Chapter XXII: Section VI: , Neurosyphilis and the War (4)

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”).