Chapter XIX: Section VI: , Neurosyphilis and the War (1)

=Traumatic exacerbation(?) in PARETIC NEUROSYPHILIS (“general
paresis”).=

=Case 92.= The case of Joseph Larkin was of note from the point of view of the Industrial Accident Board. This Irish teamster was said to have been injured in his head two or three months before coming up for examination at the age of 45. For a week Larkin had had frontal headaches, had been sleeping poorly, and had been somewhat worried. In fact, he had stopped work. The W. R. of the serum was positive and a diagnosis of PARESIS could be made. The case did not come up for consideration by the Industrial Board until two years after his initial appearance.

The =physical examination= showed irregular pupils, sluggish pupillary reactions, Achilles absent, swaying in the Romberg position, enlargement of the heart to the left, positive W. R. of the blood and of the spinal fluid.

=Mentally=, the patient’s orientation for place was poor and his memory defective. Emotionally he was depressed or apathetic and was apprehensive. His flow of thought was slow, and his insight into his condition poor.

It is interesting that a variety of causes have been assigned in this case for the condition: such as, his work, anemia, unhygienic surroundings, and arteriosclerosis.

This case is not a sharply-defined case of post-traumatic general paresis, since there had undoubtedly been a variety of mental changes before the accident. Accordingly, recovery of damages to a full amount could hardly be expected as in certain cases in which the phenomena of paresis appear only after the trauma.

Post-traumatic cranial gumma—developing 13 months after local injury
of skull.
]

=Trauma: syphilitic lesion of skull at site of injury.=

=Case 93.= The medicolegal interest of Richard Marshall is extreme, as may be seen from the following brief report by the Psychopathic Hospital to the Industrial Board.

“As to the case of Richard Marshall, a patient under the provisions
of the temporary care act from December 1 to December 10, inclusive,
this case has proved unusually interesting in that the patient has
proved to be syphilitic by the Wassermann reaction of the blood.
There is no evidence of syphilis in the examination of the
cerebrospinal fluid. The X-ray examination of the skull, taken in
connection with the Wassermann reaction of the blood, warrants the
diagnosis of syphilitic osteitis of the skull at the site of the old
injury. We regard his present condition as shown by the X-ray as a
syphilitic bone condition predisposed to by the injury. We do not
find that the patient has any features of traumatic neurosis.

“Mentally, having an actual age of 30, patient grades at 11.2 years.
It may be that patient has always been a moron. He has earned about
$8.30 a week.

“We regard the patient as deserving treatment and feel that
responsible parties in the case would do well to have such treatment
instituted.”

The principal symptom of which Marshall complained was headache chiefly felt in the region of the osteitis. There was marked sensitiveness to percussion in this area. It is of course difficult to decide whether the headache was entirely due to the gummatous lesions or whether the trauma had caused contusions of the brain as well. It is also possible that the dura underlying this area was involved.

=OCCUPATION-NEUROSIS in a granite-cutter: SYPHILITIC NEURITIS?=

=Case 94.= David Fitzpatrick was a case referred to the Psychopathic Hospital by the Industrial Accident Board. He was a granite-cutter of 52 years of age, and had begun to complain of pain in the forearm, extending back from the elbow, about six months before admission. It seems that the patient had been growing progressively worse and had thought he would have to quit work because of difficulty in grasping the hammer. A physician had told him that he must stop his work at granite-cutting or else he would entirely lose the use of his arm. He was in point of fact laid off because of slackness of work and had been unable to get work again. The pain in the arm, however, had continued and at times was very severe. Sometimes the pain and the worry led to insomnia. Fitzpatrick wanted the insurance company to pay certain accumulated bills, and maintained that he would be able to do work at $15 a week if work could be found for him. The general situation in this case can be gathered from the following abstract from the report to the Industrial Accident Board.

“Secretary Industrial Accident Board,
“Dear Sir:
“_In re_ David Fitzpatrick

referred to us with a copy of an impartial report filed by the
Massachusetts General Hospital,—we concur with said impartial report
that there is now no evidence of paralysis of the arm. We do not
find that the positive Wassermann reaction, although it indicates a
history of syphilis, has affected the patient other than possibly to
have reduced his general mental capacity. Our special tests yielded
a percentage of 62% of what a patient of his age and station should
possess. There seems, however, to be no connection between this
reduction of mental capacity and the difficulty with the arm. We
cannot connect the history of alcoholism with the arm trouble.

