

The serum and fluid W. R. were positive, albumin in fluid,
lymphocytosis.

=Neurological examination.= Unequal pupils, slight right-side mydriasis,
pupils stiff to light, weakly responsive in accommodation, reflexes
lively, fingers tremulous on extension of arms.

The patient had, December 5, 1916, an epileptiform attack with head
rotation, limb-contractions and clonic movements.

    1. Should this soldier recover for disability obtained in service?
      Marie was inclined to think military service in part responsible
      for the development of the paresis. Laignel-Lavastine thought so
      also, but that the amount assigned should be 5%–10% of the maximum
      assignable.

    2. What is the duty of the military authorities relative to so
      called traumatic paresis? Medicolegally speaking, Froissart,
      quoted by Rayneau, states that a victim of traumatic paresis _may
      or may not_ have presented mental disorders before the accident,
      that is, that the paretic symptoms may develop out of a clear sky
      as a result of the accident. The accident itself must be of a
      serious nature. The accident must be followed by phenomena
      pointing to brain injury of traumatic nature. These phenomena need
      not be characteristic symptoms of general paresis at the outset.
      The period elapsing between the trauma and the supervening
      condition of paresis must be occupied without notable
      interruption, at first by phenomena of a purely traumatic nature,
      later by signs indicating the onset and evolution of general
      paresis.

      The French invaliding process called _Réforme No. 1_ with pension
      is granted according to the governmental instructions only to
      officers, subalterns, and soldiers whose disease is due to trauma.
      In view of this governmental regulation, the military surgeon must
      write out certificates describing every cranial trauma, however
      slight, which might have a bearing on the development of paresis.
      However, he should not too readily admit trauma as a cause of
      paresis. If a long period of quietude, a period in which the
      trauma itself seems to have undergone a complete recovery,
      supervenes, then general paresis should not be reported by the
      surgeon.

      Lépine has recently noted the following features as desirable in
      board reports concerning paretics: nature of trauma, length of
      service, fatigue endured, insomnia, date of infection, treatment,
      W. R.


  =Can “gassing” light up a paresis? Example from de Massary of
  Issy-les-Moulineaux.=


=Case H.= A soldier, 35, was sent to the _Centre Neurologique_ with a
hospital ticket reading:

“Neurasthenia, general weakness following intoxication by gas.”

The soldier was thought at first to be a neurasthenic. But he soon
showed signs of more pronounced mental trouble. The voice was
suspicious. There was a slight irregularity of pupils.

An epileptiform attack occurred, followed by aggravation of symptoms.

Lumbar puncture showed pleocytosis. The W. R. of the serum proved
positive.

Yet the evident =neurosyphilis=, possibly =paretic= (de Massary’s
diagnosis), was preceded by a neurasthenia and the neurasthenia was
preceded by “gassing.”

De Massary believes the patient _and his family_ would perhaps be
justified in believing the condition produced by the injury. De Massary
is not clear as to the financial deserts of the patient. It is not a
manifest case of aggravation of antebellum symptoms, even if it be
neuropathologically an instance of acquired loss of resistance to
pre-existent spirochetes in body or brain.

    1. What adjuvant factors have been recognized in military paresis?
      Aside from syphilis, Rayneau finds that alcoholism, malaria,
      sunstroke and various intoxications serve as causes for paresis.
      Rayneau points out that the apparent integrity of the mind in
      general paresis may be such that they last in the army some time
      and have their oddities ascribed to misconduct or breaches of
      discipline. In fact the Legrande du Saulle called this early
      period in general paresis the _medicolegal period_, showing, as it
      so often does, thefts, outrages against decency, frauds, assaults,
      exhibitionism and the like. To be sure these acts are absurd and
      infantile and not difficult to recognize as of psychotic origin.


  =Syphilis may bring out epilepsy in a subject having taint. Case
  from Bonhoeffer, 1915.=


=Case I.=[28] A man of 35 in the _Landwehr_ acquired syphilis some time
in the summer of 1914. He was a good soldier, passed through several
clashes, and was promoted to _Unteroffizier_.

To understand what followed it must be stated that he had been a
bed-wetter to 11, had been practically a teetotaler (Bonhoeffer’s point
is perhaps that otherwise epilepsy might have developed sooner?), and,
when he did drink, vomited almost at once, and had amnesia for the
period of drunkenness. His father had been somewhat of a drinker. His
sister had suffered from convulsions as a child.

February, 1915, the _Unteroffizier_ lost appetite, got headaches, and
went to hospital for a time. Upon getting better, he was sent on service
to Berlin. In a Berlin hotel he had his first convulsions and
unconsciousness, biting his tongue. He was confused for several days,
and, when he had become clear, had a pronounced retrograde amnesia
together with a tendency to fabricate a filling for the lost period.

This retrograde amnesia is uncommon in epilepsy and suggests organic
disease. No sign of organic disease was found on neurological
examination. The patient had no signs of the epileptic make-up. The
serum W. R. was negative. On the whole, Bonhoeffer regards the epilepsy
as “reactive” to the syphilis, as a syphilogenic epilepsy.

As to the amnesia, it is of interest that alcohol should long before
have been able to cause amnesia in this man in the same way as does now
the syphilitic epilepsy.

    1. In view of the fact that this _Landwehr_ man appears to have
      acquired syphilis while on campaign, what is the responsibility of
      the government for treatment? The Canadian authorities, as stated
      under Case E, are in doubt whether contraction of venereal disease
      constitutes negligence on the part of the soldier. It would appear
      to us that where a government does not take suitable steps to
      prevent the acquisition of syphilis by the soldiers, the
      government must assume a measure of responsibility for the
      syphilis incurred. The government’s responsibility would be still
      greater in equity, it would appear, if commercial opportunities
      for the acquisition of syphilis are maintained under more or less
      close government supervision or (even as has been claimed for
      certain encampments on our own Mexican border) if shelter for
      illicit sex relations is afforded within the limits of a military
      camp. In a certain community, “E,” for example, it is claimed by
      Exner,[29] the district for prostitutes was “situated within the
      lines of military camps and protected and ‘regulated’ by the
      military authorities.”

      But even if the government has no legal responsibility in this
      regard, it would be well to consider the ultimate results of the
      syphilis that will probably be acquired by great numbers of
      soldiers under campaign conditions. Aside from the ravages of
      syphilis outside the nervous system, it is well known, as Weygandt
      intimates for German conditions, that the aftermath of war will be
      a high proportion of cases of neurosyphilis.

      Weygandt remarks in his review of the influence of the war upon
      psychiatry, that the opportunity for syphilitic infection in the
      campaign is considerable. In the war of 1870, the conditions in
      this regard were extremely unfavorable, and writing in 1915,
      Weygandt remarks that at present there should be a prophylaxis
      against syphilitic infection by the soldiers, which prophylaxis
      should be the most energetic possible. Continence on the part of
      the soldiers and the isolation of infected women, with examination
      by specialists, have been advocated by Neisser and by Mendel. In
      the ’80’s a great number of cases of locomotor ataxia developed in
      Germany, which were due to syphilis acquired by the soldiers and
      officers in the war of 1870.


  =Syphilis in a psychopathic subject. Convulsions 5 days after
  Dixmude. Case from Bonhoeffer, 1915.=


=Case J.=[30] A soldier in the reserves, 23, was, subsequently to his
being brought to hospital, described by his wife as a rather
over-sensitive fellow, who could hardly look at blood and was meticulous
about the household. He had always been subject to headaches, especially
after hard work. However, he had passed through his military training
well in 1910, not even having been _bestraft_.

He began service in October and fought at Dixmude on the 19th. On the
24th in the trench and while being carried back, he had several spells
of pallor, falling stiff, and then having convulsions. Brought finally
to the Charité in Berlin, he had more spells of sudden pallor, collapse
with brief convulsions, tossings in bed, and absences, post-convulsive
headaches, and mild bad humor.

There were numerous attacks several days apart in the first seven weeks.
The patient was not of an “epileptic” disposition, though he was rather
readily dissatisfied. Headaches also occurred without relation to
convulsions.

The serum W. R. was positive. Treatment by mercurial inunctions. No
further convulsions. Prognosis as to the possibility of a constitutional
epilepsy unknown.


  =SYPHILITIC ROOT-SCIATICA (lumbosacral radiculitis) in a fireworks
  man with a French artillery regiment. Case presented from Dejerine’s
  clinic by Long.=


=Case K.= No direct relation of this example of root-sciatica to the war
is claimed nor was there a question of financial reparation.

There was no prior injury. At the end of March, 1915, the workman was
taken with acute pains in lumbar region and thighs, and with urgent but
retarded micturition.

Unfit for work, he remained, however, five months with the regiment, and
was then retired for two months to a hospital behind the lines. He
reached the Salpétrière October 12, 1915, with “double sciatica,
intractable.”

There was no demonstrable paralysis but the legs seemed to have “melted
away,” _fondu_, as the patient said. Pains were spontaneously felt in
the lumbar plexus and sciatic nerve regions, not passing, however,
beyond the thighs. These pains were more intense with movements of legs;
but coughing did not intensify the pains. Neuralgic points could be
demonstrated by the finger in lumbar and gluteal regions and above and
below the iliac crests (corresponding with rami of first lumbar nerves).
The inguinal region was involved and the painful zone reached the
sciatic notch and the upper part of the posterior surface of the thigh.

The sensory disorder had another distribution objectively tested. The
sacral and perineal regions were free. Anesthesia of inner surfaces of
thighs, hypesthesia of the anterior surfaces of thighs and lower legs.
The anesthesia grew more and more marked lower down and was maximal in
the feet, which were practically insensible to all tests, including
those for bone sensation. There was a longitudinal strip of skin of
lower leg which retained sensation.

Position sense of toes, except great toes, was poor. There was a slight
ataxia attributable to the sensory disorder—reflexes of upper
extremities, abdominal, and cremasteric preserved, knee-jerks, Achilles
and plantar reactions absent.

The vesical sphincter shortly regained its function, though its disorder
had been an initial symptom.

Pupils normal.

The “sciatica” here affects the lumbosacral plexus. Signs of disorder at
one time or other affected the first lumbar distribution of the third
lumbar and first and second sacral nerves.

As to the syphilitic nature of this affection, there had been at
eighteen (22 years before) a colorless small induration of the penis,
lasting about three weeks. There was now evident a small oval pigmented
scar. The patient had married at 20 and has had three healthy children.

The lumbar puncture fluid yielded pleocytosis (120 per cmm.). Mercurial
treatment was instituted.

The treatment has not reduced the pains. Long thinks it was undertaken
too long (six months) after onset. The warning for early diagnosis is
manifest. There was somehow a delay under the medical conditions of the
army.


  =Can the “lighting up” of NEUROSYPHILIS IN CIVIL LIFE be induced by
  the domestic stress of war? A possible example from Dr. R. Percy
  Smith, London.=


=Case L.= A German Jew in London passed into the PARETIC form of
NEUROSYPHILIS shortly after the outbreak of war under conditions
suggesting that the stress of emotions directly or indirectly lighted up
the neural process.

The man was a bank-officer, 52 years old, and married. He had lived many
years in England and was in fact a naturalized citizen. He had been
under treatment for syphilis by Sir Jonathan Hutchinson, 29 years
before, namely, at the age of 23. Subsequently, Sir John had given him
permission to marry.

It proved that for years the man had had fixed pupils, absent
knee-jerks, and a perforated ulcer of the foot. However, there had been
no other mental or nervous symptoms preventing bank-officer’s work.

At the outbreak of war the man was discharged from the bank. He grew
worried and sleepless. He began to charge himself with sex irregularity.
He went down to the city and burned trust documents belonging to others.

From worry and self-accusation he passed into depression and agitation.
He developed a belief that not only he but also his German wife were to
be executed. He thought he was a criminal and was to be hanged.

The depression then altered to a condition of hilarity and loquacity.

In addition to the fixed pupils and absent knee-jerks, a speech disorder
shortly developed.

The patient was placed under care, but quickly (a few months?) passed
into an advanced stage of paretic neurosyphilis and died.


  =SHELL-SHOCK PSEUDOPARESIS (non-syphilitic). Recovery. Case from
  Pitres and Marchand of Bordeaux.=


=Case M.= June 19, 1915, a shell exploded some distance from Lieutenant
R. He remembers the gaseous smell, the bursting of several shells nearby
and a sensation of being lifted into the air. When he recovered
consciousness, he was in hospital at Paris-Plage, covered with bruises
and scratches. They told him he had been delirious and had vomited and
spat blood.

June 24, his wife came to see him, but this visit he could not remember.
Nor could his wife at first recognize him, he was so thin. He roused a
few moments and recognized his wife, but relapsed into torpor again.
Speech was difficult and ideas confused.

A few days later he was able to rise; but his mental status grew worse,
especially as to speech and writing; the latter quite illegible. There
was insomnia, or, if he slept, war dreams.

August 7, he began a period of five months’ convalescence passed with
his family, depressed, given to spells of weeping, confined to bed or
couch, unable to “find words,” conscious of his state and troubled about
it, speaking of nothing but the war, and afraid to go out for fear of
ambuscade. There was at first a slight lameness of the right leg.
Although he could walk, he felt pain in the knee on flexing the right
leg on the thigh. He walked holding this leg in extension.

On going back to the colors, he was immediately evacuated to the _Centre
Neurologique_ at Bordeaux, January 20, 1916.

Examination found a bored, impatient, irritated man, vexed that a man
who was not sick should be sent up “_comme fou_.”

Omitting negative details, =neurological examination= showed slight
lameness as above, body stiff and movements jerky; difficult, unsteady
gait. The lieutenant could stand for some time on either leg, tongue and
face tremulous during speech. Limbs moderately tremulous, especially in
the performance of test movements.

Knee-jerks and Achilles jerks absent. Other reflexes, including
pupillary, normal. Segmentary hypalgesia of right leg, especially about
knee. Tremulous speech and writing. Patient would stop short in speaking
for lack of words.

Malnutrition. Appetite good, but a bursting feeling after meals.

Skin dry, scaly on legs, fissured on fingers.

Serum W. R. negative. Fluid not examined.

=Mental examination.= Conscious and complaining of his troubles,
Lieutenant R. claimed persistently that he was not sick. Memory for
recent events was in general poor. Errands easily forgotten. Lost in the
street. Complaint of corpse odors round him. Everybody is looking at him
and making fun of him. He was apt to insult bystanders. He was afraid of
German spies. Things in shops angered him as they seemed to him to be of
German manufacture.

There were frequent periods of depression, with pallor and no
spontaneous speech for some hours to a half-day. Headaches coming on and
stopping suddenly.

As to diagnosis, the first impression, say Pitres and Marchand, was that
of general paresis. The progress of symptoms after the shock was
consistent with this diagnosis. The mental state and the physical
findings seemed consistent, although the pupils were normal. His partial
insight into his symptoms was not inconsistent with the diagnosis. He
had a characteristic self-confidence. There had been four stillbirths
(two twins) two children are alive, 11 and 13. Typhoid fever at 30.
Syphilis denied. No mental disease in the family.

The patient had never done military duty, having been invalided for
“right apex.” But he had volunteered and been accepted in September,
1914.

    1. Was this diagnosis, general paresis, at any time justified? The
      spinal fluid should of course have been examined. The peculiar
      lameness of the right leg was certainly not characteristic of
      general paresis, and was perhaps hysterical. (There was no
      limitation of visual fields or any other definite sign of
      hysteria.) Presumably some quality of speech defect, the amnesia,
      and the euphoria, together with absent knee-jerks, led to the
      diagnosis general paresis. By the 20th of March, 1916, the
      knee-jerks had become lively; the Achilles jerks normal. At this
      time the patient had gained in weight, could walk though stiffly,
      had headache (especially right frontal) and a feeling of lead in
      head, less tremor, lack of desire to undertake anything. He still
      wanted to go back into service. He still saw spies about. Dreams
      terrible; devoured by spiders, leggins instruments of torture.
      Skin still atrophic. June 4 there was no more tremor of speech or
      face. Symptoms largely disappeared except a few ideas of
      persecution. Recovery October, 1916.

    2. How was Lieutenant R. cured? Apparently by rest in the _Centre
      Neurologique_. Pitres and Marchand do not speak of the subtle
      relation between mental state and the idea of non-return to
      military service. This motive might still work even if Lieutenant
      R. kept protesting quite sincerely that he wanted to go back into
      military service.


  =SHELL-SHOCK PSEUDOTABES (non-syphilitic, serum W. R. positive).
  Improvement. Case from Pitres and Marchand of Bordeaux.=


=Case N.= Innkeeper B., 36, a shell-shock and burial victim June 20,
1915, was looked on by a number of physicians as a case of genuine
tabes.

Even eight months after the episode, he still showed (when observed by
Pitres and Marchand, February 3, 1916) absence of knee-jerks and
Achilles jerks, a slight swaying in the Romberg position, pupils
sluggish to light, incoordination, delayed sensations. There was also a
history of pains in the legs, compared by the patient to those of
sciatica. These pains came in crises, the longest of which had lasted 30
hours.

It seems that this soldier’s troubles began the day after his shock with
a feeling of swollen feet and of cotton wool under them. He stayed on
service, however, walking with increasing difficulty.

At the time of his evacuation, July 10, he could walk with great
difficulty. “Strips of lead were between his legs.” He could hardly
control movements in the dark, or descend stairs. Often his legs would
bend under him. Vesical function sluggish.

After a few months the patient could walk better. On February, 1916, he
walked thrusting his legs forward trembling, and dragging toes a little.
He could not support himself on either leg. Jerkiness and incoordination
in extension or flexion of leg on thigh.

The muscular weakness was decidedly against tabes or at all events a
pure tabes. The incoordination proved to be due, not to loss of position
sense (which was intact) but to unsteady muscular contractions. Deep
sensibility was intact.

There were no mental symptoms. There was a slight hesitation in speech
and doubling of syllables, but nothing demonstrable with test phrases.

The serum W. R. was positive. Syphilis denied.

    1. What is the cause of these phenomena? Pitres and Marchand lean to
      the hypothesis of slight internal traumatism. They believe that
      there is either (a) slight internal hemorrhage in the nervous
      system, or possibly (b) what they call “nerve cell contusion,” or
      perhaps (c) caisson-disease-like phenomena from aerial
      decompression. Some authors incriminate (d) the gases. It has been
      reported by certain French authors that shortly after shell-shock
      injury or burial there is a pleocytosis in the spinal fluid as
      well as evidence of hemorrhage. The pleocytosis is said to last
      only a short time; hence when patient arrives at a base hospital
      lumbar puncture usually discloses nothing.


            Baalim and Ashtaroth

                            Paradise Lost, Book I, line 422.




                          VII. SUMMARY AND KEY


No more important human problem now exists than syphilis. Syphilis of
the nervous system or, briefly, neurosyphilis is a highly important
fraction of the total problem. The few outstanding dates and items which
we present on the following page give but a faint idea of the amount of
observation and thinking which the medical aspects of neurosyphilis
alone have required. The present work deals with but a small fraction of
the results of this work, nor can we more than glance at the scientific
history of syphilis and neurosyphilis—a history that would form an epoch
in itself.

It is only in the most recent years that syphilology and the narrower
science of neurosyphilology have threatened to become separate
disciplines boasting full time specialized workers. Up to recent years
the contributions to the theory of syphilis have been largely
by-products of work in larger sciences and arts. Thus, the cellular
pathology of syphilis as worked out by Virchow and the more special
vascular features as worked out by Heubner were incidental in the
progress of pathological anatomy and histology. The bold procedure of
Quincke in proposing lumbar puncture also had its more general ground in
the extension of clinical medicine,—an interpretation likewise true of
the French achievements in the cyto-diagnosis and chemical diagnosis of
the lumbar puncture fluids. The careful histological definitions of the
Nissl-Alzheimer group were incidental to the application of approved and
classical pathological methods to neurological and psychiatric material.

Again, the work of Schaudinn, as well as that of Metchnikoff and Roux,
was ingenious work with the methods of parasitology and experimental
pathology. The great work of Schaudinn in establishing the constancy of
the spirocheta pallida in syphilis may be said to have started
syphilology as something approaching a special discipline. The ideas of
one of the greatest of immunologists, Bordet, were almost immediately
applied to the serum diagnosis of syphilis by Wassermann and the further
application of this method to the problems of neurosyphilis was almost
immediate, with the spirocheta pallida as an object of attack. The
commanding intelligence of Ehrlich could at once seek application of
long incubated ideas of chemotherapy with the startling outcome,
salvarsan.


