Chapter XXI: Section VI: , Neurosyphilis and the War (3)

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.