“There is some evidence that other stone workers have at times shown
such effects.

“The patient’s fairly circumstantial account of his difficulty seems
to point to a degree of myalgia or muscular pain in the region of
the forearm when held in a certain position and a feeling of
numbness in the third and fourth fingers. Whether these phenomena
are due to local pressure upon nerves in the upper part of the
forearm due to neuritis, or whether we are dealing with a functional
neuralgic phenomenon is a question.

“We have applied some special tests for faradic sensibility to all
the fingers of both hands and have found that the fingers of the
right hand are still less sensitive than those of the left,
particularly the thumb and the little finger. This test has not yet
been applied in a sufficiently large number of cases to prove any
difficult point, nevertheless the findings are in line with the
patient’s own circumstantial account of former feelings of numbness
in the third and fourth fingers of the right hand.

“Obviously, then, our opinion is that there is still to be found
some effect of the disease, whatever it was, which caused the
patient to knock off work. If we had more experience with such cases
and more data with the new test which we have applied, we should
perhaps be inclined to admit the diagnosis of _occupation neuritis_
and to suppose structural alterations in the nerve trunks
corresponding with the location of the muscular pain and the
anesthesia of fingers and the dulling of electric sense, but in the
present stage of our experience, it is probably wiser to call the
case one of _occupation neurosis_.”

It is clear that the W. R. in this case was of peculiar value in at least partially clearing up the findings, yet it must be remembered that it is a principle of the modern administration of industrial accident boards and similar organizations that it is the employer’s lookout whether the employee has syphilis. Recovery can be made as if the injury were due wholly to an accident. It was not possible however definitely to prove or disprove a relation of syphilis in the form of a syphilitic neuritis to the condition in this case.

The special tests above referred to are the electric sensory threshold tests of E. G. Martin.

=Character change: neurosyphilis.=

=Case 95.= Joseph Wilson offered a very serious social problem. He was the father of two children, and his wife was pregnant at the time of his admission to the Psychopathic Hospital. He was a husky-looking man of 33 years of age, but for the past four years he had been deteriorating in his work; he had been drinking heavily, and finally had stolen to obtain money for liquor. It was on account of his alcoholism and delinquency, which were taken as an indication of change of character, that he was sent to the hospital.

Examination on his arrival disclosed at once that there was more to the case than alcoholism, for the =neurological examination= showed that the pupils were irregular, the right being larger than the left, both reacting sluggishly to light, and there was an inequality in the reaction of the two eyes, the left being better than the right. The tendon reflexes were exaggerated, with ankle clonus on both sides, more marked on the right. There was also a marked speech defect. Otherwise the =physical examination= showed nothing of importance.

The W. R. of the blood and spinal fluid was strongly positive. The globulin test was strongly positive, the albumin was markedly increased, there were 74 cells per cmm., and a gold sol reaction of the “paretic” type.

A formal =mental examination= did not show very much of consequence; his memory showed no marked impairment, he was not deluded or hallucinated, and he had a pretty good insight into his failings. However, he was somewhat childish, and his irritability was quite marked. Were one to rely upon the mental signs alone, it is probable that a diagnosis of chronic alcoholism with deterioration would be made; but in the presence of the physical findings and the laboratory tests, the diagnosis of neurosyphilis had to be given. It is obvious that, while the patient was suffering from a progressive brain disease, and while he did show mental symptoms, there was not sufficient ground on which to commit him, and therefore he had to be turned out into the community. As a matter of fact, he was not prosecuted on account of his theft, because, although legally responsible, it was felt that his disease was at the basis of the character change which had led him into difficulties. Further developments of his relations with society had to be considered, however. It was possible to get him to discontinue the use of alcohol altogether, and for nearly a year he has taken no alcoholic liquor and has been self-supporting. However, his irritability has been very great, making it very difficult for his wife to live with him, and causing his sister to break off all relations with him.