                        =DATES, NEUROSYPHILIS=

 VIRCHOW                         PATHOLOGY                        1858
 HEUBNER                         ENDARTERITIS                     1874
 QUINCKE                         LUMBAR PUNCTURE                  1891
 RAVAUT, SICARD, NAGEOTTI, WIDAL CYTODIAGNOSIS, C.S.F.            1901
 WIDAL, SICARD, RAVAUT           ALBUMIN, C.S.F.                  1903
 METCHNIKOFF AND ROUX            TRANSMISSION TO APES             1903
 ALZHEIMER                       HISTOPATHOLOGY, BRAIN SYPHILIS   1904
 SCHAUDINN AND HOFFMANN          SPIROCHETA PALLIDA               1905
 WASSERMANN, NEISSER AND BRUCK   SERUM DIAGNOSIS                  1906
 PLAUT                           WASSERMANN REACTION, C.S.F.      1908
 EHRLICH                         SALVARSAN                        1909
 SWIFT AND ELLIS                 SALVARSANIZED SERUM              1912
 NOGUCHI AND MOORE               SPIROCHETES, BRAIN TISSUE,       1913
                                   PARESIS
 LANGE                           GOLD SOL TEST                    1913

                                                              CHART 28


The history of syphilis and neurosyphilis was now to be thickly sown
with ideas and results growing from the achievements of Schaudinn and
Ehrlich. The positive reactions in the blood and spinal fluid in the
most striking of mental diseases, general paresis, led to the impression
that general paresis itself might at last be proved to be what Mœbius
had suspected, namely, 100% syphilitic. We know how difficult is the
technical proof of spirochetosis in the brains of general paretics both
post mortem and ante mortem, but no one doubts the certainty of the
syphilitic hypothesis concerning the origin of general paresis.

The data of the gold sol reaction ultimately obtained from the ideas of
Thomas Graham concerning colloids, as developed by Szigmondi and
effectively applied by Lange, have broadened and solidified the whole
plane of attack.

The ingenious suggestions of Swift and Ellis (salvarsanized serum) and
the notable work of Noguchi and Moore (spirochetosis in paretic brains)
indicate to us as Americans what the establishment of scientific
institutes may do to permit the rapid application of new ideas to
branches of inquiry that are opened out. Scientific institutes do not
manufacture a Virchow, a Metchnikoff, a Schaudinn, a Bordet or an
Ehrlich but they directly permit such men to work and indirectly
stimulate the development of more.

The series of 137 cases here at least presented does not touch
systematically the problems of the neuropathology of syphilis, which
would themselves require a textbook of respectable size. We have,
however, presented in Part I, cases 1 to 8, some indication of the
protean nature of the material and from time to time in the remainder of
the book somewhat fuller accounts of the pathological anatomy and
histology have been presented than are strictly necessary in the
demonstration of the principles of modern systematic diagnosis and
treatment.

Our work may be said to represent psychopathic hospital practice as
available to us in our official capacities at the Psychopathic
Department of the Boston State Hospital. A word is necessary concerning
the nature of this practice. The dispensary and ward practice of a
modern state psychopathic hospital, such as the Boston institution
(founded in 1912) and the Ann Arbor institution (founded in 1906), is to
be sharply distinguished from asylum practice. Those who have not
followed the evolution of the modern psychopathic hospital with the
lowering of bars to the admission of patients and the extension of its
benefits to a group of sick persons far removed from the medicolegal
concept “insanity” may not soon grasp the general nature of psychopathic
hospital material. Psychopathic hospital practice stands, in fact,
almost midway between asylum practice in the classical sense and private
practice. This has come about through the great extension of the
so-called voluntary relation under which hundreds of patients now resort
to the beds and out-patient rooms of a psychopathic hospital, who would
formerly have remained untreated or inadequately treated. Moreover, the
broadening of the concept of mental diseases as a whole has permitted in
some parts of the world the establishment of laws under which
psychopathic and psychotic patients may be brought to psychopathic
hospitals and even to asylums under the easiest possible conditions and
restrictions, omitting court procedure altogether. The operation of the
voluntary and temporary care provisions of law has accordingly yielded
us, in the Boston institution, a great group of cases formerly not at
all accessible to hospital diagnosis and treatment. Needless to say, as
always under such conditions, we have been able to show not merely that
hospital diagnosis or treatment is of importance to a new group of
cases, but also that home treatment, especially home treatment under
supervision, is possible and even ideal for a large group of cases about
which utter darkness or profound misgivings ruled in the not very
distant past.

Accordingly, we are fain to insist that our material is of importance in
new programs of community organization for the stamping out of disease.
The work in psychopathic hospitals upon neurosyphilis in particular is
essentially a part of the public health program, although our special
work will not soon be taken over by the public health officers, so
complicated are the ramifications of medical and social diagnosis and
treatment in the neurosyphilis group.

We have tried in Part IV (medicolegal and social cases) to give a few
examples to illustrate the part played by neurosyphilis in society; but
we regard this part of our work as the least satisfactory and the least
representative in the total work. Our colleagues in social service, in
mental hygiene, in psychopathology and in criminology will easily in the
next few years provide a far more adequate basis for a full account of
the public and social aspects of neurosyphilis. One point we should
emphasize here. The psychopathic hospital worker, whether physician or
social worker, must shortly decide upon and consolidate a program with
relation to the families of neurosyphilitics.

The syphilographers of the dermatological and special syphilis clinics
have their identical problems with the families of syphilitics; but the
dispensaries for mental cases and in particular the psychopathic
hospital and asylum out-patient departments tap another reservoir of
syphilitic families at a stage when the memory of the initial horrors of
syphilitic infection is dimmed or erased. Any program for the diagnosis
and treatment of syphilis of the innocent must take into account not
only the skin, syphilis, and internal medicine clinics but also the
clinics for mental and nervous diseases wherein neurosyphilitics are not
infrequent. Whether the ultimate percentage will stand at 10, 15 or 20%
for the neurosyphilitics in mental clinics, is of no importance to the
principle. There are enough neurosyphilitics having economical
importance and humanly precious families to warrant definite steps.

The Massachusetts Commission for Mental Diseases has in the last few
years employed the services of two medical workers whose time has been
largely devoted to the applications of our recent knowledge in
neurosyphilis and has gone so far as to establish a neurosyphilis ward
in one of the district state institutions (Summer Street, Worcester,
under the Grafton Hospital Board). Special social workers in the field
of neurosyphilis have also been available from time to time. These
social workers are enabled with the support of the medical profession to
do a great deal of good, for example, with the slogan THE CHILD OF A
PARETIC IS THE CHILD OF A SYPHILITIC.

The nature of the intake of patients into psychopathic hospital wards
and out-patient clinics is such that great numbers of non-mental
syphilitics arrive for diagnosis and possible treatment. Moreover, the
existence of syphilis in non-suspects is a fact picked up by the way in
routine Wassermann serum diagnosis.

The mental clinic in the modern sense with the medicolegal bars lowered
or well nigh removed, turns rapidly into a clinic for neurological cases
as well. The German models for mental and nerve clinics are rapidly
being imitated. The result of this administrative novelty in our
hospital procedure has incidentally yielded us many representative cases
of entirely non-psychotic and even non-psychopathic neurosyphilis. Our
impression grows and deepens that _the neurosyphilitic is seldom merely
a spinal syphilitic_. The neurosyphilitic is nearly always the victim
not merely of spinal disease but also of intracranial disease. Per
contra, the victim of intracranial neurosyphilis is almost always more
or less importantly affected by spinal neurosyphilis.

The net result of the modern work on neurosyphilis has been to bring the
neurologist and the psychiatrist together upon one platform in diagnosis
and more and more upon one platform in treatment. But aside from the
clinical evidence that the neurosyphilitic is apt to be a victim of both
brain syphilis and cord syphilis, the autopsy evidence is stronger
still. Even the victim of tabetic neurosyphilis (“tabes dorsalis”)
himself is rarely found at autopsy without more or less evidence of
significant encephalic disease of a chronic inflammatory or degenerative
nature. Aside from tabes dorsalis and Erb’s paraplegia, the rule is
almost universal that neurosyphilis is a matter of the entire nervous
system.

In view of the generalization of neurosyphilitic process, one might
question the advantage of any topical grouping of neurosyphilitic
disease. Practically speaking, however, as we have shown in Chart 5, it
seems advisable to separate the neurosyphilitic diseases into six
roughly distinguishable groups. First, there is the great group that we
have chosen to term =diffuse neurosyphilis=, including many of the cases
of so-called cerebral or cerebrospinal syphilis of the neurological
clinics and the group of cases that have been treated in private
practice by internists and neurologists without recourse to
institutions. These cases have lived at home and have not been socially
hard to manage until the late phases of their disease when the victims,
if poor, are sent to almshouses and infirmaries under municipal or state
care. These are the cases which have been in the past regarded as most
amenable to the classical iodid and mercurial treatment. Indeed there is
record of numerous therapeutic successes in the group.

Whereas the lesions in diffuse neurosyphilis are chiefly chronic
inflammatory and degenerative changes of a diffuse nature (with vascular
changes incidental or subordinate to the inflammation and the
degeneration), there is an important and large group of cases that we
have termed =vascular neurosyphilis= in which the factors of
inflammation and degeneration are subordinate to vascular insults. These
are cases of syphilitic arteriosclerosis and the best examples are
victims of cerebral thrombosis. The clinical symptoms of the immediate
attacks (of apoplectiform, epileptiform or other acute nature) are not
in themselves distinguishable from the immediate effects of
non-syphilitic vascular disease; nevertheless the establishment of their
syphilitic etiology is of the utmost importance on account of the
possibilities of treatment of the underlying syphilis. For, as the
neuropathologist must always insist, the immediate effects of vascular
insults whether syphilitic or non-syphilitic are much more extensive
than the ultimate paralytic or residual irritative effects; and by
consequence a greater optimism is justifiable in the confronting of
these cases than the nihilistic observer is likely to entertain.

Physicians dealing with chronic disease in general are apt to be
somewhat nihilistic, but this nihilism is increased a hundred fold in
disease of the nervous system. How important then is any work which
shall demonstrate partial or even complete recovery from serious looking
apoplectic and other seizures, besides all of which the point of
syphilitic treatment naturally lies in the prevention of future insults
of the same sort. Therapeutic experience in this vascular group has
almost as good a toll of successes as in the diffuse neurosyphilis group
above mentioned, that is to say, the modern systematic treatment and
even the old pre-salvarsan treatments have succeeded fairly well in
removing the products of inflammation from the membranes of the nervous
system and in abolishing vascular disease.

The old principle that the dead neurone in the central nervous system
cannot be regenerated remains a perfectly firm principle; but there are
any number of neurones and even neurone systems that are not essential
to life or to the pursuit of happiness. We accordingly have just as good
a theoretical therapeutic outlook in many instances of chronic
neurosyphilis as we have in chronic diseases of many other organs. Add
to this the fact that a great number of the most sharply-defined and
grave symptoms are probably not due to destruction of neurones but to
irritation and functional disability of neurones, and the conclusion is
compelled that, as hinted above, an entirely unjustifiable pessimism and
nihilism have prevailed in some quarters. Of course, the recoil from
such pessimism with the onset of salvarsan treatment led various
enthusiasts to an undue optimism.

Another great group distinguished by the existence of spinal cord
disease is the group we have termed =tabetic neurosyphilis=, which group
contains the classical tabes dorsalis or locomotor ataxia and its
congeners.

The question of therapeutic optimism comes up most forcibly in the field
of tabes. It is hard, however, at this time to give a proper and
scientifically founded estimate of the therapeutic outcome in tabetic
neurosyphilis with modern methods. So much can be said: namely, that the
alleviation of pain and the palliation of other symptoms can be
successfully claimed as a result of the renewed interest in the
treatment of this affection. What was said above concerning the finality
of the death process in a dead neurone is very strikingly true, of
course, of some of the neurones of the posterior columns in tabes
dorsalis. Still only portions of these neurones (namely, those which run
an intradural course) are strikingly altered in a great many cases. Now
and again one is greatly astonished to observe the restoration of the
lost knee-jerk in cases of neurosyphilis (see for instance the case of
Alice Morton (1), with discussion). In short, the relation of several
tabetic symptoms to irritative conditions and functional disability of
neurones may be considered established. Naturally, moreover, if therapy
can stop the upward course of the affection as it passes from lower to
higher nerve roots (according to reasonably well-established ideas of
the genesis and progress of this affection), we are entitled to a
further degree of optimism.

The question of therapeutic optimism _versus_ pessimism is forced upon
attention in the fourth great group of neurosyphilitic diseases which we
have chosen to distinguish, namely, the group of =paretic neurosyphilis=
including the disease formerly known as general paresis, paralytic
dementia, softening of the brain and the like.

Of course, no one can gainsay there is a group of cases having in the
natural course of events a prognosis of fatality within a term of years,
say three to five years, and we have cases in our series which go to
show that even with the modern intensive treatment the characteristic
down-grade symptomatic progress and ultimate fatality occur. Still, we
have other cases diagnostically on all fours with the fatal cases that
have seemed to get either entirely well with the laboratory tests
returning to normal and without further mental symptoms, or else lose
mental symptoms on the one hand or laboratory signs on the other. We
should strongly object to any account of paretic neurosyphilis which
should insist that its necessary outcome is fatality within a term of
years. Of course, viewing our knowledge of the affection in the past, we
should be compelled to object to the generalization “_paresis fatal_” on
the evidences of the universally recognized remissions. If nature can
stop a paretic process, why cannot man do as much? Can it be alleged
that our own apparent therapeutic successes and those of others are
merely curious examples of coincidences, namely, that remissions have
chosen to occur precisely when therapy was systematically applied? The
percentage of therapeutic successes with modern intensive treatment,
wherever it may ultimately stand, is already too high for this
hypothesis of fortuitous remissions.[31]

Moreover, we believe that the details of the clinical progress of some
of the reported cases are convincing on this point. What, however, is
the distinguishing feature of paretic neurosyphilis? It is in one sense
a particular kind of diffuse neurosyphilis. The tissues are apt to show
not only encephalic but also spinal changes. There is apt to be a more
or less well-defined meningitis, but the characteristic feature, without
which the diagnosis of paretic neurosyphilis would hardly be rendered,
is the existence of disease of the cerebral cortex. This disease is
parenchymatous in the sense of showing nerve cell destruction. There is
also an interstitial reaction in the shape of a neuroglia overgrowth,
but the striking and pathognomonic feature is the infiltration of the
sheaths of the small vessels in the cortex, giving evidence of an
inflammation very intimately affecting the cellular mechanisms of the
nervous system. It is striking how often a smaller or larger share of
the cells found in the vessel sheaths are plasma cells. It does not
appear, however, that the diagnosis of paretic neurosyphilis as against
diffuse non-paretic neurosyphilis can be made in the stained sections
with complete safety on the basis of plasmocytosis in the former and
lymphocytosis in the latter. Whatever the results of careful
histological differentiation by future neuropathologists may yield, it
is at all events true that we cannot yet make an important
differentiation clinically on the basis of the differential count of
plasma cells and lymphocytes in the puncture fluids. However this may
be, there is an important distinction between diffuse neurosyphilis of
the non-paretic type and paretic neurosyphilis in that paretic
neurosyphilis rarely if ever fails to show important degrees of
intracortical perivascular inflammation with larger or smaller numbers
of plasma cells.

What has the therapeutist to face in this matter? The answer, as
elsewhere, depends somewhat upon what the future may decide as to the
habitat and toxic or antitoxic activities of the spirocheta pallida. The
early claims that the spirocheta pallida was extravascular and lay for
the most part in the parenchyma and not in the vessel sheaths were
perhaps overbold, since other workers have found the spirochete in the
vessel sheaths also (Mott).

Aside from the spirochete and its accessibility to spirochetocidal
drugs, there seems to be no reason for supposing that the perivascular
sheaths cannot be cleansed of their inflammatory contents. There is,
again, no reason why the phagocytic cells should not continue to perform
their scavenger function until such time as the degenerative process in
the parenchyma (a process not necessarily progressive in the absence of
the spirochete or its products) ceases. There is every reason to suppose
that a great many of the clinical phenomena are not necessarily due to
permanent destruction of neurones and neuronic organs (dendrites,
axis-cylinders, nets and the like) but are due to various microphysical
conditions of pressure, intoxication and the like.

The inflammatory conditions in the spinal cord of poliomyelitis, which
conditions are precisely as striking as those of the paretic cortex, are
beyond a question cleared away in the progress of the affection.
Reference to the paradigm case (1) will show the type of our argument.
There is no manner of doubt that in this paradigm case almost every
portion of the nervous system had been sometime swept by spirochetosis
and many of its small vessel sheaths stuffed with chronic inflammatory
products. As for paretic neurosyphilis itself, a great many of its most
striking clinical phenomena, such as loss of memory and disorientation,
as well as great degrees of apparent dementia, are found virtually as
often in cases with very slight anatomical changes as in cases with
marked cortical devastation. The inference is plain, that these
phenomena are to a degree functional rather than structural.

In brief, we conclude not only from therapeutic experience but also on
_a priori_ grounds that the histological conditions in paretic
neurosyphilis are not entirely hopeless, and certainly not more hopeless
than conditions in many chronic diseases outside the nervous system.
Accordingly, we plead for a temperate optimism as to therapeutic results
in general paresis.

A fifth group of neurosyphilitic cases bulking rather largely in
textbooks of pathology is the group of the =gummata=. For a variety of
reasons (therapeutic and otherwise) the actual number of gummata of the
nervous system available for clinical or even for anatomical study is
much smaller than the books might lead one to infer.

The sixth and last of the main groups of neurosyphilitic diseases is
that of the =juvenile forms=, among which we find not only diffuse forms
without a special and well-defined course, but also characteristic
examples of paretic and tabetic neurosyphilis. The distinction of a
juvenile or congenital group of neurosyphilitics is, on theoretical
grounds, perhaps hardly defensible. On practical grounds, however, the
juvenile neurosyphilitics do form a group having special relations to
feeblemindedness, epilepsy and the like.

We must be clearly understood as to the rough, six-unit classification
just given. It is practical merely. For comparison we have given in
other charts more expanded lists of the diagnostic entities in
neurosyphilis among which that of Head and Fearnsides is of special
interest, see Chart 2, page 21.


We shall now proceed to a brief analysis of the findings in our chosen
series of 137 cases. We shall not reproduce the case headings of these
cases, but expand their statements where necessary and tie them together
so far as possible into a reasonable and systematic statement of the
situation in neurosyphilis. The footnotes will contain references to
other cases in which identical points are illustrated as in the leading
cases. The leading cases will in all instances be placed first in the
footnotes.

The paradigm[32] shows meningeal, vascular and parenchymatous lesions
and thus illustrates our definition of the term DIFFUSE which means
precisely meningeal, vascular and parenchymatous. The meningeal lesions
gave rise to two prominent sets of lesions, first, the marked tabetic
lesions of the spinal cord (due to the spinal root neuritis incidental
to the spinal meningeal inflammation), secondly, the characteristic
asymmetrical and focal atrophy of cranial nerves incidental to a now
largely extinct meningeal process at the base of the brain. The vascular
lesions are responsible for another important and characteristic factor
in the case, namely, the bilateral pyramidal tract sclerosis; the
bilateral cysts of softening of the corpora striata are characteristic
effects of old syphilitic cerebral thromboses. The parenchymatous
disease in our paradigm is everywhere obvious, less so perhaps in the
cortex itself than elsewhere, although here also evident in the shape of
lesions suggesting an early phase of tissue atrophy.

The paradigm is of interest in demonstrating what in broad lines must be
taken as an ascending disease proceeding not only from spinal cord to
encephalon but also traceable as proceeding from lower parts of the
spinal cord to upper parts thereof and from the lower encephalon to the
higher structures of the cerebral cortex itself.

The paradigm insistently calls attention to the advantage of persistent
therapy not only in its display of remarkable successive recoveries from
permanent looking symptoms but also histologically from the remnants of
inflammatory process to be found in an otherwise almost wholly
dismantled nervous system with extinct lesions.

TABETIC NEUROSYPHILIS[33] (“tabes dorsalis”), of course, often proceeds
to death without special complications of syphilitic nature. We have
chosen a case, however, to demonstrate a terminal complication with
vascular insult. Incidentally the case shows another complication
inasmuch as the cause of death was rupture of aortic aneurysm. It is
important to bear in mind these complications in tabes dorsalis which go
to prove that the spirochetosis of tabetic neurosyphilis is not limited
to the region of the spinal roots or to the spinal region in general.
Tabetic neurosyphilis is apt to be only a part of a total picture of
neurosyphilis just as neurosyphilis itself is only a part of the general
syphilitic process.

Our case of PARETIC NEUROSYPHILIS[34] (“general paresis”) is a
characteristic one in duration (three years and three months). The
aortic sclerosis almost constantly found in neurosyphilis and especially
in paretic neurosyphilis is here also shown. The spinal cord showed
lesions which are also almost always found in paretic neurosyphilis. The
characteristic frontal emphasis of the atrophic and indurative lesions
is shown. There is also a display of gross changes in the pia mater. The
characteristic so-called granular ependymitis or sanding of the
ventricular surface is shown. The case is distinguishable from the
paradigm in not showing the effects of vascular insults in the shape of
cysts of softening. The cerebellar sclerosis of the case is fairly
characteristic of paretic cases. There is even a suggestion of atrophy
in the temporal region suggesting the so-called Lissauer’s paresis.
Clinically the case belongs in the classical grandiose group of paretics
(“O. K. No. 1 superfine”).

VASCULAR NEUROSYPHILIS[35] is illustrated in a fourth autopsied case. It
may be noted that the pia mater in this case is practically normal. The
tissues outside the area of softening due to the syphilitic thrombosis
of nutrient vessels are practically normal. The case was one of almost
complete sensory aphasia with word-deafness. The clinical picture is
accordingly quite distinct from those of the paradigm (1) and of the
case of general paresis (3) just discussed.

JUVENILE PARESIS[36] is illustrated by a case with exceedingly extensive
lesions, largely meningeal and parenchymatous. The cerebral lesions are
atypical since in places they suggest the tuberous sclerosis of
Bourneville. The brain atrophy is extreme (965 grams) and it is possible
that this apparent brain atrophy was in part hypoplasia, since the
spirochetosis of this case was doubtless congenital. However, clinically
the patient was fairly normal up to the age of 18.

A case of so-called SYPHILITIC EXTRAOCULAR PALSY[37] demonstrates a
characteristic meningeal process more extensive than the clinical
symptoms would have indicated. In fact, focal clinical nerve palsies are
as a rule, if not constantly, partial phenomena of a far more extensive
process of neurosyphilis. They are far more limited clinically than
anatomically and histologically. It seems at first sight improper to
term them cases of diffuse neurosyphilis in view of their clinical
focality, yet they are best described as partial cases of diffuse
neurosyphilis.

A case of GUMMA[38] of the left HEMISPHERE is presented which appears to
have led to death in about four years from onset. This case, like many
others, is not an example of purely focalized syphilitic process
inasmuch as cysts of softening indicating slight vascular insults are
present elsewhere (pons). There is also a degree of leptomeningitis,
particularly basal.

Our discussion of the nature and forms of neurosyphilis is completed by
a rare case probably belonging in the so-called _cervical hypertrophic
meningitis of Charcot_ but actually due to a GUMMA OF THE SPINAL
MENINGES.[39] The importance of therapeutic optimism is emphasized in
this case as in the paradigm. Theoretically the meningeal inflammation
of neurosyphilis ought to be almost entirely if not entirely removed by
therapy, and these two cases, like several others in the series, seem to
illustrate this possibility.


Neurosyphilis sometimes receives the clinical diagnosis neurasthenia
simply through omission to apply proved diagnostic methods. An instance
is given in which the PARETIC form of NEUROSYPHILIS (“general paresis”)
received the diagnosis _neurasthenia_[40] for a period of five years, at
any time during which period it would doubtless have been possible to
render the correct diagnosis and apply treatment.

Neurosyphilis may imitate not only the psychoneuroses but also the
psychoses themselves. We present a case of an architect, which looked
almost precisely like _manic-depressive psychosis_[41] and had a history
of attacks, but in which the positive serum W. R. led (in accordance
with hospital rules) to an examination of the spinal fluid. The spinal
fluid tests proved the case to be one of PARETIC NEUROSYPHILIS.

However, a positive serum W. R., even when associated with mental
symptoms, and when those mental symptoms include grandiosity, does not
prove the existence of neurosyphilis either in its paretic or
non-paretic form. Our instance seems to be one of MANIC-DEPRESSIVE
PSYCHOSIS.[42] The spinal fluid tests were entirely negative. The course
of the disease was also that of manic-depressive psychosis. In the
absence of positive spinal fluid tests, the diagnosis neurosyphilis was
excluded.

Neurosyphilis and even PARETIC NEUROSYPHILIS may result in symptoms that
would ordinarily lead to the diagnosis _dementia praecox_.[43]

It is important not to rule out neurosyphilis on the ground of a
_negative serum_ W. R. The fluid W. R. may turn out positive. We present
a case (of a salesman)[44] in which the serum W. R. was repeatedly
negative (even salvarsan did not act provocatively) yet the spinal fluid
W. R. proved positive. The case was clinically one of classical PARETIC
NEUROSYPHILIS (“general paresis”). It is a good rule to proceed to
lumbar puncture, even when the serum W. R. is negative, if there are
suspicious symptoms (e.g., speech defect and memory impairment,
grandiosity) or signs (e.g., marked reflex disorder, especially
pupillary disorder).

DIFFUSE NEUROSYPHILIS was above defined as
“meningovasculoparenchymatous.” This disease is typically associated
with six positive tests (positive serum W. R., positive fluid W. R.,
pleocytosis, gold sol reaction, positive globulin reaction and excess
albumin). One or more and frequently several of these six tests are
likely to run mild in diffuse neurosyphilis; that is to say, these tests
are apt to run milder than the identical tests in paretic neurosyphilis
(“general paresis”). The clinical course of the diffuse, and especially
the meningovascular cases, is likely to be protracted. The prognosis as
to life is good, barring fatal vascular insults. The illustrative
case[45] was a case with slow course. There was a series of attacks
followed by a paralytic stroke, a finding highly typical of the diffuse
form of neurosyphilis. The spinal fluid reactions were mild, suitable to
the general principle above stated.

These tests are likely to run stronger, as above stated, in paretic
neurosyphilis (“general paresis”), than in the diffuse form. In
particular, the gold sol reaction is likely to be shown in what is
termed “paretic” form rather than in what is termed “syphilitic” form.
The clinical course of PARETIC NEUROSYPHILIS is likely to be brief. A
characteristic case[46] with very heavy globulin and albumin tests is
presented.

TABOPARETIC NEUROSYPHILIS[47] (“taboparesis”) is clinically a
combination of the symptoms of tabetic (“tabes dorsalis”) and those of
paretic neurosyphilis (“general paresis”). First comes the tabes
dorsalis lasting often for many years. Afterward follows a
characteristic general paresis. The ultimate paretic picture is likely
to retain, however, various characteristics of tabes. The laboratory
tests in the paretic phase of taboparesis are characteristic of general
paresis and not of tabes dorsalis. The prognosis after the paretic phase
has arrived is that of general paresis.

The diagnosis of the neurosyphilitic forms would be easy if these
principles were always carried out to the letter. The important fact is
as follows: diffuse (that is, meningovasculoparenchymatous
neurosyphilis) may look like paretic neurosyphilis (“general
paresis”)[48] at certain periods of the clinical and laboratory
examination. This fact is of obvious importance. The general prognosis
of diffuse neurosyphilis is regarded as good _quoad vitam_. The general
prognosis of paresis is bad. If, however, the differential diagnosis
cannot be rendered at particular phases of a given case, then no safe
prognosis can be offered in the individual case. In particular no
prognosis affecting the administration or non-administration of modern
systematic treatment can or should be offered in these doubtful phases.

It is not always safe to exclude neurosyphilis even when the _fluid_ W.
R. is _negative_.[49] Particularly in vascular neurosyphilis the fluid
W. R. and even all the other laboratory signs in the spinal fluid may
sometimes be negative. A positive serum W. R. yields the correct pointer
to diagnosis. Of course, also in many cases of vascular neurosyphilis
one or more of the laboratory signs may be suggestive even when the
fluid W. R. is negative. Theoretically there may be cases in which all
the six tests are negative and yet the diagnosis neurosyphilis be the
correct one.

A clinically important sign in neurosyphilis is the so-called
_seizures_. These occur both in DIFFUSE NON-PARETIC NEUROSYPHILIS[50]
and in PARETIC NEUROSYPHILIS.[51]

_Aphasia_ is likewise a symptom in both these forms of neurosyphilis,
namely, in the DIFFUSE non-paretic[52] and in the PARETIC form.[53]

The literature contains reference not only to seizures and aphasia as
characteristically paretic but also to _remissions_. Remissions like
seizures and aphasia are found in both the PARETIC[54] and NON-PARETIC
forms of neurosyphilis.[55] They have important bearings on prognosis in
all forms of neurosyphilis and are of especial significance in the
evaluation of treatment. (Remissions coincident with apparent cure.)

So far we have been dealing with cases of neurosyphilis in which there
was no doubt of the existence of mental symptoms. There are cases,
however, in which although the laboratory signs of neurosyphilis exist,
proving beyond doubt the existence of a chronic inflammatory reaction
and allied pathological conditions in the cerebrospinal axis, there are
no mental symptoms of neurosyphilis. We have called some of these cases
PARESIS SINE PARESI[56] and present examples.

To illustrate complications we give a case of PARETIC NEUROSYPHILIS with
autopsy in which there were ante mortem signs of HERPES ZOSTER[57] or,
at all events, of a skin eruption limited to the area of a thoracic
nerve.

A case of GUMMA of the brain[58] in which decompression was warranted
and performed is presented. The fluid W. R., as in many such cases, was
negative; serum positive.

A case of CRANIAL NEUROSYPHILIS (extraocular palsy[59] without mental
symptoms) showed a positive Wassermann serum test and a negative spinal
fluid.

The laboratory reactions in TABETIC NEUROSYPHILIS[60] (“tabes dorsalis”)
run somewhat like those of diffuse non-paretic neurosyphilis and are
accordingly milder than those of paretic neurosyphilis. The fluid W. R.
and the gold sol reaction in particular are apt to run mild. The
clinical course of tabes dorsalis is well known to be protracted and the
prognosis _quoad vitam_ is good except that we must always bear in mind
the possibility of vascular insults and complications of a syphilitic
origin in the rest of the body.

It is important to remember that TABETIC NEUROSYPHILIS is often quite
atypical[61] clinically and may even show no single symptom warranting
the old clinical name locomotor ataxia.

There are even cases in which the name tabes dorsalis is not warranted
in view of the fact that the lesions are not low in the cord but are
higher up (TABES CERVICALIS[62]).

A rare form of neurosyphilis is ERB’S SYPHILITIC SPASTIC PARAPLEGIA[63]
against which one needs to consider a number of non-syphilitic spinal
cord diseases. Our case showed a weakly positive serum W. R., a negative
fluid W. R., and the other tests of the spinal fluid were moderately
positive.

SYPHILITIC MUSCULAR ATROPHY[64] is classified by Head and Fearnsides
both in their meningovascular group and in their group of the so-called
syphilis centralis. Our case affecting in large part the small muscles
of the hands in a teamster, may be due either to spinal parenchymal
lesions or to root neuritis or to both.

It is a little extraordinary and very important that the _laboratory
signs_ are apt to be positive even in the SECONDARY period of SYPHILIS.
Perhaps a third of all cases of syphilis in the secondaries would, if
tested, yield positives precisely like those of full-blown paretic or
diffuse neurosyphilis. Strangely enough, these signs may occur without
clinical symptoms. The illustrative case,[65] a mechanic, yielded
various mental symptoms. The cases of secondary syphilis with laboratory
signs of neurosyphilis but without clinical symptoms are of the greatest
theoretical importance in relation to the problem above mentioned of
_paresis sine paresi_. It may well be inquired whether in some instances
the neurosyphilis of the secondaries does not persist until the
exhibition of mental or physical symptoms of neurosyphilis years later.
It must be remembered that this conception is hardly more than a
hypothesis at the present time. That such signs of chronic inflammation
could exist without symptoms is not so surprising when one thinks of the
startling immediate improvement seen after treatment or even in
remissions without treatment. One is reminded of the crisis in pneumonia
wherein clinical improvement takes place entirely independent of the
mechanical conditions in the lung which just after the crisis remain as
suppurative as before.

The diagnosis of JUVENILE NEUROSYPHILIS is made upon the same lines as
that of neurosyphilis in the adult. We present two cases, one with optic
atrophy[66] and the other with signs of congenital syphilis antedating
the symptoms of paresis.[67]

Congenital syphilis is also apparently capable of producing a simple
form of FEEBLEMINDEDNESS,[68] that is to say, a form of disease
non-paretic, non-tabetic, and without special tendency to vascular
insults.

We present a case of JUVENILE TABETIC NEUROSYPHILIS (“juvenile
tabes”).[69] The tests were all positive.

The line of separation between typical and atypical cases of
neurosyphilis is vague and indistinct and some of the cases classified
by us amongst puzzles perhaps belong under systematic diagnosis and vice
versa. The section on PUZZLES AND ERRORS in the diagnosis of
neurosyphilis is introduced by six cases of error in the diagnosis of
the paretic form of neurosyphilis.[70] These errors were made known by
autopsy. Aside from the sixth case, whose etiology must remain in doubt
and which was a unique case of PERIVASCULAR GLIOSIS, there is ground for
the belief that the other five cases in this Danvers Hospital study of
diagnostic errors were perhaps actually syphilitic though not of the
paretic form of neurosyphilis. At all events, the brain tissues in these
cases failed to show the plasma cell deposits which are characteristic
in the sheaths of the intracortical vessels in paretic neurosyphilis.

A case illustrates the complication of TABES by _arteriosclerotic
symptoms_, in which case the arteriosclerosis may naturally have been of
syphilitic origin. Two cases especially illustrate the possibility of
confusing the ataxia of general paresis with CEREBELLAR ATAXIA. These
cases showed lesions of the cerebellar structures, notably of the
dentate nucleus. No one can read these cases or any of the autopsied
cases in our series, without perceiving how fundamental and even
critical is the demand for autopsies in fatal cases of neurosyphilis.
The practitioner who can secure an autopsy in a fatal case of
neurosyphilis and have the tissues worked up by approved
neuropathological methods is almost bound to add his bit to neurological
theory. Even cases of classical tabes dorsalis are often signally
important to the theorist on account of the relations of the neural to
the non-neural complications.

We then proceed to a group of cases without special order in which a
variety of diagnostic questions arose.

A case of questionable neurosyphilis in the secondary stage of syphilis
brings up the problems of syphilitic _neurasthenia_.[71]

Syphilis may act as _agent provocateur_ of HYSTERIA as Charcot
insisted.[72]

A case illustrative of difficulties in diagnosis between neurosyphilis
and manic-depressive psychosis follows.[73]

A case for diagnosis is given which shows that errors in the diagnosis
of neurosyphilis are entirely possible even when abundant clinical and
laboratory data are available. A case with a weakly positive Wassermann
reaction in the spinal fluid finally turned out to be one of BRAIN
TUMOR.[74]

Some questions as to the diagnosis of NEUROSYPHILIS _versus Idiopathic
Epilepsy_ are brought up by a case in which phenomena of paresis seemed
to have occurred very early, about two years after the initial
syphilitic infection.[75]

A case of PARETIC NEUROSYPHILIS is offered in which _hemiplegia_ and
_hemitremor_ strongly suggested _vascular_ lesions; but the autopsy
showed no coarse lesions and merely confirmed the diagnosis paresis
microscopically.[76]

An autopsied case of PARETIC NEUROSYPHILIS is given, in which the pupils
persisted in reacting normally. Herpes zoster-like lesions in life
yielded no special signs at autopsy (all root-ganglia looked alike above
and below zone of “shingles.”)[77]

An example of NEUROSYPHILIS, probably PARETIC, yielded symptoms highly
suggestive of _manic-depressive psychosis_.[78] An interesting feature
in this case was the birth of a healthy child nine months after the
onset of the psychotic attack.

An example of _exophthalmic goitre_[79] following the acquisition of
SYPHILIS showed at autopsy a heavy scarring of the optic thalamus and
unilaterally atrophic process in the cerebral cortex.

We come to some questions concerning the _Argyll-Robertson pupil_. It is
agreed on all hands that the Argyll-Robertson pupil is characteristic of
the paretic and tabetic forms, but the sign occurs also in other
neurosyphilitic conditions;[80] in fact the sign does not necessarily
indicate neurosyphilis as an instance of PINEAL TUMOR demonstrates.[81]

The question raised above as to the possibility that neurosyphilis may
exist in the absence of positive findings in the spinal fluid is
illustrated in a man, a mechanic, who claimed syphilitic infection and
showed an _Argyll-Robertson pupil_ on one side.[82] The serum W. R. was
positive; the _fluid tests_ were _negative_.

An extraordinary case is given in some detail in which NEUROSYPHILIS in
the form termed DISSEMINATED ENCEPHALITIS[83] proved fatal within seven
months of the initial infection.

We have frequently mentioned the classical assumption that paretic
neurosyphilis (“general paresis”) is a fatal disease. Some have
suggested that there is another form clinically almost identical with
general paresis except that it pursues a long course and the suggestion
has been made that these cases be termed _pseudoparesis_.[84] We are of
the opinion that this term should be dropped and advocate the use of the
word pseudoparesis only for non-syphilitic disease looking like paresis,
such as alcoholic pseudoparesis and the like.

The question whether there is a form of mental disease SYPHILITIC
PARANOIA[85] is raised by a case with auditory hallucinations, ideas of
persecution and attacks of excitement. The diagnosis of alcoholic
hallucinosis was actually made although there is no proof that the
patient ever drank alcohol.

Alcohol may cause symptoms identical with those of paretic
neurosyphilis, including seizures, Argyll-Robertson pupils, speech
defect and mental symptoms. The differentiation is readily made by the
negative laboratory findings. An illustration is given in our case of
the alcoholic teamster. Cases such as this bear the name ALCOHOLIC
PSEUDOPARESIS.[86]

However, when the clinical picture is the same as in the case of our
teamster, the alcohol may only be a complicating factor in
neurosyphilis, as shown by our next case of the _alcoholic_ steamfitter
who in fact was shown to have NEUROSYPHILIS.[87]

Sometimes cases of apparently frank _alcoholism_, even with apparently
characteristic delirium tremens and neuritis, prove to be essentially
neurosyphilitic.[88] On the other hand, true combinations of ALCOHOLISM
and NEUROSYPHILIS occur which it would be proper to classify under
either heading and in which therapy must take serious account of both
conditions.[89]

As above stated, we elect to use the term pseudoparesis only for
non-syphilitic cases. There are other forms of pseudoparesis than
alcoholic pseudoparesis. The question of _Diabetic Pseudoparesis_ is
raised by an exceedingly complicated case of which our best
interpretation is that the patient, a proved syphilitic (with syphilitic
osteomyelitis (?)), a huge doorkeeper, was perhaps suffering from an old
SYPHILITIC scarring of the PITUITARY body.[90] Neither this case nor a
second case, one of PARETIC NEUROSYPHILIS with _glycosuria_ is actually
entitled to the diagnosis diabetic pseudoparesis. The second case of
paretic neurosyphilis with glycosuria brings up some unanswerable
questions as to the pancreatic or basal meningitic or other origin for
the glycosuria.[91]

_Isolated symptoms_ are often presented by neurosyphilitics (e.g.,
hemianopsia);[92] but we tend to regard these cases as due to focal
lesions that are merely part and parcel of DIFFUSE LESIONS.

A neurosyphilitic case (a steward) with the rather unusual complication
(for our northern region) of severe MALARIA producing cerebral
thrombosis is reported.[93]

The diagnosis _Dementia Praecox_[94] was actually made in the case of a
young school-teacher in whom the laboratory findings proved conclusively
that the condition was one of NEUROSYPHILIS. The gold sol reaction in
this case was mild. The chief lesion at autopsy was a fresh looking,
gelatinous pial exudate over the spinal cord which turned out to contain
an almost pure display of very numerous plasma cells.