Here, then, is a man with a marked CHARACTER CHANGE as the result of neurosyphilis, so that it is difficult for him to maintain the usual social relations. It does not seem possible to remove him from the community.

1. May one speak of general paresis without mental symptoms? If one
considers general paresis a mental disease, of course it cannot
exist without mental symptoms. However, if one considers the
disease as a chronic syphilitic meningoencephalitis characterized
by its pathological anatomy, then one may readily speak of general
paresis although no real evidence of mental symptoms can be
discovered. It would seem that we must take this attitude with our
present conception of brain localization, for it is easy to
conceive of a general paretic process affecting areas which do not
definitely relate to psychic function. And further, such a process
may exist but not be of such a grade as to cause mental symptoms.

=The neurosyphilitic’s family should not be forgotten in diagnosis
and treatment.=

=Case 96.= The Bornstein family is remarkable. Let us hang the story on Becky, the mother, an Austrian woman of 43 years, who appears to have been perfectly well up to within a year. About a year ago, Mrs. Bornstein began to suffer from severe headaches, which were treated with apparent success by an osteopath: at all events, Mrs. Bornstein recovered therefrom in about six months. However, two months later, she had a convulsion, with foaming at the mouth, blueness of face, and general muscular stiffening. The convulsion lasted for several minutes. Again, a fortnight before admission, the patient had five convulsions of an identical nature in a single night.

Moreover, since the first convulsion, Mrs. Bornstein’s =mental condition= has altered and become variable, so that at times she is excited, at times depressed. She would assert inaccurately that there was some one in the house, and that she had at different times committed crimes of a heinous nature. Now and then she would seem to see moving pictures. Her memory was poor and she seemed to believe that events of five or six years ago had just happened.

The pupils were sluggish, the knee-jerks and ankle-jerks were absent, there was slight ataxia, and there was speech defect. The suspicion of neurosyphilis was so strong that it seemed surprising that the W. R. of the blood serum, even after repeated tests and after the provocative injection of salvarsan, proved negative. However, the spinal fluid yielded a positive W. R., and a gold sol reaction of the “paretic” type, together with 12 cells per cmm., and a marked increase of albumin, with positive globulin. It would seem warrantable to make a diagnosis at least of syphilis of the nervous system in this case, but it is a question whether we should be warranted in making the diagnosis general paresis.

That the diagnosis is doubtful may perhaps be seen from the variety of diagnoses in the rest of the family. In the first place, Mrs. Bornstein’s husband admits syphilitic infection many years before. He states also that his wife after marriage showed signs of syphilis and received some treatment, although limited. It is stated also that the husband himself at this time has a positive W. R. and has stiff pupils and petit mal attacks. The oldest son, 22 years of age, is confined in an institution with juvenile paresis. The second son has recently died at the age of 20 years, receiving a diagnosis of rupture of the aorta. A third son, 19 years of age, has the appearance of having achondroplasia, although the proportions of his limbs do not quite correspond with those of an achondroplast. The fourth son, 17 years of age, is suffering from caries of the spine. A fifth son, 14 years old, is neurotic and has the so-called Olympic forehead. The sixth and last son died shortly after birth of unknown cause.

=Neurosyphilitic’s normal-looking family proved syphilitic.=

=Case 97.= Walter Heinmas was a draughtsman 33 years of age when he was brought to the Psychopathic Hospital suffering from mental disease. This was diagnosed as general paresis, both on account of the clinical symptomatology and on account of the laboratory findings. In fact, it was a case of the classical type with marked euphoria and grandiosity.

As is the routine procedure at the Psychopathic Hospital, in the case of all syphilitic patients, the family was sent for. This consisted of the wife and two daughters, aged 9 and 7 respectively. The patient denied any knowledge of a syphilitic infection. The wife, also, gave no history of any primary, secondary, or tertiary symptoms; there had been no abortions, miscarriages, or stillbirths; both children had been born at term and had been entirely healthy. Examination showed that the mother had no signs referable to syphilis, and that both the children were mentally well endowed, with good physique and showing no stigmata of congenital syphilis. Still the W. R. of all three (the mother and the two children) was positive in the blood serum. These tests were repeated several times on the children, with and without injections of salvarsan, and they remained consistently positive.

1. Are these children to be considered congenital syphilitics
despite the absence of stigmata or symptoms? We must consider
these children as congenital syphilitics and candidates for the
group frequently spoken of as _syphilitis hereditaria tarda_.

2. What is the frequency of syphilitic involvement in the mate and
children of paretics? In our series, we have found that about 15%
of the marriages where one member develops paresis, result in
sterility; that in 18% abortions, miscarriages and stillbirths
occur; and that in 15% positive W. R. is obtained. We have adopted
the motto: “=The families of paretics are the families of
syphilitics.=”

=Neurosyphilis: question of marriage.=

=Case 98.= Mr. Jacobs’ wife was admitted to the hospital with a diagnosis of general paresis. A few weeks after her admission, she died as a result of her disease. According to our routine, her husband and the children were examined for evidences of syphilis.

Mr. Jacobs’ blood serum was found on repeated tests to be positive. He resolutely denied any knowledge of a syphilitic involvement, but it was later learned from his brother that about two years before his marriage—that is, more than 25 years before we saw him—he had acquired syphilis and had had a very small amount of treatment.

Mr. Jacobs was put upon antisyphilitic treatment in the form of injections of .3 gram of salvarsan every two weeks with occasional intramuscular injections of mercury salicylate. After seven months of treatment, the blood serum still remained positive. At about this time, the patient came to us to ask about getting married again. He said that he was living with his sister, who kept telling him that he was the cause of his wife’s death, and this was so unpleasant that he desired to start a home for himself again!

1. What advice should be given? It is a general opinion that the
longer the period after the initial infection, the less the
chances of infecting a partner. This chance is further reduced
under antisyphilitic treatment, of which a considerable amount had
been given in the case of Mr. Jacobs. However, when one considers
the trickiness of syphilis and the fact that there is some chance
of infection, which we would apparently overlook if we gave him
permission to marry at this time, the only possible course was to
tell the patient that he should not consider marriage until his
Wassermann had become negative and remained so for some time. The
children in this case were negative.

2. What is the physician’s duty to the family of a syphilitic
patient? It is our firm conviction that it is the duty of every
physician to his syphilitic patient, to the patient’s family, and
to the community, to examine the mate and the children for
evidence of syphilis acquired or congenital and to offer treatment
if it is found to be needed. This is one of the chief means at our
disposal today to prevent the late disasters of syphilis, acquired
or congenital, for by such examinations the syphilitic condition
is discovered before lesions have occurred which are irreparable.
We know that the mate and children of a syphilitic patient have
been exposed to syphilitic involvement, and it is our duty as
physicians in possession of such knowledge, and as guardians of
the public health, to investigate such cases, so that if they be
found to have syphilis, steps may be taken to treat them early.

3. How much danger is there of causing unhappiness and breaking up
families by this procedure? This question offers a chance for many
theoretical answers. The facts are, however, that in doing this as
a routine for nearly three years and examining several hundred
families, there has been no instance to our knowledge in which a
family has been broken up or grave difficulties have been
encountered by this procedure.

4. In what percentage are the mates or children of neurosyphilitics
found to show definite symptoms of syphilis? It is our opinion
that the situation in regard to neurosyphilitics is the same as
for syphilitics in general: That the same laws of attenuation of
virus, and of chance occur here as elsewhere.

Just as this book is going to press, we have learned that the distraught Mr. Jacobs, still desirous of starting a home for himself and feeling entirely well, consulted a physician. This physician took a sample of blood and had it tested at a competent laboratory, which reported the blood negative.

On the strength of this test, the physician felt himself warranted in recommending, or at least not advising against, Mr. Jacobs’ marriage, which has probably now taken place.

Although there is some doubt what ethical relation a state institution shall maintain with physicians in private practice, we took occasion to call the attention of our patient’s new counsellor to the fact of the patient’s neurosyphilis. We noted that the man’s serum had been constantly positive (Massachusetts State Board of Health findings) in a score or more of observations. We called attention to the fact that lumbar puncture had shown positive signs of neurosyphilis, including a positive W. R., globulin, excess albumin, pleocytosis, and positive gold sol. These facts, according to a letter received from the private practitioner in question, have not altered his opinion in the slightest to the effect that our patient is completely normal and entirely suitable for marriage. It is clear that he regards the patient as not a victim of General Paresis.