The question of LUES MALIGNA[95] is brought up in a rectifier of spirits
in whom the characteristic tremendous destruction of tissue, toxemia and
failure to react to antisyphilitic treatment were illustrated. Moreover,
this case had a trauma (cautery) to the tonsil, as in other cases of
lues maligna.

A case somewhat suggestive of _brain tumor_, of _neurosyphilis_ and of
_multiple sclerosis_[96] turned out to be MULTIPLE SCLEROSIS (the fluid
showed a pleocytosis and a moderate amount of globulin with a paretic
type of gold sol reaction).

As a foil to this case that we regard as multiple sclerosis, we present
a second case with nystagmus, optic atrophy and spasticity in which the
suspicion of _multiple sclerosis_ might well be raised but which the
tests demonstrated to be NEUROSYPHILITIC.[97]

An even stranger imitation of well-defined non-syphilitic entities was
presented by a case apparently of _Huntington’s chorea_[98] (except for
absence of the hereditary taint) which case, however, proved to the
surprise of all diagnosticians to be one of NEUROSYPHILIS.

Frequent errors of diagnosis must occur in the field of the senile
psychoses. We present a case that would at first blush warrant the
diagnosis of _senile arteriosclerotic psychosis_[99] in a sea captain of
75 years (wife dead 15 years before of general paresis) who turned out
to be a characteristic case from the laboratory standpoint of
NEUROSYPHILIS.

The Protean nature of the symptomatology of neurosyphilis is
sufficiently established. Still, a case that might fit into textbooks
concerning DISSOCIATION OF PERSONALITY[100] is certainly a clinical
oddity, as illustrated by a fugacious musician.

A case with strong suspicions of _neurosyphilis_ of _tabetic_ type
turned out to be more probably one of neural complications in PERNICIOUS
ANEMIA.[101]

NEUROSYPHILIS IN JUVENILES presents puzzling conditions.

One case was marked clinically by _attacks of excitement_.[102] It is
impossible to place this case among the main groups of juvenile
neurosyphilis.

Another case of FEEBLEMINDEDNESS,[103] also NEUROSYPHILITIC in origin,
presented physical symptoms and laboratory signs of paretic
neurosyphilis; yet this case had been considered one of _simple
feeblemindedness_.

A case apparently of JUVENILE PARETIC NEUROSYPHILIS in a 15 year old boy
presented the rather unusual complication of shocks with
quadriplegia,[104] a _vascular complication_ not usually expected in the
paretic type of neurosyphilis in adults.

Epileptic phenomena[105] are rare as the effect of JUVENILE
NEUROSYPHILIS, but occur as demonstrated in a case which slipshod
methods of diagnosis might well have regarded as one of _idiopathic
epilepsy_.

A case of JUVENILE PARETIC NEUROSYPHILIS with the complication of
ADDISON’S DISEASE[106] is given (autopsy confirmation).

The puzzle in diagnosis offered by syphilis in the secondary stage[107]
is illustrated by a case which showed the characteristic NEUROSYPHILITIC
complications of the SECONDARY STAGE of syphilis. This patient may well
have been a moron at the outset and exhibited some reactions (refusal to
talk) explicable on the basis of feeblemindedness. She was a
neurosyphilitic only in the sense of the neural complication that we
find in the secondary stage of syphilis. As stated above, we do not yet
know what the fate of these neural complications of secondary syphilis
is to be. The frequency of this finding in secondary syphilis is
probably too great to warrant the hypothesis that it must always go on
to a chronic neurosyphilis; but we certainly are warranted in regarding
these cases as potential chronic neurosyphilitics.

A case of TABOPARETIC NEUROSYPHILIS in which the heavy exudate
characteristic of paresis became a soil for a growth of the typhoid
bacillus is presented with autopsy.[108] This fatality with TYPHOID
MENINGITIS is merely a concrete example of the many complications which
syphilitics and especially neurosyphilitics have to sustain.


The case series then goes on to illustrate, though quite inadequately, a
variety of MEDICOLEGAL AND SOCIAL complications of neurosyphilis. It is
well known that many social complications with grave moral, economic and
even political difficulties occur.

Our series starts with a “public character”[109] whose eloquence and
reformatory efforts led to a considerable notoriety. The autopsy in this
case showed singularly few lesions despite the fact that the case was
microscopically one of wholly characteristic PARETIC NEUROSYPHILIS. The
question might arise how far we are entitled to correlate the
reformatory efforts of this always eccentric character with syphilis.
The man himself a physician, was aware of the doubt which his
Argyll-Robertson pupils threw upon his medical situation. He explained
them on the basis of an old smallpox! We are inclined to think that the
whole of this man’s life, from his giving up of medical practice to live
as a kind of literary and political hack, was due to subtle changes of
neurosyphilitic origin. The fact that there was a certain delinquent
streak in the man is not inconsistent with this idea. Interestingly
enough, a fall on the ice in the man’s 61st year actually started up the
fatal process, a condition of affairs amply illustrated in cases of
neurosyphilis, brought out by trauma that come to the attention of the
Industrial Accident Board in connection with claims for compensation.

A case of sudden _grandiosity_[110] illustrates an episode of
NEUROSYPHILITIC origin. Such a person might well be regarded by the lay
newspaper reader as a crank or a grafter but the neurosyphilitic
possibility should always be entertained in cases of this order.

As against the social difficulties that look in the direction of the
classical paretic grandeur, we present a case of apparent _suicidal
attempt_ by gas, which attempt was followed by a period of amnesia that,
taking into account the laboratory findings, was probably
NEUROSYPHILITIC.[111]

Vistas of extraordinary interest are opened out by studies of the
relation of neurosyphilis to _delinquency_. The case of the psychopathic
reformer (Case 83) above mentioned was one in which the delinquency may
possibly have been related to acquired syphilis. We present also a case
of juvenile neurosyphilis, a young man of reform school type[112] in
which JUVENILE PARETIC NEUROSYPHILIS was established. This patient, in
fact, deteriorated very rapidly to a condition of considerable dementia
a few months after the diagnosis was established.

A striking case of so-called DEFECTIVE DELINQUENCY is presented, an
alcoholic prostitute of the reformatory group.[113] The NEUROSYPHILIS in
this case was a complication rather than an original factor in the
delinquency.

One case of PARESIS SINE PARESI was that of an habitual criminal[114]
and forger who, without showing mental or physical symptoms of
neurosyphilis, yielded the laboratory signs of paretic neurosyphilis.
Again, as in the case of the prostitute just mentioned, the
CRIMINALITY[115] seems to have antedated the neurosyphilis and even to
have been hereditary.

By way of introducing the next group of Industrial Accident Board cases,
we present a case of JUVENILE PARESIS with initial TRAUM.

The Industrial Board group is of note in that the signs of the traumatic
form[116] of paretic neurosyphilis do not occur immediately upon the
accident. Some time elapses in which the physical, chemical or
parasitological changes have time to work themselves out in the injured
tissues. Many hypotheses may be raised as to the reason why a trauma
lights up a syphilitic process. Of course, =false claims=[117] =may be
made for compensation by neurosyphilitics= in whom the symptoms were
already in existence before the accident and in whom they may not even
be markedly exacerbated by the accident. The false claimants can
probably not readily frame a story which the expert psychiatrist cannot
discredit if he is allowed to perform laboratory tests and give the
patient the benefit of thorough examination. However, some cases of
established PARETIC NEUROSYPHILIS are perhaps truly subject to
_exacerbations_[118] of the clinical process and it may well be held
that such exacerbations warrant partial compensation.

The fact that a trauma may light up a syphilitic process is illustrated
in a case that came to the Psychopathic Hospital, in which a SYPHILITIC
LESION developed in the skull AT THE SITE OF SKULL INJURY.[119]

A case of OCCUPATION-NEUROSIS[120] that might be interpreted as a
_syphilitic neuritis_ is presented. The case is still in doubt as to its
scientific evaluation.

The workmen’s compensation group of syphilitic cases is of extraordinary
general interest since it indicates that employers may well be on the
lookout not to employ known syphilitics unless fortified by special
insurance arrangements. Whether in future employers may desire =to
employ only W. R. negative workmen= is one of the highly complicated
questions _re_ workmen’s compensation and health insurance.

But the problems of neurosyphilis are not merely medicolegal and broadly
public or social. The most appealing difficulties lodge within the bosom
of the family. Now and then a case of INCOMPATIBILITY OF TEMPERAMENT,
perhaps complicated by _alcoholism_, occurs which tests prove to be
NEUROSYPHILITIC.[121]

Special attention should be drawn to a certain NEUROSYPHILITIC
FAMILY[122] in which both parents and five children showed a variety of
syphilitic diseases, including syphilis without apparent neural
complications, paretic neurosyphilis, juvenile paresis, aortic aneurysm,
achondroplasia and caries of the spine, and an as yet indefinite
neurosis. There was a sixth child that died shortly after birth, as well
as three stillborn.

One =cannot conclude= from the normal[123] look of a neurosyphilitic’s
family =that the normal-looking members are not syphilitic=, as
illustrated by the family of our draughtsman.

The most =intricate social complications= may arise. We present a case
of a syphilitic man (a well-to-do merchant) who was apparently being
goaded into a second marriage[124] because he was continually being
charged with having caused his first wife’s death. This he had actually
done in a certain sense because his wife had died of general paresis,
having contracted syphilis from him.


In the fifth section on THERAPY, we have attempted to outline some of
the principles and problems that arise in the treatment of
neurosyphilis. Enough has probably been said concerning the attitude of
optimism or pessimistic nihilism that may be adopted toward the whole
subject. It must be borne in mind, however, that a great deal of the
work on treatment of neurosyphilis is still in the experimental stage.
As a rule, each case must be considered separately and individually and
the prognosis can be made satisfactorily only after treatment has been
given. This section contains a group of cases that have been treated
rather intensively and the results of this treatment are indicated. The
section is introduced by _five untreated cases_, the brains and cords of
which have been studied post mortem. These illustrate the pathological
conditions which we have to meet, and from these examples we can draw
the theoretical conclusion that some cases are beyond the aid of therapy
on account of the brain destruction. Others, in which the symptomatology
bespeaks just as grave a situation, turn out on autopsy to have very
little actual damage to the brain tissues and therefore should
theoretically at any rate be amenable to antisyphilitic therapy.

In order to get any adequate conception of the possibilities of
therapeutic results in cases of neurosyphilis, one must consider the
pathological changes that occur and how far these changes are reparable.
In cases in which the destruction of tissue is marked, it is, of course,
out of the question to expect to get any marked clinical improvement. A
case of spastic hemiplegia[125] in paretic neurosyphilis is given with
the autopsy findings as an illustration of irreparable damage that may
occur to the parenchymatous structure, thus precluding any chance of
functional recovery.

On the other hand, there is a group of cases in which the symptoms may
be exceedingly severe and yet the actual destruction of tissue be almost
nil. This point is illustrated by a case[126] in which _total duration
of symptoms_ terminating in death was _only 22 days_. At autopsy there
was very little in the way of macroscopical lesions, and microscopically
there was no marked evidence of destruction in the parenchymatous
tissue. The lesions were represented chiefly by perivascular
infiltration. According to all our modern ideas, this type of reaction
is resolvable under antisyphilitic treatment. Though this case was one
of very short duration, similar pathological pictures may be obtained in
cases of considerably longer standing. It is also of great importance to
remember that symptomatically such a case may be in no way distinguished
from a case with marked atrophy.

Another autopsied case is given which shows an exceedingly =marked
meningitis=.[127] The meningitic processes according to the literature
and experience react very readily to antisyphilitic treatment in the
form either of mercury and iodid or in combination with salvarsan. The
lesion here present would probably have improved had intensive treatment
been given. Clinically the diagnosis of general paresis was made and, as
has been the rule in the past, treatment was not given on the ground
that it had no value in paresis. While this is an extreme case of
meningitis, it is to be remembered that the vast majority of cases of
paretic neurosyphilis show some degree of meningitis. Just as in the
marked meningitis of the diffuse neurosyphilis, so with the meningitis
of the paretic form, improvement is expected under treatment. As a part
or even the whole of the symptomatology in a given case may be due to
this meningitic process, we have reason occasionally to expect marked
improvement as the result of antisyphilitic treatment.

As a contrast to this case with marked meningitis, another case of
=marked atrophy=[128] is given. Here the atrophy was very perceptible on
macroscopical examination and the mere view of the brain at once
indicated that in such a case important results from treatment were not
to be expected.

The =topographical variation= of the lesions in neurosyphilis must be
remembered when treatment is to be instituted. Thus very marked lesions
may exist in portions of the brain which do not give any very definite
localizing symptoms. As a result, one may be led to believe from
clinical evidence that the case is a very mild one though the lesions
may really be very extensive. The topographical distribution must,
therefore, be taken into consideration in trying to estimate the damage
done. This point of topographical distribution of the lesions is
illustrated by a case.[129]

It has been generally recognized that =clinical improvement=, if not
cure, may be =readily obtained in the group of diffuse neurosyphilis=,
i.e., so-called cerebral and cerebrospinal forms of syphilis. These are
cases in which the parenchyma is very slightly, if at all, affected and
in which the lesion is chiefly in the meninges and blood vessels,
irritative rather than degenerative. A case[130] is given to illustrate
this point. In our experience systematic intravenous salvarsan therapy
associated with mercury and iodid gives remarkably good results in the
vast majority of this group of cases.

It is generally conceded that antisyphilitic treatment, particularly
salvarsan, has a very satisfactory result applied to diffuse
neurosyphilis. But the same good results may be obtained in cases which
are not so typically of the diffuse type. An illustration is given in
the case of a machinist in which the diagnosis was in doubt between
paretic, tabetic or diffuse neurosyphilis.[131] The result of treatment
was as satisfactory as could be expected in any type of neurosyphilis
and this in a case of several years’ duration with Argyll-Robertson
pupils.

As a rule, the Argyll-Robertson pupil is taken as a grave omen for
treatment, an idea based upon a conception that the Argyll-Robertson
pupil so frequently represents the old so-called “parasyphilitic” cases,
which, in the past were taught as being incapable of improvement by the
ordinary antisyphilitic methods.

A second case[132] with Argyll-Robertson pupil shows again that the
=prognosis may be very good despite the Argyll-Robertson sign=.

But even in the diffuse neurosyphilis, the symptomatic results of
treatment may not be entirely happy. Under treatment it may be possible
to reduce the spinal fluid tests to negative without, however, as in the
case of our hemiplegic lady,[133] making the physical or mental symptoms
disappear. In other words, it may be possible to stop the active
progress of the disease without removing the symptoms.

One is always warned of the danger of intravenous salvarsan therapy in
hemiplegic cases due to arteriosclerotic conditions. While this warning
is well justified, it does not mean that the most intensive treatment is
contraindicated, as shown in the case of our hemiplegic machinist.[134]
Such may be given over long periods of time with the most satisfactory
results.

A case[135] is given which illustrates the value of antisyphilitic
treatment in cases showing symptoms of intracranial pressure due to
syphilitic disease. In the case of the woman which we cite, we believe
that the symptoms of intracranial pressure were probably due to a
gummatous new growth, although it is possible that they were due to a
marked meningitic process. However, the results of a limited amount of
antisyphilitic treatment in this case were very brilliant. Similar
results may often be obtained in gumma of the brain. This is not always
true, however, and it may become necessary to use surgical procedure in
order rapidly to overcome the effects of intracranial pressure.

While it has always been conceded that treatment would greatly help
cases of diffuse and vascular neurosyphilis, the utmost pessimism has
existed concerning the results to be obtained by treatment in cases of
tabetic and paretic neurosyphilis. Only in the last five or six years,
due to the stimulus of Ehrlich’s discovery of salvarsan and the
introduction of the intraspinous methods of therapy, have intensive work
and study been given to the treatment of these cases. And though it has
been by no means settled in the minds of the various workers in this
field, as to what the ultimate results of such treatment will be and
though some do not believe that there is any good to be expected from
our present methods, still the majority of men who are treating these
cases systematically feel very much encouraged.

=At times very brilliant results= are to be obtained by intraspinous
treatment =in tabetic neurosyphilis= (“tabes dorsalis”). A very striking
illustration is given of a case of this sort in which the symptoms dated
only a few months but which had all the classical symptoms, signs and
laboratory tests. Five intraspinous injections of mercurialized serum
were sufficient to cause the disappearance of the subjective symptoms
and to reduce the spinal fluid test to negative.[136]

It must be emphasized that the best results in cases of tabetic
neurosyphilis are usually to be expected in cases in which the symptoms
are of short standing. Where the process is of long duration and much
destruction of spinal cord tissue has occurred, the best one can expect
is that the activity and progress may be halted. This is illustrated by
our case of a baker, 43 years of age, who had been suffering from the
symptoms of tabes for some years. Under treatment it was possible to get
an entirely negative serology of the blood and spinal fluid.[137]
Despite this evidence that the activity of syphilis had ceased, the
symptoms continued unabated. We are ready to believe, however, that much
good was accomplished. For the patient should not have any further
untoward developments or the appearance of any new symptoms. These,
without such treatment, might well be expected. At times excellent
clinical results are obtained in long-standing cases.

The results of treatment in paretic neurosyphilis (“general paresis”)
have been considered even less hopeful than in tabetic neurosyphilis
(“tabes dorsalis”); indeed, it has often been stated that the patients
are made worse by treatment. Recent work, however, supports a much more
optimistic viewpoint. We feel that =intensive treatment has been of the
greatest value in a number of cases of paretic neurosyphilis=. Two cases
are given which show the most satisfactory and brilliant results of
intensive intravenous salvarsan therapy in cases diagnosed as general
paresis. The first case, an excellent salesman, 46 years of age, with
most aggravated mental symptoms, recovered symptomatically and all his
tests were rendered negative.[138] He has now remained entirely well and
economically efficient for about two years without further treatment.
The other case,[139] a housewife, also with very marked symptoms
suggestive in all ways of general paresis, also recovered rapidly under
treatment and her tests became negative. Her remission has now lasted
for nearly three years without further treatment.

At times it is not possible to get the spinal fluid tests to become
negative in cases of paretic neurosyphilis under the most intensive
salvarsan therapy. In spite of this, the clinical condition of the
patient may improve so greatly that the patient can be considered
=clinically recovered=. An illustration is given of an undertaker[140]
who was brought from a condition of the greatest cachexia and mental
confusion to a condition of robust appearance and mental efficiency
under intravenous salvarsan therapy, in spite of the fact that his tests
were very slightly if at all reduced in intensity. He has been able to
resume his former occupation and his former life with great satisfaction
to himself and his family.

Improvement in paretic neurosyphilis under treatment is not to be
expected very early. =Two or three months of active treatment= may
elapse before one sees signs of improvement. Indeed, as illustrated by
our case of the shipping clerk, this improvement may begin to make its
appearance only after more than four months of intensive treatment
consisting of two injections of salvarsan per week.[141] In spite of the
long delay in this case, complete clinical recovery occurred and the
tests became almost negative at the end of a year of treatment.

It is not only in the central nervous system that the syphilitic process
may resist the most intensive treatment. In the case of the speculator,
a victim of paretic neurosyphilis, which we cite, a perennially
recurrent iritis appeared after several months of the most intensive
salvarsan treatment which was apparently sufficient to reduce the
symptoms of the paretic neurosyphilis,[142] but not of non-neural
syphilis.

We give the case of a charwoman having the diagnosis of paretic
neurosyphilis, who, under intensive treatment, made a symptomatic
recovery. The interesting point in her findings is that all the tests in
the spinal fluid became negative except the gold sol reaction which
remained of the “paretic” type.[143] There is no general rule as to the
reaction of the spinal fluid tests under treatment. At times one test is
the first to disappear under treatment; again it is another. We have
seen many cases in which the gold sol was the first test to become
negative and others, as the case given, in which it is the last to show
any change. As in our undertaker, symptomatic clinical improvement may
be practically complete without any change in the spinal fluid tests.

One must remember that it is the condition of the patient that is of
first importance; not so much the laboratory tests. Having shown the
clinical recoveries with the tests remaining positive, we now have to
report two cases in which there was =improvement= as shown =by the tests
but no clinical improvement=. The first patient, a bank teller[144] of
39 years, with a diagnosis of paretic neurosyphilis, received intensive
intravenous salvarsan for several months. Under this treatment all the
tests became negative except the gold sol which remained of the paretic
type. In spite of this, there was not the slightest improvement in his
mental condition.