5. What is the significance o the negative observation eventually
obtained in Jacobs’ serum? One’s first thought is to impugn the
accuracy of the laboratory work, but against this suspicion is the
excellent reputation of the laboratory in question, and the
agreement in the majority of its findings with those of the State
Board of Health. It is more likely, as we assured the private
practitioner at whose request the observation was made, that this
negative test was an exceptional and isolated observation such as
is not infrequent in long series of observations, particularly
those made under therapeutic conditions. In so important a matter,
we are inclined to feel that the physician in question should have
resorted to two more observations at intervals before running
counter to the position taken by the hospital.

——many a hard assay
Of dangers, and adversities, and pains.

Paradise Regained, Book IV, lines 478–479.

V. SOME RESULTS OF TREATMENT

Cases 99–103 show the Variety of Structural Lesions that Treatment has to face.

=SPASTIC HEMIPLEGIA in PARETIC NEUROSYPHILIS (“general paresis”),
showing marked degenerative changes, a condition in which therapy
could be theoretically of very little avail. Autopsy.=

=Case 99.= James McDevitt arrived at the Danvers Hospital, July 20, 1906 (saying that he came to be “thawed out”), and died less than six months later: January 12, 1907. He was 34 years of age. He had been a shoe-worker after leaving school, had worked eight years with the General Electric Co., and had then become a bartender. He had, however, stopped work in September, 1905, and we may safely say that mental symptoms had begun insidiously at about that time. His symptoms, if there were any, had been masked by a heavy alcoholism, but an obvious change had appeared in November, 1905. The patient lost ambition, smoked and loafed about his room, and developed speech disorder. He denied venereal disease, nor was there any superficial evidence of such.

=Physically=, the patient showed little or no disorder except acne of the trunk, patches of eczema on the left lower chest, and numerous brownish scars along both tibiae.

=Neurologically=, the Romberg position was maintained, but the gait was very unsteady on attempts to walk a straight line; fingers, tongue, and face were tremulous, and finer movements were performed with marked incoördination. No direct or consensual light reactions could be obtained in the pupils, which were dilated and irregular.

The condition of the reflexes is important on account of the autopsy findings. The abdominal and cremasteric reflexes were prompt, and the knee-jerks equal and very lively. Achilles and normal plantar reactions were present; there was no clonus; the arm reflexes were very brisk.

=COMMON THERAPEUTIC CONCEPTION=

[M]VP = TYPICAL PARESIS

MV[P] = TYPICAL CEREBROSPINAL SYPHILIS

[M]V[P] = TYPICAL SYPHILITIC ARTERIOSCLEROSIS

(M = Membranes, V = Vessels,
P = Parenchyma, [] = not involved)

CHART 21

The =mental symptoms= need not detain us. Consciousness was clear; orientation for time, place, and to some extent for persons, was imperfect. Arithmetic had been largely forgotten. Handwriting was irregular and scrawling, and in places unintelligible. Although the patient claimed that his memory was intact, it was decidedly imperfect. He remarked that John D. Rockefeller, a Chicago king, was President; the General Electric Works had almost 50,000 people at work; and in fact Lynn was one of the largest cities in the state, having over 12,000 people. The height of patient’s room was estimated at 25 feet. There was a slight euphoria. There was never any doubt of the =diagnosis= of PARETIC NEUROSYPHILIS (“general paresis”).

Five months after admission, slight convulsions developed, after which the patient was more dull and demented; he became bedridden. More convulsions followed, leaving the right arm and hand useless. There were clonic spasms of the muscles of both lower legs. Decubitus developed and death occurred.