The second case, a young man of 29 years in whom the symptoms of
neurosyphilis had recently appeared, under treatment showed a marked
diminution in the intensity of the spinal fluid tests, notwithstanding
which the patient became more and more demented and died after a series
of convulsions.[145]

Of course, good results indicated above in some of our cases of paretic
neurosyphilis are not to be expected in every case no matter how
intensive the treatment. We give a case of paretic neurosyphilis in
which the most intensive intravenous salvarsan therapy gave no
satisfactory results. This was followed by several intraventricular
injections of salvarsanized serum. The results of this combined
treatment, however, were still not satisfactory, and the patient
died.[146]

In order to emphasize as strongly as possible what we believe is a great
=advantage of systematic intensive treatment= for neurosyphilis, we
offer two cases in different time periods of neurosyphilis. The first is
a printer with the symptoms of diffuse neurosyphilis six months after
the appearance of his chancre.[147] These symptoms appeared despite
three injections of salvarsan, injections of mercury and mercury by
mouth. Under intensive treatment (meaning injections of salvarsan twice
a week and continued injections of mercury), complete recovery occurred
in a few weeks.

The second case is that of a waiter with signs and symptoms of
neurosyphilis in whom the diagnosis lay between the diffuse and paretic
forms.[148] This patient developed his symptoms in spite of continuous
antisyphilitic treatment during the six years since his infection. This
treatment had been comparatively mild, consisting in great part of
mercury by mouth. However, he had had courses of injections of mercury
and several injections of salvarsan. Under a systematic course of
intravenous injections of salvarsan twice a week for a number of months,
all symptoms disappeared and the spinal fluid tests became negative as
well as the W. R. in the blood serum.

A final case is offered which indicates that antisyphilitic treatment
may occasionally be of service in improving the mentality of a
FEEBLEMINDED CONGENITAL SYPHILITIC.[149]

No attempt has been made in this section to give a per cent evaluation
of the results of treatment in any one group of neurosyphilis. Two
charts (charts 25 and 26), however, are appended which give an
indication of some of our results. It seems to us, however, that it is
too early to make any definite statements as to how far treatment will
take us in the groups of neurosyphilis. We do feel decidedly, however,
that many patients, in whatever group of neurosyphilis the diagnosis may
place them, will respond to intensive systematic antisyphilitic
treatment. =It is unfair to give an entirely grave prognosis in any case
of neurosyphilis until the effect of treatment has been tried.=


In a separate section, entitled NEUROSYPHILIS AND THE WAR, we have
presented fourteen cases selected from British, French and German
writers in the war literature of 1914–16. Most of these cases were
naturally somewhat inadequately reported under the critical conditions
of literature made in the war. We present the cases for what they are
worth: at all events they draw attention to the extraordinary interest
of the neurosyphilis problem in relation to the war.

Such cases as A, one of tabes dorsalis apparently developing paresis by
a process akin to shell-shock, is of value in the interpretation of the
development of paresis in civil life. By “shell-shock” we commonly refer
to a condition in which there is no actual traumatic injury of the
brain. The hypothesis must be then that the explosion in some way
indirectly caused an alteration of living conditions of the spirochetes,
permitting the development of paresis.

Case B similarly seems to be a case in which a latent syphilis has
turned shell-shock into tabes dorsalis.

Cases C, D, E bring up the question of aggravation of neurosyphilis _by_
service and _on_ service, respectively.

Case F likewise shows how, in the determination of amount of pension,
the probable duration of the neurosyphilitic process is important.

Case G seems to show that war stress alone, without the emotional or
physical effects of shell-shock, may kindle a latent syphilis into
paretic neurosyphilis.

Case H similarly suggests that the “gassing” process may effect the same
result.

Case I seems to show that the neuropathically tainted person may have
latent epilepsy brought out through syphilis, the syphilis in this case
having been acquired during the first summer of the war.

Case J was an interesting case of a syphilitic who, after the stress of
the Battle of Dixmude, became an epileptic.

Syphilitic root-sciatica was developed in Case K at work in the war
zone.

Case L is one of a civilian who apparently would not have developed
paresis at precisely the moment when he did, if he had not been
discharged as a German Jew from his long-held bank position in London.

Two cases, M and N, are cases of shell-shock, non-syphilitic; yet the
picture of paresis in the one case and of tabes in the other was for a
long time almost convincing to the examiners. They are better termed
cases of pseudoparesis and pseudotabes, using the prefix “pseudo”, as
usual, to signify a non-syphilitic imitation of the disease in question.

To sum up in the most general way the lessons of this book, we may
emphasize again (1) _the unity-in-variety of the phenomena of
neurosyphilis_, (2) _the value of a hopeful approach to the therapy of
all cases of neurosyphilis_, _even the paretic form_, and (3) _the value
of applying syphilis tests to every case of neurosis or psychosis_.

(1) RE _unity-in-variety of neurosyphilitic phenomena_.

The unity of these phenomena is confirmed, theoretically, by the common
factor of spirochetosis: practically, by the Wassermann reaction,
positive in serum or spinal fluid! Almost at this point the unity of
phenomena ceases. Neither chronicity, nor evidence of mononuclear cell
deposits, nor evidence of serious structural damage to the nervous
system, nor presence of other positive tests than the W. R.,[150] nor
existence of mental or nervous symptoms or signs, is a common feature of
neurosyphilis. Sometimes the nervous system appears to harbor
spirochetes in the most cordial manner as guest-friends (_paresis sine
paresi_.) Again, perhaps as an expression of elaborate processes of
immunity, the spirochetes take effect in relatively huge gummata.
Sometimes the neurosyphilitic process rises as if by a regular process
of siege from spinal nerve-root to spinal nerve-root (tabes dorsalis and
diffuse neurosyphilis). Again, the nervous system is taken by storm, as
it were (disseminated encephalitis). Very frequently the neurosyphilis
is simply an indirect effect of blood-vessel disease, and huge masses of
tissue are scooped out in necrosis with dependent secondary
degenerations; and later the extinct lesions of vascular origin may or
may not betray evidence of their syphilitic origin. Sometimes diffuse
processes run on, apparently, with perfect fatalism to a mortal issue in
a few years both with and without treatment. Again treatment appears to
accomplish much (see fuller discussion under 2). The laws governing the
preference of processes to lodge in membranes, vessels, and parenchyma,
and in all combinations of these, have not been worked out. Hardly a
case of neurosyphilis, properly studied ante mortem and post mortem, but
would throw important light on our medical approach to one of the great
problems of civilization, the problem of syphilis as a whole.

(2) RE _value of a hopeful approach to the therapy of neurosyphilis_.

The prognosis of neurosyphilis is not worse than that of the chronic
diseases in general. In fact, the prognosis of neurosyphilis _quoad
vitam_ is either good or dubious, certainly not bad. The surprising
reversals of form which the spirochete shows in certain remissions are
always to be awaited. Treatment of neurosyphilis has certainly effected
amazing results, not so much by way of Ehrlich’s _therapia sterilisans
magna_ as by means of systematic intensive treatment. Even paretic
neurosyphilis (general paresis) seems to have been cured. Preparetic
phases are theoretically hopeful. Nor is it so certain that paretic
neurosyphilis will ultimately prove a perfectly distinct species of
neurosyphilis. General paresis seems to us at least to be more closely
related to diffuse neurosyphilis than is tabes dorsalis to diffuse
neurosyphilis. In any particular case, moreover, =during a good part of
the early months or years=, =it is difficult or impossible to tell the
paretic from the non-paretic forms of diffuse neurosyphilis by any
combination of clinical observations and tests=. In the instance of more
protracted neurosyphilis, e.g., tabetic, the outlook for vascular
complications is such that antisyphilitic treatment directed at
prevention of these complications is scientifically warrantable, even if
the tabetic process itself proves unassailable. The old distinction of
syphilis and parasyphilis, so striking and apparently satisfactory when
introduced by Fournier, seems to be a false distinction which should be
dropped. Therapeutically, we should approach all cases of neurosyphilis
without bias or nihilistic prejudgments.

(3) RE _universal applicability of syphilis tests in nervous and mental
cases_.

The importance of putting every neurosis or psychosis through syphilis
tests is not based alone on the frequency of neurosyphilis, though
neurosyphilis is surely frequent enough. The importance of universally
applying these tests is established by the experience of lingering
doubts both in the physician’s mind and (nowadays increasingly) in the
patient’s and friends’ minds, so long as these tests are not applied.
Nor should the positive serum Wassermann reaction fail to be followed by
lumbar puncture and appropriate tests. The general practitioner
confronting neuroses or psychoses—and what practitioner does not?—must
not expect valuable results from consultation with neurologists and
psychiatrists when he does not carry to these specialists the results of
at least the serum W. R. in his patient. Not only are practitioners,
specialists, and patients subject to discomfiture on the eventual and
delayed proof of syphilis or neurosyphilis, but valuable time has been
lost to treatment. How often the physician of yore (and really not so
long since) had to be regarded as an eccentric virtuoso if he tested
urine as routine! Well, for routine use in nervous and mental diseases,
the Wassermann serum reaction is at least as important as urinalysis.
Nor would we cease our homily with the general practitioner. We know
neurologists and psychiatrists who use the Wassermann test _only when it
is likely to be positive_! But they are dying out.




                               APPENDIX A


In appendix A a brief outline is given of the six tests (W. R. on blood
serum and spinal fluid, cell count, globulin test, albumin test, gold
sol test). This is not intended as a complete working manual but rather
as indicating the methods used in diagnosis in the cases presented
herein. For more complete details the reader may be referred to
textbooks on the subject of serology, among which may be mentioned
Kaplan: “Serology of the Nervous System”; Plaut, Rehm and Schottmüller:
“Leitfaden zur Untersuchungen der Zerebrospinalflüssigkeit”; Kolmer:
“Infection, Immunity and Specific Therapy,” and, for the Wassermann
technique, an article by Dr. W. A. Hinton in M. J. Rosenau’s “Preventive
Medicine and Hygiene.”

Our own W. R’s. have been performed at the Wassermann laboratory of the
Massachusetts State Board of Health (formerly the Neuropathological
Testing Laboratory, Harvard Medical School), under the supervision of
Dr. W. A. Hinton. The other tests are performed at the Psychopathic
Hospital. It is very important that a close relationship should exist
between the clinician and the Wassermann laboratory if the most is to be
obtained from the reactions. This relationship has been effectively
close between the authors and the above-mentioned laboratory; and has
enabled us to get very much clearer ideas about certain cases than could
otherwise have been obtained.

=Cell Count.= In order to obtain the number of cells per cmm., the
examination should be made of the fresh fluid as soon as possible after
this is withdrawn. The most convenient counting chamber for this purpose
is the so-called Fuchs-Rosenthal counting chamber, the ruled spaces of
which contain slightly over 3 cmm. (an ordinary blood cell counting
chamber may be used). According to the method used by us the cells are
stained in a pipette with Unna’s polychrome methylene blue. Using a
white-counting pipette, stain is drawn up to the first or second marking
and the remainder of the pipette filled with spinal fluid. This makes no
change in the dilution for practical purposes. After two or three
minutes the staining is satisfactory and the counting may be done. With
this stain a differential count may be made. Plasma cells stain a
lavender as contrasted to the blue of the lymphocytes. The
characteristic halo surrounding the eccentric nucleus is visible. The
blood cells do not assume color with this stain; hence it is unnecessary
to add any acetic acid.

For permanent preparations, and more accurate differential counts of the
spinal fluid, the Alzheimer method may be used. The technique is given
in a paper by H. A. Cotton and J. B. Ayer as follows:[151]

1. Lumbar puncture in the usual manner.

2. 96% alcohol, in proportion to twice the amount of cerebrospinal
fluid, is added drop by drop and well mixed.

3. Centrifuge the mixture for one hour at high speed in a glass tube
with conical end. (An ordinary electric urinary centrifuge apparatus can
be employed, the tube to be well stoppered to prevent evaporation.)

4. The supernatant fluid is poured off, leaving a small coagulum in the
bottom of the tube.

5. Add absolute alcohol—alcohol and ether—ether, each separately for one
hour, to dehydrate and harden coagulum.

6. The coagulum can now be gently loosened from the bottom of the tube
by a long needle. The tube is then inverted, and the coagulum allowed to
fall into the hand by a quick tap on the end of the tube. Care must be
taken not to squeeze or handle the coagulum. The hand is placed over a
small homeopathic vial, containing thin celloidin, and the coagulum
allowed to drop into the celloidin, where it remains over night (twelve
hours usually).

7. Coagulum is placed in thick celloidin which is allowed to evaporate
slowly. It is then mounted on blocks and sections cut 14µ in thickness.

8. The sections are stained and mounted according to the following
procedure:

(_a_) Remove celloidin by absolute alcohol and ether.

(_b_) 80% alcohol.

(_c_) Water.

(_d_) Sections are carried on glass or platinum needle into a dish of
Pappenheim’s pyronin-methyl green stain and kept in a water bath at 40°
C. five to seven minutes.

(_e_) Quickly cool dish in running water.

(_f_) Wash off superfluous stain in plain water.

(_g_) Absolute alcohol to differentiate—until no more stain comes away
from section.

(_h_) Clear in Bergamot oil.

(_i_) Mount in balsam.


The normal cell count may be stated as being up to 6 cells per cmm.;
from 6 to 12 cells may be considered as suggestive of pathological
condition and more than 12 cells per cmm. as definitely pathological.
The type of cell in syphilitic diseases is preponderantly the small
lymphocyte. A low percentage, that is, very rarely over 20%, of large
lymphocytes, endothelial phagocytic cells, polymorphonuclear leucocytes
and plasma cells may also be found. The finding of plasma cells in any
number in the spinal fluid is suggestive although not conclusive
evidence for the diagnosis of paretic neurosyphilis.

=Globulin= is an albumin which is precipitated by half saturation with a
salt. A very simple and satisfactory test is known as the Nonne-Appelt
test, which has been modified by Ross-Jones. Into a test tube of small
diameter, run 1 cc. of spinal fluid. Place under this fluid with a
pipette, 1 cc. of a saturated solution of ammonium sulphate
((NH_{4})_{2}SO_{4}). If any globulin is present a white,
sharply-defined ring will form at the junction of the two fluids.
According to our readings, a ring that is just visible with the aid of a
black background is called 1+, a ring that is just visible without the
black background, 2+; a ring easily perceptible, 3+ and a relatively
very heavy ring, 4+. On shaking the tube, if globulin is present, the
fluid will show turbescence.

Another simple globulin test used in our laboratory as a check on the
Nonne-Appelt test is the Pandy test. A few cc. of a clarified 10%
solution of phenol are placed in a watch glass. One drop of spinal fluid
is run into this solution. A milky turbescence indicates globulin.

The presence of globulin in the spinal fluid is always an indication of
abnormality of the cerebrospinal axis. There is nothing differential in
this finding as it occurs in all inflammatory processes. However, it is
characteristically present in most cases of neurosyphilis (exception to
the rule: the pure vascular type does not show globulin in a very high
per cent).

=Albumin Test.= Albumin in small quantities is present in all spinal
fluids. Increase over the normal amount occurs in pathological
conditions such as most cases of neurosyphilis, especially in those in
which globulin is found. Any albumin precipitant may be used for rough
clinical calculation, comparing the amount of precipitate with that from
the normal fluid. Our method is to place 1 cc. of spinal fluid in a
small test tube of about 5 mm. diameter and to precipitate the albumin
by the addition of 3 drops of 33⅓% of trichloracetic acid. This test has
its chief value as confirmatory of the globulin test, since in the vast
majority of instances where globulin is found there will also be found
an increase in albumin.

The =Gold Sol Reaction= is an empirical test discovered by Carl Lange in
the utilization of the work of Zsigmondi with solutions of colloidal
gold and albumins. Briefly the details of the test are as follows:

Ten tubes are set up in a rack. To the first tube 1.8 cc. of a 0.4% of
salt solution is added and to each of the following tubes 1 cc. of this
solution. Then to the first tube containing 1.8 cc. of salt solution one
adds 0.2 cc. of the spinal fluid to be tested. This gives a dilution of
1 to 10. From this tube 1 cc. is pipetted into the second tube and this
process continued through the ten tubes. This gives dilutions of spinal
fluid of 1 to 10, 1 to 20, 1 to 40, etc., to 1 to 5120 in the last tube.
Then 5 cc. of colloidal gold solution is added to each tube. A positive
reaction is indicated by the precipitation or throwing down of the
colloidal gold into its metallic form. This produces a change in color.
This precipitation may be partial or complete and the amount of
precipitation is indicated by the color and is read as follows:

The unchanged fluid is called 0; a slight change giving a red-blue as 1;
a further change giving a blue-red as 2; a straight blue as 3; a
lavender or violet as 4; and the colorless fluid representing complete
precipitation as 5. The numbers are placed in a row, indicating the tube
in which the color occurs. The fluid from a case of paretic
neurosyphilis will give a complete precipitation beginning in the first
tube and running through a number of tubes and then grading off. It may
be indicated 5 5 5 5 4 3 1 0 0 0. The characteristic reaction of fluids
from tabetic and diffuse neurosyphilis is less strong than from the
paretic. The greater part of the reaction will take place, however, in
the first five tubes, but as a rule it will not begin very strongly in
the first two. A characteristic reaction is 1 2 3 3 2 1 0 0 0 0. Another
reaction that may be considered characteristic of the tabetic or diffuse
form is 3 3 3 2 1 0 0 0 0 0. Fluids from non-syphilitic cases as a rule
give a reaction having its greatest intensity beyond the fifth tube,
that is, in the high dilutions.

A reaction characteristic of brain tumor or tuberculous meningitis is 0
0 0 0 1 3 3 2 1 0.

The conclusions that may be drawn from the gold sol reaction have been
summarized by one of the authors as follows:

1. Fluids from cases of general paresis will give a strong and fairly
characteristic reaction, especially if more than one sample is tested,
in the vast majority of cases.

2. Very rarely a general paresis fluid will give a reaction weaker than
the characteristic one.

3. Fluids from cases of syphilitic involvement of the central nervous
system other than general paresis often give a weaker reaction than the
paretic, but in a fairly high percentage of cases give the same reaction
as the paretics.

4. Non-syphilitic cases may give the same reaction as the paretics;
these cases are usually chronic inflammatory conditions of the central
nervous system.

5. When a syphilitic fluid does not give the strong “paretic reaction,”
it is good presumptive evidence that the case is not general paresis;
and this test offers a very valuable differential diagnostic aid between
general paresis, tabes and cerebrospinal syphilis.

6. The term “syphilitic zone” is a misnomer, as non-syphilitic as well
as syphilitic cases give reactions in this zone; but no fluid of a case
with syphilitic central nervous system disease has given a reaction out
of this zone (test thus valuable negatively). Any fluid giving a
reaction outside of this zone may be considered non-syphilitic.

7. Light reactions may occur without any evident significance, while a
reaction of no greater strength may mean marked inflammatory reaction.

8. Tuberculous meningitis, brain tumor and purulent meningitis fluids
characteristically, though not invariably, give reactions in higher
dilutions than syphilitic fluids.

9. The unsupplemented gold sol test is insufficient evidence on which to
make any diagnosis, but used in conjunction with the Wassermann
reaction, chemical and cytological examinations, it offers much
information looking toward the differential diagnosis of general
paresis, cerebrospinal syphilis, tabes dorsalis, brain tumor,
tuberculous meningitis, purulent meningitis.

10. We believe that no cerebrospinal fluid examination is complete for
clinical purposes without the gold sol test.


The =Wassermann reaction= as carried out in the Wassermann Laboratory is
based on the principles of the original method—the only essential
modification consists in the employment of cholesterinized alcoholic
extracts of human hearts as antigen instead of aqueous extracts of
foetal livers from cases of congenital syphilis. Experience has shown
that properly standardized antigens made from human hearts are much more
sensitive in the detection of true cases of syphilis.