We may set the total duration of symptoms in the case of James McDevitt at a little over a year; nor is there any evidence of previous or prodromal symptoms beyond a total period of about 15 months, unless we may regard his leaving the General Electric Works to become a bartender some nine years before death, as a symptomatic change of character. In any event, it is of note that the =autopsy= showed singularly few lesions. Death was due doubtless to complications following decubitus, and there was a slight acute splenitis. The kidneys showed some parenchymal change. The aorta showed many patches of sclerosis, with calcification or ulceration throughout its length. These changes were not characteristic of syphilitic disease. There was considerable coronary arteriosclerosis and a slight mitral valvular sclerosis. There was a brown atrophy of the heart muscle, somewhat surprising in a man of 34 years. The =brain= was practically normal, weighed 1200 grams, and showed convolutions normal in size, relation, and arrangement. There was no sclerosis grossly evident in the blood vessels. The pia mater appeared to contain a considerable excess of clear fluid. The calvarium was of normal thickness and showed diploë and the dura mater failed to show adhesions. There were no macroscopic signs of lesion in the spinal cord.

=Microscopically=, the lymphocytosis, plasmocytosis, and phagocytosis of the perivascular spaces, (relative?) increase in blood vessels, the gliosis, and evidence of nerve cell destruction, taken together warranted the diagnosis of PARETIC NEUROSYPHILIS. It was plain that the nerve cell destruction was best marked in the _inner layers of the cortex_. The microscopic study of the spinal cord showed that there was very possibly a slight sclerosis of the posterior columns in the lumbar region, but this was so slight that it could hardly be noted in the myelin sheath stains (Weigert). Very sharply marked, on the other hand, were the _bilateral pyramidal tract lesions_ in the lumbar and thoracic regions, less marked at the cervical levels.

Without attempting to analyze carefully all these findings, it is interesting to note in this case a foil to the usual spinal cord picture of paretic neurosyphilis. The spinal cord, ordinarily normal, or perhaps more usually affected by a degree of posterior column sclerosis, in this case showed such well marked pyramidal tract sclerosis that we may perhaps place the case in a subordinate group of SPASTIC PARETIC cases of NEUROSYPHILIS. The source of the pyramidal tract disease lodges, however, in the cortex cerebri itself, being part and parcel of the lesions mentioned above as affecting more directly the inner layers of the cortex. Many of the so-called giant, or Betz, cells had undergone a complete destruction. It will be remembered that clonic spasms of the muscles of the legs appeared in the fortnight preceding death, and that there had been convulsions for about six weeks before death. There was no evidence at the autopsy why the right arm and hand should have become useless, whereas the left upper extremity remained normal. This case, then, forms an exception to the ordinary paretic neurosyphilis group in that the brunt of the microscopic process was borne by the inner layers of the cortex. The cells of origin of the pyramidal tract fibres had been cut in this lesion, and had become subject to partial or complete destruction. Note, however, that the lesion remained a microscopic one and that the marked convulsions were not related to gross lesions, thereby following the rule for paretic seizures.

Bilateral pyramidal tract sclerosis, secondary to destruction of large
motor (Betz) cells of motor (precentral) cerebral cortex—paretic
neurosyphilis.
]

From the standpoint of possible treatment, it is of course true that few organs of the body showed grave lesions save in the calcified and ulcerated aorta, which conceivably might have become quiescent under appropriate treatment. But, although the brain was almost if not quite normal in the gross, and although its membranes showed practically no lesion, treatment would not have been very promising. To be sure, the exudate might have been cleared away if the spirochetes responsible therefor had been destroyed by treatment. Yet the destruction of the giant cells of origin of the pyramidal tract fibres to such an extent as in this case could hardly have been compensated for by any known process. So far as we are aware, the destruction of considerable numbers of the smaller association elements of the brain is subject to the compensation of other elements of the nervous system, which conceivably might be re-educated or newly educated to perform certain processes. The histological picture in a case like that of McDevitt accordingly leads to the hypothesis that so well marked a spastic paresis, even in the presence of otherwise favorable signs, would be of especially baneful portent therapeutically.

=NEUROSYPHILIS with total duration of symptoms twenty-two days. The
comparatively MILD BRAIN LESIONS, INFLAMMATORY AND NOT DEGENERATIVE
in type, suggest the possibility that therapy might have been
successful. Autopsy.=

=Case 100.= Jacob Methuen, 35, was a carpenter from Newfoundland. He was working upon a certain Thursday with his brother, who noticed that Jacob was lifting the tools about in an unusual manner and talking strangely to his fellow workmen. He fell asleep, going home in the street car, and said afterward that he felt dazed and peculiar. He talked all kinds of nonsense to his wife upon arrival. Methuen remained in bed next day, fancying he was going to die, calling his family together, and saying good-bye to them. He remained in bed all through the next day, but on Sunday appeared better,—more active, and in fact quite natural. He slept only an hour Sunday night, calling to his wife that it was time to get up. On Monday he began to be irritable to his wife, and accused her of flirting with his brother and intending to elope with him. He struck his wife several times, and when two brothers came to watch him, accused them both of trying to steal his wife, and struck them. Tuesday he remained in bed until late at night, when he arose and tried to assault the family.

It seems that another brother of the patient had died but eleven days before his admission to the hospital and five days before the onset of Jacob’s symptoms. Since his brother’s death he had been dwelling upon religious matters, and in fact the day after his brother’s death, he waked up during the night, saying that he was too happy to sleep, that he heard the Master’s voice, and at times the devil’s voice; that there was to be a modern miracle and his spiritual life from now on would be different.

Eleven days after admission to the hospital, Methuen died, making a total duration of symptoms, beginning at his brother’s death, of 22 days.

=NEUROSYPHILITIC LESIONS=

=LESIONS OF THE SECONDARY PERIOD=

(1) INTERSTITIAL ENCEPHALITIS OR MYELITIS
(“meningitis”)
(2) PARENCHYMATOUS ENCEPHALITIS OR MYELITIS
(“encephalitis,” “myelitis”)

=LESIONS OF THE TERTIARY PERIOD=

(1) CHRONIC INTERSTITIAL ENCEPHALITIS OR MYELITIS
(“gummatous meningitis”)
(2) CHRONIC PARENCHYMATOUS ENCEPHALITIS
(“dementia paralytica”)
(3) CHRONIC PARENCHYMATOUS MYELITIS
(“tabes dorsalis”)

“We have shown that the central nervous system is affected by
syphilis at the same periods and in the same manner as are other
internal organs. In addition the ‘parasyphilitic’ lesions are also
of a typically syphilitic nature, being directly comparable to the
parenchymatous affections found elsewhere in the body. They are
‘tertiary’ lesions differing only from the so-called ‘gummatous’
processes in the central nervous system in that their localization
is in the parenchyma while that of the latter is in the
interstitial tissues.”

McIntosh and Fildes, 1914

CHART 22

=Physical examination= showed a man 5′ 9″ tall, weighing 149 pounds, rather pale and poorly nourished, with a somewhat enlarged heart and no evidence of venereal disease.

=Neurologically= there was a slight facial and digital tremor, but otherwise no symptom or reflex disorder except that the tendon reflexes were generally increased; the knee-jerks especially were very vigorous. There was no speech defect. His handwriting was fairly legible.

The patient was very noisy and uncontrollable, tearing clothing and biting, striking the attendants, refusing food, talking rapidly, loudly, and incoherently. His manner suggested auditory hallucinations but no positive evidence of these was obtained. His clothes could not be kept on him. The following is a sample of his reactions: As the examiner entered, the patient stood stark naked and glaring. He started to talk as follows: “Methuen,—I, Saviour, come to life and ought to die—— Now I lay me—— Now I die—— The heart beats—— No, I ain’t going to die—— I am going out soon. I want my clothes—— You can’t hold me; I am strong.” (Struggles violently with the attendants.) “I am God. God. I know you, you can’t fool me.——I am here——I can do you all. How many doctors are there here?” (Struggles violently. Looks at examiner.) “He is writing something. Sir, you can’t fool me in a million years. Do you understand that, doctor? You can’t fool me. Write all the prescriptions you want to. Ten thousand years; you hear that, doctor? Ten thousand years. You can’t fool me; ten thousand years. Ten thousand years are but a day for the spirit of the Lord,” etc., etc.

The excitement continued unabated. The patient became entirely disoriented, and finally almost unable to move. He lay in bed trying to talk and muttering broken gibberish, still attempting to struggle to the extent of his limited strength.

The =autopsy= showed no sign of lesion (brain weight 1380 grams), unless, perhaps, the occipital regions were slightly firmer than the rest of the brain. Death was apparently due to a bilateral pneumonia, bronchial type. There was an acute splenitis. The only chronic lesions of the body were a bilateral chronic adhesive pleuritis and a slight sclerosis of the arch of the aorta.