=Antigens.= Three antigens are used, each being an alcoholic extract of
human heart which is saturated at room temperature with cholesterin.
These antigens differ slightly in their sensitiveness. Before the test
is made each antigen is diluted with 0.85% salt solution in the
proportion of four parts of the cholesterinized antigen extract to
sixteen parts of 0.85% salt solution. The amount to be used, the dosage,
is carefully determined by testing each antigen against a large number
of known positive and known negative specimens of blood. The dosage of
the antigens employed is less than one-half the amount which inhibits
hemolysis when the antigen is incubated for one hour with the hemolytic
system which consists of complement, amboceptor and cells in the proper
proportions. These antigens are designated as A, B, and C. Antigen A is
the most sensitive. B and C are very similar to each other
quantitatively and qualitatively.

=Specimens to be tested.= The serum which separates from the clot is
withdrawn, centrifugalized if necessary, and then heated at 55 degrees
for thirty minutes. 0.1 cc. of serum is used in the test and 0.2 cc. of
each specimen is used as a control to exclude the presence of
anti-complementary substances. Spinal fluids are tested in two ways. As
a routine 0.5 cc. of the spinal fluid is used in the test and 1.0 cc. is
used in the control; or when especially requested spinal fluids are
titrated by using respectively 1.0, 0.7, 0.5, 0.3, and 0.1 cc. of the
spinal fluid for each test and 1.0 cc. of spinal fluid for the control.
Spinal fluids are not inactivated.

=Complement.= The complement is obtained from the serum of guinea pig’s
blood. No complement is used when older than eighteen hours. A 10%
solution and 0.85% salt solution is used in the test. The amount used is
twice the minimum quantity necessary to hemolyze the sensitized cells.

=Sheep’s Corpuscles.= A 5% suspension of sheep’s corpuscles in 0.85%
salt solution is prepared from defibrinated sheep’s blood. The
corpuscles are washed three times and for each washing four to five
times as much 0.85% salt solution is used as the original volume of the
defibrinated blood.

=Amboceptor.= The amboceptor is prepared by injecting sheep’s corpuscles
into a rabbit. The serum of this rabbit which contains amboceptor is
diluted with 0.85% salt solution so that 0.25 cc. will hemolyze 0.5 cc.
of a 5% suspension of sheep’s corpuscles. In the test twice the quantity
or 0.5 cc. of amboceptor is used.

=Sensitized Cells.= The sensitized cells consist of equal parts of
washed sheep’s corpuscles and diluted amboceptor. This mixture is
incubated in a water bath at 37° C. for a half hour to effect the
sensitization of the cells.

=Technique of the Wassermann Test.= One-tenth cubic centimeter of each
inactivated specimen of serum and 0.5 cc. of each uninactivated specimen
of spinal fluid is pipetted into a separate tube. A mixture is freshly
prepared in salt solution, each cubic centimeter of which contains the
proper amount of antigen A (the most sensitive antigen), and two units
of a 10% solution of guinea pig serum (complement). One cubic centimeter
of this mixture is pipetted into each test tube. These tubes are then
incubated for forty minutes in a water bath at 37° C. At the end of this
period, sensitized cells are added, and the tubes are again incubated in
a water bath at 37° C. for one hour. Each specimen which shows any
degree of inhibition of hemolysis is retested in the afternoon. For this
second test antigen A is again used and in addition antigens B and C. A
control is also made for each specimen retested to eliminate any
possibility of the inhibition of hemolysis being due to
anti-complementary substances in the serum or spinal fluid tested. The
technique of the second test differs in no wise from that of the first,
except for the use of a control in each retested specimen and the
employment of three antigens instead of one. The degree of positiveness
is noted for each retested specimen and compared with the degree of
positiveness obtained for the corresponding specimen with the same
antigen-complement-salt solution mixture in the morning’s test. The
specimen is retested on the next day when discrepancies occur between
the morning reading for antigen A and the afternoon reading for antigen
A. From the above description it will be noted that the negative
specimens have but a single test with one antigen only, while the
positive specimens are retested, thus permitting a confirmation of any
positive reaction. In this way attention is focalized on the positive
specimens.

=Interpretation of Results.= Antigen C (the weakest of the three
antigens) is used entirely for diagnostic purposes and any specimen
showing the slightest degree of inhibition with this antigen and
stronger degrees of inhibition with the other antigens is reported as
positive. The specimens which are strongly or moderately positive with
antigens A and B and negative with antigen C are reported as doubtful.
In testing spinal fluids by the titration method, antigen C is used and
the readings are based upon the degree of inhibition of hemolysis noted.
The intensity of this inhibition is indicated by Arabic numerals: “5”
indicates complete inhibition, while “1” means a faint cloudiness, hence
a weak reaction. Intermediate numbers show relative intensity varying
between complete inhibition “5” (strong positive) and slight inhibition
“1” (weak positive); “—” equals no inhibition (negative).

Although it is commonly believed that the recent administration of
antisyphilitic treatment will affect the reaction by making it negative,
this is not our experience, and it is, therefore, not necessary that
treatment be withdrawn for a short period before the specimen is
submitted for examination.

The reaction as carried out in this laboratory has the following
diagnostic significance: =Positive indicates syphilis=, except very
rarely in acute febrile conditions such as malaria and pneumonia.
=Negative does not exclude syphilis.= In obscure conditions a series of
less than three negatives has little diagnostic significance. =Doubtful
suggests syphilis.= It is therefore advisable to submit three or more
specimens in such a case, and interpret a persistently or
predominatingly doubtful reaction as indicative of syphilitic infection.

=Bruck Test.= A new serum test for syphilis has recently been described
by C. Bruck.[152] Following are recent results in our laboratory with
this test.[153]

This new test for the diagnosis of syphilis by C. Bruck has aroused much
interest. The scientific standing of Bruck and the simplicity of the
technique led us to overcome our prejudice, that has been the offspring
of the numerous tests that have been offered of late. Bruck states that
since the discovery of the complement fixation test for syphilis by
Wassermann, Neisser and himself in 1906, he has been trying to find a
simple chemical reaction that would take the place of the complicated
technique of the Wassermann reaction. This method, as he has published
it, was worked out and is being used at the front, in the present war,
where complete laboratory equipment is not available.

Commencing our experiments with a great deal of scepticism, we were much
surprised at the results obtained, which are given below. Whatever may
be the final status of the test in the determination of syphilis, we
feel that there is a great deal of interest in the fact that this simple
chemical reaction does pick out certain differences in the composition
of blood sera and that apparently a large number of syphilitic sera
differ in their chemical composition percentage from the majority of
non-syphilitic sera.

The technique, while exceedingly simple, offers many chances for errors
and individual variations so that we have thought it well to give
directions and cautions at some length.

Bruck’s[154] technique is described as follows: “The test is made with
0.5 cc. clear serum in a test tube, to which is added 2 cc. of distilled
water, and the whole shaken. Then, with a precision pipette, 0.3 cc. of
the ac. nitr. purum of the German pharmacopeia is added and the whole
thoroughly shaken and then set aside at room temperature for ten
minutes. Then 16 cc. of distilled water at room temperature is added,
and closing the tube with the finger, it is shaken up and down three
times carefully, not vigorously enough to make it foam. This is repeated
ten minutes later, and the tube is then set aside for half an hour. By
this time the precipitate is entirely dissolved in the tube with the
normal serum, while the syphilitic serum shows a distinct, flocculent
turbidity. In two or three hours, or better still, in twelve hours, the
gelatinous and characteristic precipitate is piled up on the floor of
the test tube.”

The acid is prepared by diluting the Acidum nitricum of the U. S. P.
(Sp. gr. 1.403) with distilled water until the hydrometer shows the
specific gravity 1.149, which corresponds to the nitric acid of the
German pharmacopeia, but since this requires a special hydrometer, a
simpler method is to make a 25 per cent solution of the Acidum nitricum,
which will give about the proper specific gravity.

The serum is obtained by allowing 10 cc. of blood to stand at room
temperature for an hour, and then centrifuging. Serum that has stood for
some time may be used as well as the fresh, and even bloody serum does
not seem to confuse the results to any great degree. The serum gives the
same results with or without inactivation. Post mortem blood gave
results as constant as that obtained during life, in the few cases that
we had in this series. But the reaction may be influenced markedly by
the size of the test tubes. We have found that the 13×1.9 cm. is the
most favorable size.

When one first thinks of this test it appears very simple and probably
somewhat crude as a chemical reaction, but there are certain precautions
that must be observed, and several hundred normal and syphilitic sera
should be tried before the investigator can feel that he has a refined
routine technique. There is the personal equation which must be watched,
for here is probably the greatest source of error, and readily explains
why two different persons get widely varying results with the same sera
if they have done only a few dozen tests. We must take it for granted
that the reaction is a quantitative one, where some positive reactions
may differ only slightly from the normal non-syphilitic, and,
furthermore, any normal serum may be made to give a positive reaction,
and almost any positive serum be made to give a negative by improper
manipulation at some point in the test. There are as many places for
error to creep in as there are steps in the process. Bruck has omitted
many details in his publication, which allow personal variations, and so
we have tried to develop a routine process that will eliminate as many
of these as possible.

We shall here attempt to explain the methods which we have found most
satisfactory and at the same time indicate the places where error is
likely to occur. The 0.5 cc. of serum is added to 2 cc. of distilled
water, and shaken thoroughly. Now add slowly exactly 0.3 cc. of acid
from a precision pipette, care being taken it does not flow down the
side of the tube. The tube should be shaken gently while the acid is
being added, for this prevents the formation of a flocculent precipitate
in normal serum which is difficult to dissolve later. After the acid is
added shake each tube gently to make sure that these flakes do not
persist. It is difficult to shake each tube in exactly the same manner,
as must be done if we expect uniform results.

The first 250 tests of this series were made by allowing the tubes to
stand for ten minutes as Bruck advocates. Then we found that practically
all sera gave a positive reaction if allowed to stand 15–20 minutes, and
so in the other tests of the series an attempt was made to make the
reaction more sensitive by allowing the tubes to stand only 6–7 minutes.
During this time the tubes should be shaken gently once or twice. The
manner in which the 16 cc. of water is added also influences the
reaction. If allowed to flow freely in upon the precipitate, the
positive may be forced into solution as well as the negative. Both
pipette and tube should be slanted and the water allowed to flow down
the side of the tube without disturbing the precipitate. If all has gone
well up to this point, we may see a marked difference between the normal
and syphilitic precipitates, in that the normal will begin to go into
solution at once, thus clouding the water, while a positive precipitate
will be composed of large flakes which show little or no tendency to go
into solution or cloud the water above. It must be remembered that the
most flocculent positive precipitate will go into solution if the fluid
is splashed or shaken too hard while the tube is being inverted. If any
doubt as to the character of the precipitate now exists, it may be
allowed to stand ten minutes longer, and again inverted as before, or
even repeated several times during the next hour or two. We see no
reason why the tubes should be left to stand over night, for during this
time a precipitate usually settles in the normal tubes. This, however,
differs from the syphilitic precipitate in that it is still finely
granular and goes back into solution readily when the tubes are
inverted.

In view of these possible grounds for error, it is only logical to run
controls of known positive and known negative sera along with each group
of unknown bloods, and even then certain tubes will seem doubtful, in
which event the test should be repeated with added precaution to see if
a definite positive or negative reaction may be obtained.

In the last tests of this series we seemed to aid the reaction by
rendering the serum-water solution alkaline by one or two drops of 10
per cent potassium hydroxide before the acid was added. The positive
sera have a larger precipitate, while the normal seem to dissolve more
readily.

                                TABLE I

       Syphilis: nervous system involved.

       General Paresis  Wassermann and Bruck agree positively  47
                        Wassermann and Bruck agree negatively   7
                        Wassermann and Bruck at    variance    10

       Tabes Dorsalis   Wassermann and Bruck agree positively   3

       Cerebrospinal    Wassermann and Bruck agree positively   8
                        Wassermann and Bruck agree negatively   3

       Juvenile Paresis Wassermann and Bruck agree positively   1

       Summary:         Wassermann and Bruck agree positively  59
                        Wassermann and Bruck agree negatively  10
                        Wassermann and Bruck at    variance    10

                                TABLE II

       Syphilis: nervous system not involved.

       Syphilis         Wassermann and Bruck agree positively  12
                        Wassermann and Bruck at    variance     5

       Congenital Syph. Wassermann and Bruck agree positively   3
                        Wassermann and Bruck agree negatively   2

       Summary:         Wassermann and Bruck agree positively  15
                        Wassermann and Bruck agree negatively   2
                        Wassermann and Bruck at    variance     5

                               TABLE III

       Non-syphilitic: Wassermann reaction negative.
                        Doubtful or positive Bruck             86
                        Bruck test negative                   216

       Total for three groups:
                        Wassermann and Bruck agree positively  74
                        Wassermann and Bruck agree negatively 230
                        Wassermann and Bruck at    variance   101

The tests here reported were made on blood sera obtained from patients
admitted to the Psychopathic Hospital and its Out-Patient Department. As
a routine Wassermann test is made on each patient who enters the
hospital, it was only necessary to take another tube of blood from each
patient, and check the results in each instance with the Wassermann
reaction. As it takes several days to get the report from the Wassermann
laboratory of the State Board of Health, there was no chance of being
prejudiced by a previous knowledge of the Wassermann reaction. The cases
for the most part were those of mental disease; the majority in good
general physical health.

A comparison of the total number with the Wassermann reaction shows that
there was a general agreement of 304 of the 405 cases tested, or a
percentage agreement of practically 75%. In considering the cases of
syphilis of the central nervous system in a group by themselves, we find
that the agreement is closer, since 69 of the 79 cases tested, or 87%
agreed without any question of doubt. It will be noted that in several
cases of general paresis, the Wassermann reaction, which was repeated at
intervals, was negative, and in most of these cases the Bruck test was
negative also. Our few cases of congenital and latent syphilis also
checked very closely with the Wassermann test. In the various groups of
mental cases in this series, no factor of interference was discovered.
It is also of interest that in the cases where the blood was obtained
post mortem, the Bruck test agreed with the Wassermann result obtained
on ante mortem blood serum. Further work on post mortem sera will be
reported. Some of the patients not included in the syphilitic groups
that have a negative Wassermann and no clinical signs of syphilis, give
a history of previous infection at some time, which might partly account
for the variations in the two tests.

CONCLUSIONS

    1. We present results of the Bruck sero-chemical test in 405 cases.
      In 101 of these cases there were definite clinical manifestations
      of syphilis, in which the Wassermann and Bruck tests agreed
      positively in 74 or 75%. The two tests agreed negatively in 12
      instances, and were at variance in 15.

    2. In the group which showed syphilis of the nervous system we had
      64 cases of clinically certain general paresis, of which the
      Wassermann and Bruck tests agreed in 54 instances, or practically
      85%. In other forms of central nervous system involvement the
      agreement was 100% in the 15 cases tested.

    3. In the cases with no apparent involvement of the nervous system
      the agreement was somewhat less, being 76%. This may be in keeping
      with the fact that the Wassermann test was not so strongly
      positive in these cases.

    4. The advantages of the test are: (1) the short time required to do
      the test; (2) the limited amount of apparatus necessary, and (3)
      the simplicity of the technique.

    5. The disadvantages of the test seem, for the most part, to be
      bound up in the personal variations that are apt to occur.

    6. We are here dealing, most probably, with a quantitative chemical
      difference in the protein content of syphilitic and non-syphilitic
      sera, the nature of which is not understood by us. It is our hope
      that this may be brought to light in the near future in the field
      of chemistry.




                               APPENDIX B
       COMMON METHODS OF TREATMENT USED IN CASES OF NEUROSYPHILIS


The =treatment for neurosyphilis= according to the viewpoint of the
authors =is treatment for syphilis=. It is necessary in order to cure a
case of neurosyphilis to cure the syphilis in the patient. Accordingly,
the methods of treatment best adapted for the cure of syphilis are
indicated in the treatment of neurosyphilis. As experience shows that it
is often more difficult to cure the neurosyphilitic cases, treatment
will have to be pushed with greater intensity than in some non-nervous
system syphilis. In general, then, the methods that have been applied by
the syphilologist will be used in the treatment of cases of
neurosyphilis. In addition, methods attempting to bring the drug into
local contact with the central nervous system have been devised. The
methods of treatment have been in part indicated in Chart 27.

The method chiefly used in treatment of the cases of this book is what
we have called =intensive systematic intravenous treatment=. The
treatment consists of intravenous injections of salvarsan (or a
substitute for salvarsan, as arsenobenzol and diarsenol) given in a dose
of about 0.6 gram and repeated twice a week over a period of a number of
months. In addition, injections of mercury salicylate averaging 0.065
gram once a week are given and potassium iodid by mouth. As indicated,
the important point is to keep up treatment for a long period of time.
This method has produced practically no untoward results, certainly no
more untoward results than are to be expected with salvarsan in smaller
quantities and it has seemed to us that the therapeutic results have
been as satisfactory as in any other form of treatment.

Specialized forms of treatment intended to place the drug in contact
with the central nervous system may be described under the headings of
=spinal intradural treatment= and =cerebral subdural= and
=intraventricular treatment=.

Three main therapeutic agents have been largely used. These are (1)
salvarsanized serum according to the =method of Swift-Ellis= (=in
vivo=). The serum according to this method is prepared as follows: An
intravenous injection of salvarsan is given to a patient and blood
withdrawn at the end of one-half hour. This is allowed to clot. The
serum is removed and after inactivation at 56° C. for one-half hour it
is ready for use. The average dose is 15 to 30 cc. of serum. As a matter
of fact, it is not necessary to use the blood serum from the same
patient to whom the intraspinous injection is to be given. (2) The
salvarsanized serum according to the =method of Ogilvie= (=in vitro=).
Blood serum is prepared from any patient and to it is added salvarsan in
such a strength that the amount to be injected, 10 to 30 cc. of serum,
will contain 0.0001 to 0.001 gm. (3) Mercurialized serum according to
the =method of Byrnes=. Mercury bichloride is added to blood serum in
such proportion that the amount of serum to be injected will contain
from 0.00065 gram to 0.0026 gram.

The method of intraspinous injection is to perform lumbar puncture,
withdraw an amount of fluid approximately equivalent to the amount to be
injected; then allow the serum to be injected to run in by gravity.

For the =cerebral=, =subdural and intraventricular= injections, the same
sera may be used as for the intraspinous. Five or six times as much
salvarsan may be given, but a smaller amount of serum may be advisable,
that is, 10 to 15 cc. To perform injections a trephine opening is made
in the calvarium about the size of a dime. The location of choice for
the opening is slightly back of the longitudinal prominence just to the
right of the median line, to avoid the frontal sinus. For subdural
injections a curved needle is thrust between the dura and the brain and
the serum allowed to flow in slowly by gravity. For the intraventricular
injections a blunted spinal puncture needle is thrust through the brain
substance into the 3rd ventricle. When the 3rd ventricle is reached the
clear cerebral fluid will flow out; then after withdrawing a sufficient
amount, the serum may be introduced by gravity. The trephining may be
done under local anesthesia but as a rule it is better to induce general
anesthesia. The subsequent injections can be made without recourse to
any anesthesia whatsoever, as they are practically painless.

All procedures both in the injections and in the preparation of sera are
naturally to be performed under aseptic conditions.




                                 INDEX


 Abscess, tonsillar, associated with neurosyphilis, 250.

 Addison’s disease in juvenile paretic, 279.

 Agraphia, 101.

 Albumin test, 474.

 Allbutt, Clifford, 257.

 Alcoholism, chronic, 227.

 Alcoholic dementia, 237.
   epilepsy, 229.
   hallucinosis, 225.
   pseudoparesis, 222, 223, 451.

 _Allergie_, 129, 204.

 Alzheimer, 428.
   method, 472.

 Amboceptor, 477.

 Amnesia, 195.

 Anaphylaxis, 129.

 Anatomical formulae, 25.

 Antigens, 476.

 Aortic aneurysm, 35, 439.

 —— sclerosis, 41, 46, 135.

 Aphasia, 31, 43, 101, 262, 445.

 Apoplexy, 197.

 Argyll-Robertson pupil, 209, 212, 217, 291, 450.
   as isolated symptom, 217.
   in alcoholism, 214, 229.

 Arndt, Junius and, 249.

 Arsenobenzol, 375, 377, 389, 486.

 Arteriosclerosis, cerebral, 101.
   not a contraindication to intensive salvarsan therapy, 359.
   radial, 68.

 Ascending lesion, 23.

 Asymmetrical lesions, 19.

 Ataxia, 31, 223.

 Atheromatous degeneration, 35.

 Atrophy, cerebellar, 39.
   cerebral, 47, 134, 205.
   parenchymal, 41.
   pontine, 39.

 Atypical case congenital neurosyphilis, 270.

 Ayer, J. B., 472.


 Ballet, 72.

 Barrett, A. M., 54, 175, 187, 212, 218, 219.

 Bechterew, 219.

 Binet and Simon, 304.

 Binet scale, 277.

 Birnbaum, 403.

 Blood pressure, high, 70, 262, 124.

 Bly, 252.

 Bonhoeffer, 404, 415, 417.

 Bordet, 427.

 Bratz, 278.

 Bruck test, 479.

 Bruck, C., 479.

 Bumke, 214.


 Canavan, 256.
   and Southard, 70.

 Cell count, 471.

 Cerebral syphilis, see diffuse neurosyphilis.

 Cerebrospinal syphilis, see diffuse neurosyphilis.

 Cervical hypertrophic meningitis of Charcot, 56, 441.

 Chancre, extragenital, 75, 342.

 Character change, neurosyphilis, 314.

 Charcot, 60, 186.

 Choroiditis, 242.

 Christian, 407.

 Cimbal, 403.

 Civilization and syphilis, 76.

 Clinical evidences of syphilis, 131.

 Clouston, 158.

 Collins, Joseph, 145.

 Compensation in neurosyphilis, 309, 402, 456.

 Complement, 477.

 Conduct disorder, 38.

 Congenital syphilis, absence of stigmata, 318.
   as cause of feeblemindedness, 159, 447.
   involvement of nervous system in, 274.

 Congenital neurosyphilis, 270, 395.
   resembling feeblemindedness, 272.

 Conjugal neurosyphilis, 263.

 Convulsions, 43, 101, 248, 362.
   cause of in paretic neurosyphilis, 232.
   in psychopathic subject with syphilis, 417.

 Corneal opacity, syphilitic, 234.

 Cotard, 73.

 Cotton, H. A., 472.

 Craig, C. B., 152, 196.

 Cramer, 125.

 Cranial neurosyphilis, 140.
   tenderness, 139.

 Crises, gastric, 367.

 Cysts, ependymal, 59.
   of softening, 27, 36, 54.

 Cytorrhyctes luis, 381.


 Dana, Charles L., 65, 77, 78.

 Dazed states, 264.

 Deafness, 63.

 Decompression, 138.

 Defective delinquent—diffuse neurosyphilis, 300, 455.

 Dejerine-Tinel, 61.

 Delinquency and juvenile neurosyphilis, 298.

 Delirium tremens, 332.

 Dementia, 137.

 Dementia paralytica, see paretic neurosyphilis.

 Dementia praecox, 74, 185, 247.

 Depression, 95, 126.

 Depressive drugs, 189.

 Diabetes, and neurosyphilis, 240.
   insipidus, 190.

 Diabetic pseudoparesis, 238.

 Diarsenol, 377, 389, 391, 486.

 Differential diagnosis, alcoholism and neurosyphilis, 227, 231, 234,
    236.
   brain tumor, diabetic pseudoparesis and neurosyphilis, 238.
   diffuse and paretic neurosyphilis, 165, 193, 247.
   manic-depressive psychosis and neurosyphilis, 69.
   multiple sclerosis and neurosyphilis, 253, 255.
   neurasthenia and neurosyphilis, 65, 183.
   senile arteriosclerotic psychosis and neurosyphilis, 262.

 Diffuse neurosyphilis, cerebrospinal syphilis, cerebral syphilis,
    spinal syphilis, 17, 80, 85, 97, 103, 122, 140, 183, 193, 300, 331,
    342, 359, 433, 439, 443.
   premonitory symptoms, 342.
   prognosis, 80, 103, 124, 433, 443.
   spinal fluid findings in, 348.
   symptoms, 99.
   treatment, 98, 103, 184, 302, 390.
   treatment, results, 343.

 Diplopia, 50, 184, 253, 356.
   causes, 140.

 Donath, 401, 403.

 Drastich, 407.

 Duco and Blum, 403.

 Dupré, 407.

 Dysdiadochokinesis, 231.


 Ehrlich, 184, 428, 429.

 Encephalitis, 27, 248.
   disseminated, 218.

 Endarteritis, 220.

 Ependymal cysts, 59.

 Ependymitis, 40, 47, 49, 134.

 Epilepsy, 192.
   alcoholic, 229.
   brought out by syphilis, 415.
   Jacksonian, 103.
   parasyphilitic, 194.
   relation to juvenile neurosyphilis, 277.
   syphilitic, 103, 194.
   syphilogenic, 415.

 Epileptic neurosis, 195.

 Erb’s syphilitic spastic paraplegia, 147.
   treatment of, 148.

 Euphoria, 73.

 Excited states, 95.

 Exner, M. J., 416.

 Exophthalmic goitre, syphilitic (?), 205.

 Extraocular palsy, 140, 441.

 Eye changes in neurosyphilis, 257.

 Eye muscles, paresis of, 17, 50.

 Facial paralysis, 53.

 Families of neurosyphilitics, 275, 316, 318, 320, 373, 431, 457.

 Family of neurosyphilitic, normal-looking, but syphilitic, 318.

 Familial syphilis, 299, 306.

 Farrar, C. B., 411.

 Fearnsides, Head and, 21, 140, 150, 193, 217, 374, 378.

 Feeblemindedness, 395.
   and congenital syphilis, 159.

 Fernald, W. E., 159, 273, 396.

 Fildes, McIntosh and, 129, 329.

 Focal changes, 221.
   meningitis, 50.
   softenings, pontine, 54.

 Fournier, 142, 222, 186, 194, 381.

 Franz, 357.

 Froissart, 413.

 Fugue, hysterical, 264.


 Garnier, 407.

 General paresis, see paretic neurosyphilis.

 Glands, 270.

 Gliosis, 39, 47, 49, 136, 180.

 Globulin, 229.
   tests, 473.

 Glycosuria, 238, 241.

 Goddard, 397.

 Gold sol reaction, 247, 474.
   in brain tumor, 100.
   paretic, 85, 98.
   paretic, other tests negative, 383, 385.
   in purulent meningitis, 100.
   syphilitic, 85, 98, 345.

 Graham, Thomas, 429.

 Grandiosity, 72, 295, 455.

 Graves, W. W., 157.

 Grilli, 407.

 Gross, 257.

 Gumma, see gummatous neurosyphilis.

 Gumma of tonsil, 250.

 Gummatous neurosyphilis, 53, 56, 137, 138, 140, 221, 362, 438.


 Hallucinations, 53.
   in paretic neurosyphilis, 249.

 Hauptmann, 348.

 Head and Fearnsides, 21, 140, 150, 193, 210, 217, 374, 387.

 Headache, 53, 63, 122, 247, 352.
   causes of, 209.

 Hecht, 399.

 Hemianopsia in neurosyphilis, 242.

 Hemiplegia, 31, 45, 80, 122, 262, 360.
   causes of, 389.

 Hemitremor, 197.

 Heredity, neuropathic, 84.

 Herxheimer reaction, 152.

 Heubner, 427, 428.

 Hinton, W. A., 471.

 Huntington’s chorea, 258.

 Hutchinsonian teeth, 45.

 Hydrocephalus, 134, 306.

 Hyperreflexia, explanation of, 233.

 Hypochondriacal ideas, 133.

 Hysteria, 185, 301.

 Hysterical symptoms, 18.


 Incontinence, vesical in tabetic neurosyphilis, 144.
   rectal, 56.

 Incubation period of neurosyphilis, 152.

 Infectiousness of neurosyphilis, 95.

 Insight, 95.

 Insomnia, 63.

 Intracranial pressure, 139, 362.

 Intraspinal lesions, 95.

 Intraspinous therapy, 122, 366, 486.
   unpleasant results of, 366.

 Intraventricular injections, 389, 487.

 Involution-melancholia, 187.

 Iodine, untoward results, of, 363.

 Iritis, 17.


 Järisch-Herxheimer reaction, 72.

 Joffroy, 214.
   and Mignot, 64.

 Junius and Arndt, 249.

 Juvenile neurosyphilis, 438, 447.
   relation to epilepsy, 277.

 Juvenile paresis, see juvenile paretic neurosyphilis.

 Juvenile paretic neurosyphilis, juvenile paresis, 45, 154, 157, 272,
    275, 298, 306, 440.
   age of onset, 158.
   and Addison’s disease, 279.
   and delinquency, 298.
   prognosis, 156, 158, 162, 273, 275.
   treatment, 154, 161, 278, 299.

 Juvenile paretic neurosyphilis, with initial trauma, 306.
   congenital amputation of toes in, 158.

 Juvenile tabetic neurosyphilis, 161, 447.


 Kaplan, 255, 471.

 Kéraval, 257.

 Key, 427.

 Knee-jerks, absence of, 223.
   lively, 75.
   return of, 24.

 Koefod, Solomon and, 243.

 Kolmer, 471.

 Kraepelin, 65, 66, 69, 88, 91, 95, 187, 225, 249.

 Krafft-Ebing, 84.


 Laignel-Lavastine, 413.

 Lange, C., 428, 429, 474.

 Lancinating pains, 92, 141.

 Lépine, 408, 413.

 Leptomeningitis, 47, 54, 135.

 Lewandowski, 210.

 Liability of paretic, 295.

 Lissauer’s paralysis, 38.

 Locomotor ataxia, see tabetic neurosyphilis.

 Long, 418.

 Lucke, Baldwin, 93, 144.

 Lues maligna, 250, 452.

 Lumbar puncture, untoward effects, 352.
   treatment of, 354.

 Lüth, 278.

 Lymphocytosis, 23, 30, 40, 49.


 McDonagh, 381.

 McIntosh, Fildes and, 129, 329.

 Malaria, cerebral, simulation of paretic neurosyphilis, 245.

 Mallory and Wright, 472.

 Manic-depressive psychosis, 68, 71, 77, 187, 202, 291, 384, 442.

 Marie, Chatelin and Patrikios, 412.

 Marie, 408, 414.

 Martin, E. G., 313.

 Massary, de, 414.

 Mattauschek and Pilcz, 347.

 Medicolegal and Social, 454.
   period of paretic neurosyphilis, 414.

 Meilhon, 407.

 Memory, failing, 63.

 Meningitis hypertrophica cervicalis of Charcot, 56.
   sympathica, 19.
   syphilitic, 103.

 Mercurialization, 98.

 Mercury, 58, 83, 85, 98, 148, 193, 235, 376, 377, 389, 391, 395, 486.
   untoward results of, 363.

 Metasyphilis, 89.

 Metchnikoff and Roux, 427, 428.

 Microgyria, occipital, 47.

 Mignot, Joffroy and, 64, 66.

 Migraine, 19.

 Mitchell, H. W., 218.

 Mœbius, 429.

 Mott, F. W., 158, 257, 308, 396, 437.

 Multiple sclerosis, 253, 256.
   relation of syphilis to, 254.
   spinal fluid findings in, 254.

 Muscular atrophy, 149, 446.
   syphilitic relation to amyotrophic lateral sclerosis, 150.

 Muscular weakness, 279.

 Myerson, A., 196.


 Nageotti, 428.

 Nausea, 63.

 Neisser, 399.

 Nerve trunk tenderness, 148, 234.

 Nervousness, 63.

 Nervous indigestion, 63.

 Neurasthenia, 63, 183.

 Neuritis, cranial, 51.
   optic, 365.
   root, 235.
   syphilitic, 235.

 Neurorecidive, 152, 153, 184, 196, 235.

 Neuroses, relation of syphilis to, 186.

 Neurosyphilis, 187, 238, 240, 242.
   aggravated on military service, 404.
   atypical, 258, 346.
   atypical case resembling hysterical fugue, 264.
   dates, 428.
   forms of, 20, 21, 28, 29, 95.
   galloping, 328.
   history of, 427.
   incubation period, 152.
   infectiousness of, 95.
   laboratory findings in, 82.
   latent, 142, 203.
   lesions, 303.
   lighted up by stress of military service, 412.
   and marriage, 319.
   prevention, 320.
   onset, 64.
   in primary stage, 186.
   in secondary stage, 185, 283, 390.
   in secondary stage, prognosis, 390.
   in secondary stage, treatment, 153.
   spinal, 23.
   and the war, 399, 466.

 Nissl-Alzheimer method, 427.

 Noguchi, 381.
   and Moore, 428, 429.

 Nonne, 82, 125, 152, 186, 195, 196, 214, 216, 235, 254, 265.
   -Apelt test, 473.

 Numbness, 56.

 Nystagmus, 45, 253, 256, 279.


 Obersteiner, 249.

 Occupation-neurosis, 312.

 Ogilvie method, 487.

 Operation for gumma, 139.

 Optic atrophy, 256.
   in juvenile paretic neurosyphilis, 154.

 Optic thalamus, syphilitic lesion of, 205.

 Osteitis, syphilitic, 311.

 Ozena, 350.


 Pains, 31.

 Pandy test, 474.

 Paralysis, 123.
   recovery from, 342.
   of respiration, 248.

 Paranoia, syphilitic, 225.

 Paraphasia, 19, 43.

 Paraplegia, 26, 30.

 Parasyphilis, 89.

 _Paresis sine paresi_, 126, 186, 204, 303, 445.

 Paresis, see paretic neurosyphilis.

 Paretic neurosyphilis, dementia paralytica, general paresis, softening
    of the brain, 37, 63, 68, 74, 78, 80, 85, 97, 131, 188, 192, 197,
    199, 202, 227, 241, 262, 289, 295, 309, 314, 323, 338, 372, 375,
    377, 382, 384, 386, 388, 392, 435, 440, 442.
   adjuvant causes of, 414.
   causing social complications, 289.
   causes of death in, 197.
   course, 85.
   duration, 88.
   forms, 95.
   improvement, 377.
   incidence among officers, 407.
   incidence among soldiers, 402.
   lesions of, 131.
   “lighted up” by domestic stress in civil life, 420.
   “lighted up” by “gassing,” 414.
   mortality from, 89.
   nomenclature, 88.
   onset, 192.
   pathology of, 436.
   prognosis, 435, 444.
   symptoms, 90, 131.
   symptoms, mental, 87.
   symptoms, physical, 86.
   versus diffuse neurosyphilis, 165.
   versus vascular neurosyphilis, 169, 172.
   with very marked meningitis, 332.
   with very marked brain atrophy, 335.
   without mental symptoms, 315.
   traumatic exacerbation, 310.
   traumatic form, 308, 413.
   traumatic, shell-shock, 401.
   treatment of, 85, 370, 372, 377, 382, 384, 386, 388, 392.
   treatment, results of, 351.

 Pensions for disabilities resulting from venereal disease, 409.

 Pensions for neurosyphilis, 411.

 Peripheral neurosyphilis, 19.

 Perivascular infiltration, 41.

 Pernicious anemia with spinal symptoms, 267.

 Petit mal attacks, 195.

 Pförringer, 61.

 Phobia, 67.

 Pilcz, Mattauschek and, 347.

 Pitres and Marchand, 421, 424.

 Plaut, 249, 348, 428.

 Plaut, Rehm and Schottmüller, 471.

 Plasmocytosis, 40, 49, 55.

 Pleocytosis, 23, 220, 247, 344.
   effect of antisyphilitic treatment on, 244, 376.
   in remissions, 243.
   significance of, 243.
   spinal fluid otherwise negative, 270.

 Polydipsia, 190.

 Polyuria, 190.

 Pontine hemorrhage, 219.
   softening, 54.

 Posey and Spiller, 257.

 Potassium iodid, 58, 85, 98, 193, 222, 376, 377, 389, 486.

 Preparesis, 65, 77, 78.

 Prince, Morton, 195.

 Psammoma, 213.

 Pseudoneurasthenia, 66.

 Pseudoparesis, 449.
   alcoholic, 222, 229, 451.
   diabetic, 238.
   senile, 263.
   shell-shock, 421.
   syphilitic, 223, 371.

 Pseudoparetic neurosyphilis, 222.

 Pseudotabes, shell-shock, 424.

 Psychogenic neurosyphilis, 189.

 Psychographic disturbance, 228.

 Psychopathic personality, 302.

 Ptosis, 350.

 Pupillary reaction, changes in, 261.
   signs, 69.

 Pupils, Argyll-Robertson, see Argyll-Robertson pupils.
   irregular, 79, 201.
   normally reacting in paretic neurosyphilis, 199.
   sluggish reaction to light, 188.
   stiff as isolated symptom, 265.

 Purkinje cells, binucleate, 48.

 Putnam, James J., 19, 56.

 Pyramidal tract lesion, bilateral, 326.
   sclerosis, 44.


 Quadriplegia in juvenile paretic neurosyphilis, 275.

 Quincke, 427, 428.


 _Randsklerose_, 24.

 Ravaut, 428.

 Ravaut, Sicard, Nageotti, Widal, 428.

 Rayneau, 407, 413, 414.

 Recovery, 77.

 Recurrences, 70.

 Redlich, 403.

 Régis, 73.

 Remissions, 122, 435, 445.

 Retardation, 187.

 Retention of urine, 56.

 Retinitis, hemorrhages, 365.

 Richards, R. L., 402, 404, 406, 409.

 Robertson, A. R., 59.

 Rod cells, 226, 297.

 Romberg sign, 141, 216, 279.

 Root-sciatica, syphilitic, 418.

 Rosenau, 471.

 Ross-Jones test, 473.

 “Rum fit,” 229.

 Ryder, Charles T., 42.


 Saddle-shaped nose, 210.

 Salivation, 98.

 Salmon, Thomas W., 89.

 Salvarsan, 75, 83, 85, 193, 222, 377, 389, 486.
   provocative, 78, 79.
   untoward results of, 363.

 Salvarsanized serum, 75.

 Schaudinn, 427, 429.

 Sciatic pain in neurosyphilis, 149.

 Seizures, 31, 64, 83, 103, 444.
   causes of in paretic neurosyphilis, 194.
   Jacksonian, 392.
   minor, 392.

 Senile arteriosclerotic psychosis, 262.

 Sensitized cells, 478.

 Sérieux and Ducaste, 96.

 Shaikewicz, 404.

 Shanahan, 278.

 Sheep’s corpuscles, 477.

 Shock, 42, 81.

 Sicard, 428.

 Six tests, 80, 85.
   in tabetic neurosyphilis, 141.

 Smith and Solomon, 479.

 Social cases, 454.
   service, 232.

 Solomon, 142, 255.
   and Koefod, 243.
   Smith and, 479.
   Southard and, 202, 303.

 Somnolence, 45.

 Southard, E. E., 48, 134, 212.
   and Canavan, 70.
   and Solomon, 202, 303.
   and Taft, 397.

 Spasms, clonic, 326.

 Spastic hemiplegia in paretic neurosyphilis, 323.

 Spastic paraplegia, Erb’s, 147, 306.

 Spasticity, 18, 256.

 Speech defect, 69, 133.

 Spiller, 150.
   Posey and, 257.

 Spinal fluid findings in secondary stage of syphilis, 151, 185, 283.
   in juvenile paretic neurosyphilis, 275.
   negative in diffuse neurosyphilis, 140.
   negative in gummatous neurosyphilis, 138.
   negative in neurosyphilis, 216.
   negative in tabetic neurosyphilis, 269.
   in tabetic neurosyphilis, 141.

 Spinal fluid, withdrawal for therapeutic purposes, 377, 379.

 Spinal syphilis, see diffuse neurosyphilis.

 Spirochetes, “drug fastness,” 381, 394.
   strains, 76, 263, 276, 381, 394.

 Steida, 405.

 Sterility in tabetic neurosyphilis, 144.

 Stier, 407.

 Stokes, Wile and, 186.

 Suicide, 92, 126, 240, 296, 301.

 Summary, 427.

 Syphilis aggravated by service, 406, 411.
   on service, 409.

 Syphilis as cause of diabetes, 241.
   as cause of feeblemindedness, 396.
   hereditaria tarda, 160, 318.
   history of, 427.
   lesions in, 329.
   of lung, 211.
   from Mongolian, 76.
   primary, 65.
   secondary, 65.
   tertiary, lesions in, 329.

 Syphilitic feeblemindedness, pathology of, 160.
   neuritis, 312.
   psychosis, 91.

 Syphilophobia, 67, 361.

 Syphilotoxins, 72.

 Swift, 129, 212.

 Swift and Ellis, 428, 429.
   method, 428, 487.


 Tabes dorsalis, see tabetic neurosyphilis.

 Tabetic neurosyphilis, tabes dorsalis, locomotor ataxia, 30, 31, 141,
    146, 366, 367, 434, 446.
   associated with cerebral symptoms, 177.
   atypical, 143.
   cervical, 146.
   course, 141.
   with negative spinal fluid findings, 269.
   prognosis, 94.
   shell-shock, 403.
   “shell-shocked” into paretic neurosyphilis, 401.
   symptoms, 93.
   symptoms in order of frequency, 145.
   treatment, 145, 366, 367.
   plus vascular neurosyphilis, 175.
   with vascular insult, 30, 439.
   versus pernicious anemia, 267.

 Taboparesis, see Taboparetic neurosyphilis.

 Taboparetic neurosyphilis, taboparesis, 92, 135, 195, 284, 443.
   course, 92.
   nomenclature, 94.
   prognosis, 92, 443.
   and typhoid meningitis, 284.

 Taft, A. E., Southard, E. E., and,

 Talon, 407.

 Taylor, E. W., 50.

 Temperature, paretic, 376.

 Tests, changes under treatment, 102.
   changed to negative in paretic neurosyphilis without clinical
      improvement, 385.
   changed to less strongly positive in paretic neurosyphilis without
      clinical improvement, 386.

 Therapeutic conception, 324.

 Thibierge, 399.

 Thierry, 158.

 Throbbing in head, 63.

 Thrombosis, cerebral, 36, 42, 342, 357, 360, 124.

 Thymus, persistent, 282.

 Tibial exostoses, 100.

 Tigges’ formula, 248.

 Todd, J. L., 406, 409.

 Transient deafness, 18.
   blindness, 18.
   paralysis, 124.
   paralysis, condition in which occurs, 123.

 Trauma and juvenile neurosyphilis, 278, 306.
   neurosyphilis, 456.
   paretic neurosyphilis, 199, 308, 310.
   syphilitic osteitis, 311.

 Treatment of neurosyphilis, 67, 75, 83, 124, 148, 184, 222, 235, 299,
    328, 332, 335, 342, 346, 350, 351, 355, 384, 390, 392, 395, 419,
    439, 457.
   case in which theoretically of no avail, 323.
   methods, 356, 486.

 Treatment of syphilis, effect on development of neurosyphilis, 142,
    347.

 Tremor, 197.
   intention, 256

 Tubercle, 80.

 Tuberous sclerosis of Bourneville, 47.

 Tumor, cerebral, 53, 191, 238, 253.
   pineal, 213.


 Unconsciousness, 53.
   causes of, 389.


 Vascular changes, 220.

 Vascular neurosyphilis, 31, 42, 72, 296, 359, 433, 440.
   plus tabetic neurosyphilis, 175.
   prognosis, 433.
   versus paretic neurosyphilis, 169, 172.

 Veeder, B. S., 274.

 Vertigo, 122.

 Viet, 278.

 Virchow, 427, 428.

 Vomiting, 53, 63.


 Warthin, 241.

 Wassermann reaction, 191.
   and alcoholism, 230.
   in congenital syphilis, 160, 271.
   meaning of “doubtful,” 360.
   negative in diffuse neurosyphilis, 184.
   negative in juvenile paretic neurosyphilis, 298.
   negative in spinal fluid in spinal syphilis, 148.
   negative in spinal fluid in neurosyphilis, 101.
   negative in neurosyphilis, 252.
   negative in paretic neurosyphilis, 77.
   technique, 476.
   titrations in spinal fluid, 348.

 Wassermann, Neisser and Bruck, 428.

 Weiler, 214.

 Weygandt, 403, 404.

 Widal, Sicard, Ravaut, 428.

 Wiles and Stokes, 186.

 Word-deafness, 35, 43.


 X-ray diagnosis of bone conditions, 136.


 Yerkes-Bridges, 304.


 Ziehen, 409.

 Zsigmondi, 429, 474.

-----

Footnote 1:

  The cases chosen to illustrate the propositions of the boxed headings
  always illustrate several other points. See the footnotes of Section
  VI for lists of cases illustrating special points. The names assigned
  to the cases are fictitious and chosen to suggest race or descent.

Footnote 2:

  Notes of Dr. James J. Putnam.

Footnote 3:

  M = meningeal
  V = vascular
  P = parenchymatous

Footnote 4:

  E. E. Southard: Lesions of the granule layer of the human cerebellum;
  _Journal of Medical Research_, XVI, 1907.

Footnote 5:

  Proof of marked parenchymatous lesions must hang on post mortem data;
  the inference here as to the presence of parenchymatous lesions is a
  clinical inference.

Footnote 6:

  Reprinted from an article by Southard & Solomon: “Latent neurosyphilis
  and the question of _Paresis sine paresi_.” Boston Medical & Surgical
  Journal, XXIV, 1.

Footnote 7:

  Solomon: “How Shall Latent Syphilis be Treated? The Prophylaxis of
  Syphilis of the Central Nervous System.” Interstate Medical Journal,
  XXIII, 8.

Footnote 8:

  Joseph Collins: Syphilis of the Brain, _Journal American Medical
  Association_, July 10, 1915, Vol. LXV, pp. 139–144.

Footnote 9:

  A. M. Barrett has recently discussed this subject in a paper in the
  _Journal of the American Medical Association_, Vol. LXVII, Dec. 2,
  1916.

Footnote 10:

  Reprinted from an article by Southard & Solomon: “Latent neurosyphilis
  and the Question of _Paresis sine paresi_.” Boston Medical and
  Surgical Journal, XXIV, 1.

Footnote 11:

  E. E. Southard. A case of glioma of the pineal region, _Am. Jour. of
  Ins._, Vol. LXI, 1905.

Footnote 12:

  Since this was written Collins has had further difficulties related to
  his neurosyphilis, improving under treatment.

Footnote 13:

  Warthin: “Persistence of active lesions and spirochetes in the tissues
  of clinically inactive or ‘cured’ syphilitics,” _American Journal of
  Medical Sciences_, CLII, 1916.

Footnote 14:

  “The Significance of Changes in Cellular Content of Cerebrospinal
  Fluid in Neurosyphilis,” _Boston Medical and Surgical Journal_,
  CLXXIII, 27.

Footnote 15:

  Plaut: Ueber Halluzinosen der Syphilitiker, Berlin, 1913.

Footnote 16:

  Borden S. Veeder: Hereditary Syphilis in the Light of Recent Clinical
  Studies; Am. Jour. of Med. Sc., CLII, 1916.

Footnote 17:

  Reprinted from article by Southard and Solomon: “Latent Neurosyphilis,
  the Question of _Paresis sine paresi_,” _Boston Medical and Surgical
  Journal_, XXIV, 1.

Footnote 18:

  (This case was furnished by Dr. D. A. Haller from the Peter Bent
  Brigham Hospital series.)

Footnote 19:

  Fernald, W. E. Standardized Fields of Inquiry for Clinical Studies of
  Borderline Defectives. Mental Hygiene, Vol. 1, No. 2, April, 1917.

Footnote 20:

  Goddard, H. H., Feeblemindedness, its Causes and Consequences, 1914.

Footnote 21:

  W. E. Fernald and E. E. Southard. Waverley Research Series in the
  Pathology of the Feebleminded. Proceedings of the American Academy of
  Arts and Sciences, 1917.

Footnote 22:

  Thibierge. La Syphilis dans l’armée, 1917.

Footnote 23:

  Hecht. Wien. klin. Woch., xxix, 51.

Footnote 24:

  Donath. Beiträge zu den Kriegsverletzungen und -er-krankungen des
  Nervensystems. Wiener klin. Wehnschr., No. 27–8, 1915.

Footnote 25:

  Duco et Blum. Guide pratique du Médecin dans les Expertises
  médicolégales militaires. Paris, 1917.

Footnote 26:

  Birnbaum. Kriegsneurosen und -psychosen auf Grund der gegenwärtigen
  Kriegsbeobachtungen: Sammelbericht. Z. f. d. ges. Neurol. u.
  Psychiat., Bd. XII, H. 1, 1915.

Footnote 27:

  Weygandt. Kriegseinflüsse und Psychiatrie. Jahreskurse f. ärztl.
  Fortbildung, Maiheft, 1915.

Footnote 28:

  Bonhoeffer. Erfahrungen über Epilepsie und Verwandtes im Feldzuge.
  Monatschr. f. Psychiat u. Neurol., Bd. 38, H. 1–2, 1915.

Footnote 29:

  Exner, M. J., Prostitution in its relation to the army on the Mexican
  Border, _Social Hygiene_, Vol. 3, 2, April, 1917.

Footnote 30:

  Bonhoeffer, _loc. cit._

Footnote 31:

  We have recently reviewed the outcome in 300 _untreated_ cases of
  paretic neurosyphilis (Psychopathic Hospital material, strictly
  comparable with treated cases) finding but 5 now capable of
  self-support and 10 more in normal-looking remission. This percentage
  is far lower than that in treated cases (at present, July, 1917, 50 in
  200 capable of self-support).

Footnote 32:

  Alice Morton (1).

Footnote 33:

  Francis Garfield (2).

Footnote 34:

  John Dixon (3).

Footnote 35:

  James Pierce (4).

Footnote 36:

  John Lawrence (5).

Footnote 37:

  Flora Black (6).

Footnote 38:

  Mrs. Lecompte (7).

Footnote 39:

  John Wyman (8).

Footnote 40:

  Greeley Harrison (9). _Also_
  Albert Robinson (45),
  Alice Caperson (46),
  Abel Bachmann (74).

Footnote 41:

  Lyman Agnew (10). _Also_
  Ethel Hunter (47),
  Bessie Vogel (52),
  Isaac Thompson (83),
  Juliette Lachine (11).

Footnote 42:

  Juliette Lachine (11). _Also_
  Lyman Agnew (10),
  Ethel Hunter (47),
  Bessie Vogel (52),
  Isaac Thompson (83).

Footnote 43:

  Henry Philipps (12). _Also_
  Bridget Curley (59),
  Margaret O’Brien (68),
  Annie Martin (117).

Footnote 44:

  William Twist (13). _Also_
  Lester Crane (20),
  Thomas Donovan (23).

Footnote 45:

  John Jackson (14). _Also_
  Martha Bartlett (21),
  Paolo Marini (28),
  Margaret O’Brien (68).

Footnote 46:

  Pietro Martiro (15). _Also_
  Meyer Levenson (22),
  Achilles Akropovlos (50).

Footnote 47:

  Joseph Sullivan (16).

Footnote 48:

  Gregorian Petrofski (17). _Also_
  Richard Lawlor (25),
  John Bennett (34),
  Julius Kantor (54),
  Albert Forest (112).

Footnote 49:

  Frederick Wescott (18). _Also_
  Martha Bartlett (21),
  James Burns (56),
  Victor Friedburg (108).

Footnote 50:

  Agnes O’Neil (19). _Also_
  Michael O’Donnell (24).
  John Edwards (104).
  Arthur Bright (121).

Footnote 51:

  Lester Crane (20). _Also_
  Greeley Harrison (9).
  David Borofski (49).
  David Collins (61).

Footnote 52:

  Martha Bartlett (21). _Also_
  Agnes O’Neil (19),
  Vivian Walker (87).

Footnote 53:

  Meyer Levenson (22). _Also_
  Albert Forest (112).

Footnote 54:

  Thomas Donovan (23). _Also_
  William Twist (13),
  Bessie Vogel (52),
  David Collins (61).

Footnote 55:

  Michael O’Donnell (24). _Also_
  Alice Morton (1).

Footnote 56:

  Richard Lawlor (25). _Also_
  Bessie Vogel (52),
  —— —— (88).

Footnote 57:

  John Morrill (26).

Footnote 58:

  David Tannenbaum (27).
  _Also_ Mrs. LeCompte (7),
  Annie Rivers (109).

Footnote 59:

  Paolo Marini (28). _Also_
  Flora Black (6).

Footnote 60:

  Mario Sanzi (29). _Also_
  Stephen Green (30),
  Paul Halleck (31).

Footnote 61:

  Stephen Green (30). _Also_
  Paul Halleck (31),
  Henri Lepère (105),
  Ivan Rokicki (111).

Footnote 62:

  Paul Halleck (31).

Footnote 63:

  Margaret Neal (32).

Footnote 64:

  Joseph Graham (33).

Footnote 65:

  John Bennett (34). _Also_
  Alice Caperson (46),
  Florence Fitzgerald (81),
  Vivian Walker (87),
  Arthur Bright (121).

Footnote 66:

  Mary Coughlin (35).

Footnote 67:

  Theresa Mullen (36). _Also_
  John Lawrence (5),
  John Friedreich (77),
  Gridley Ringer (78),
  James Arnold (80).

Footnote 68:

  Isaac Goldstein (37).

Footnote 69:

  Archibald Sherry (38).

Footnote 70:

  Caroline Davis (39).
  H. F. (40).
  Samuel North (41).
  Elizabeth Brown (42).
  Robert Allen (43).
  John Hughes (44).

Footnote 71:

  Albert Robinson (45). _Also_
  Greeley Harrison (9).

Footnote 72:

  Alice Caperson (46). _Also_
  Florence Fitzgerald (81).

Footnote 73:

  Ethel Hunter (47). _Also_
  Lyman Agnew (10),
  Bessie Vogel (52),
  Juliette Lachine (11).

Footnote 74:

  Milton Safsky (48). _Also_
  Daniel Falvey (55).

Footnote 75:

  David Borofski (49). _Also_
  Lester Crane (20).

Footnote 76:

  Achilles Akropovlos (50).

Footnote 77:

  Daniel Wheelwright (51).

Footnote 78:

  Bessie Vogel (52). _Also_
  Lyman Agnew (10),
  Juliette Lachine (11),
  Ethel Hunter (47).

Footnote 79:

  Carrie Pearson (53).

Footnote 80:

  Julius Kantor (54). _Cf._
  James Burns (56).
  Henri Lepère (105).
  Frederick Stone (106).

Footnote 81:

  Daniel Falvey (55). _Cf._
  Francis Murphy (60).

Footnote 82:

  James Burns (56). _Also_
  Frederick Wescott (18),
  Martha Bartlett (21),
  Victor Friedburg (108).

Footnote 83:

  John Summers (57).

Footnote 84:

  Peter Burkhardt (58).

Footnote 85:

  Bridget Curley (59).

Footnote 86:

  Francis Murphy (60).

Footnote 87:

  David Collins (61).

Footnote 88:

  Joseph Buck (62).

Footnote 89:

  Albert Fielding (63).

Footnote 90:

  Calvin Hall (64).

Footnote 91:

  Donald Barrie (65).

Footnote 92:

  Lawrence Washington (66).

Footnote 93:

  Joseph Temple (67).

Footnote 94:

  Margaret O’Brien (68). _Also_
  Henry Phillips (12).
  Bridget Curley (59).
  Annie Martin (117).

Footnote 95:

  Frank Mason (69).

Footnote 96:

  Annie Kelly (70).
  James Lauder (71).

Footnote 97:

  James Lauder (71).

Footnote 98:

  Margaret Green (72).

Footnote 99:

  Marcus Chatterton (73).

Footnote 100:

  Abel Bachmann (74).

Footnote 101:

  Mrs. Brown (75).

Footnote 102:

  James Seabrook (76).

Footnote 103:

  John Friedreich (77). _Cf._
  Isaac Goldstein (37).

Footnote 104:

  Gridley Ringer (78).

Footnote 105:

  John Doran (79).

Footnote 106:

  James Arnold (80).

Footnote 107:

  Florence Fitzgerald (81). _Also_
  John Bennett (34),
  Alice Caperson (46),
  Vivian Walker (87),
  Arthur Bright (121).

Footnote 108:

  Frederick Estabrook (82).

Footnote 109:

  Maj. Isaac Thompson, M.D. (83).

Footnote 110:

  Lester Smith (84).

Footnote 111:

  Annie Marks (85).

Footnote 112:

  Frank Johnson (86).

Footnote 113:

  Vivian Walker (87).

Footnote 114:

  —— —— (88). _Cf._
  Richard Lawlor (25).
  Bessie Vogel (52).

Footnote 115:

  Margaret Tennyson (89).
  John Lawrence (5).
  Mary Coughlin (35).
  Theresa Mullen (36).
  John Friedreich (77).
  Gridley Ringer (78).
  James Arnold (80).

Footnote 116:

  Joseph O’Hearn (90).

Footnote 117:

  Levi Sussman (91).

Footnote 118:

  Joseph Larkin (92).

Footnote 119:

  Richard Marshall (93).

Footnote 120:

  David Fitzpatrick (94).

Footnote 121:

  Joseph Wilson (95).

Footnote 122:

  Becky Bornstein (96).
  Walter Heinmas (97).
  Mr. Jacobs (98).

Footnote 123:

  Walter Heinmas (97).

Footnote 124:

  Mr. Jacobs (98).

Footnote 125:

  James McDevitt (99).

Footnote 126:

  Jacob Methuen (100).

Footnote 127:

  John Baxter (101).

Footnote 128:

  Theodosia Jewett (102).

Footnote 129:

  A. W. (103).

Footnote 130:

  John Edwards (104). _Cf._
  Henri Lepère (105),
  Frederick Stone (106),
  Arthur Bright (121),
  Agnes O’Neil (19),
  Paolo Marini (28).

Footnote 131:

  Henri Lepère (105). _Cf._
  Julius Kantor (54).

Footnote 132:

  Frederick Stone (106).

Footnote 133:

  Greta Meyer (107). _Cf._
  John Jackson (14).

Footnote 134:

  Victor Friedburg (108).

Footnote 135:

  Annie Rivers (109).

Footnote 136:

  Mr. McKenzie (110). _Cf._
  Ivan Rokicki (111).

Footnote 137:

  Ivan Rokicki (111).

Footnote 138:

  Albert Forest (112). _Cf._
  Gussie Silverman (113),
  Walter Henry (114),
  William Rosetti (116),
  Annie Martin (117),
  Levi Morovitz (122),
  Peter Burkhardt (58).

Footnote 139:

  Gussie Silverman (113).

Footnote 140:

  Walter Henry (114).

Footnote 141:

  Henry Ryan (115).

Footnote 142:

  William Rosetti (116).

Footnote 143:

  Annie Martin (117). _Cf._
  William Roberts (118).

Footnote 144:

  William Roberts (118).
  John Silver (119).

Footnote 145:

  John Silver (119).

Footnote 146:

  James McGinnis (120).

Footnote 147:

  Arthur Bright (121). _Cf._
  Levi Morovitz (122),
  John Bennett (34).

Footnote 148:

  Levi Morovitz (122).

Footnote 149:

  Robert Matthews (23). _Cf._
  Isaac Goldstein (37).

Footnote 150:

  For cases in which, without autopsy we have risked the diagnosis
  neurosyphilis _in the absence of W. R. in serum or fluid_, see William
  Twist (13), Frederick Wescott (18), Martha Bartlett (21), Thomas
  Donovan (23), Paolo Marini (28), Margaret Neal (32), Bridget Curley
  (59), Victor Friedburg (108), Ivan Rokicki (111).

Footnote 151:

  From Mallory and Wright: Manual of Laboratory Technique.

Footnote 152:

  Bruck. Münch. med. Wochen. Jan. 22, 1917.

Footnote 153:

  Smith and Solomon. Boston Medical and Surgical Jour.

Footnote 154:

  Bruck: Journal of American Medical Association, Vol. lviii, No. 12,
  March 24, 1917, p. 944.

------------------------------------------------------------------------




                          TRANSCRIBER’S NOTES


 1. Pg. 456, added footnote anchor for footnote A.
 2. Silently corrected typographical errors and variations in spelling.
 3. Archaic, non-standard, and uncertain spellings retained as printed.
 4. Footnotes were re-indexed using numbers and collected together at
      the end of the last chapter.
 5. Enclosed italics font in _underscores_.
 6. Enclosed bold font in =equals=.
 7. Subscripts are denoted by an underscore before a series of
      subscripted characters enclosed in curly braces, e.g. H_{2}O.