Paretic neurosyphilis (“general paresis”) macroscopically normal,
microscopically characteristic. Treatment does not have to face
massive destructive processes already complete.
]

=Microscopically= there was a distinct though mild degree of lymphocytosis of the perivascular spaces in many regions. Somewhat extended _search failed to reveal plasma cells_, and it is certain that if plasma cells existed, they must have occurred in very small numbers.

Here, then, was a case of DIFFUSE NEUROSYPHILIS (with brain picture consistent) with symptoms lasting but 22 days and with an appearance of acute mania. It is to be noted that this case arrived at the hospital on the eleventh day of his symptoms. The case occurred long before the development of the temporary care system in Massachusetts. It is probable, or at any rate possible, that he would have been brought to the hospital far earlier, say, upon the sixth day, had the modern temporary care system been installed at that time. The routine W. R. examination would then have been made. With more effective hydrotherapy, it is possible that the patient’s life might have been prolonged and that treatment might have been effective. So far as we can see, the case would have been a singularly good one for treatment despite the practical unmanageability of the case under ordinary home treatment, and even under hospital conditions where modern hydrotherapeutic appliances are not available.

=PARETIC NEUROSYPHILIS showing very MARKED MENINGITIS, suggesting
that therapy might have produced improvement. Autopsy.=

=Case 101.= We report the case of John Baxter, a boat tender of 48 years, because this particular victim of PARETIC NEUROSYPHILIS seems to have had the most markedly thickened and altered meninges in our whole series. Of course, the therapeutic theory upon which we now proceed in the treatment of non-paretic and possibly even of paretic neurosyphilis is that, other things being equal, the meningitis can be removed by treatment, or in the course of treatment, so that the degree of ultimate recovery rather depends upon the condition of the brain substance itself than upon the condition of the meninges. Here, at all events, is an example of the most highly meningitic neurosyphilis that we have seen.

Curiously enough, two of Baxter’s brothers were also patients at the hospital at which Baxter died, and a number of the other members of the family are reported as “nervous.” It seems that at 35 Baxter began to drink heavily and had never given over the habit of alcoholism.

Upon admission to the hospital, in fact, he showed a sufficiently typical picture of delirium tremens. His consciousness was clouded, he had vivid visual hallucinations and was very apprehensive.

His heart was enlarged to the left; the pulse, 120, was of increased tension and irregular; there was peripheral arteriosclerosis; the teeth were poor; the tongue coated; and the mouth foul. The urine showed a trace of albumin and rare hyalin casts.

=Neurologically=, the gait was somewhat unsteady, there was an extreme tremor of the whole body, including the tongue and fingers. The Romberg sign was negative although there was marked swaying. The pupils were equal and reacted normally; the knee-jerks were markedly exaggerated, the arm reflexes somewhat exaggerated. The remainder of the reflexes upon systematic examination were negative.

A high degree of chronic leptomeningitis. Pia mater thick, opaque,
concealing brain. In paretic neurosyphilis (“general paresis”).
]

Upon arrival, Baxter was put to bed, but he barricaded his door and fought with the attendants. The tremor increased, the hallucinations were both visual and auditory. After a few days, Baxter became so weak that he could not move. He refused to eat for a period of two days, explaining in whispers that he did not wish to be poisoned; a voice had told him the food was to be poisoned. The voice was of agreeable tones, probably belonging to a lady; it did not speak, but sang to him. The clouding of consciousness failed to clear up, as in delirium tremens, so that, though patient was admitted March 3d, it was hardly possible to speak freely with him until more than a month later, April 9th. A good-natured conversation would run as follows:

“What is your name?” “Baxter.” “First name?” After long pause, “Don’t know.” “John?” Pause of 7 seconds, “Yes, I think it is.” “How old are you?” “There are legs——there is a body——up to here——” “Say the alphabet.” Term not understood. “Say the _a_, _b_, “Oh yes; a, b, c, d (long pause), e, f; I cannot _c_.” say it, I did not have much education; I am not intelligent.” (In point of fact, the patient had a good grammar-school education, and had long worked as a clerk in a grocery store, with good wages.) There was some speech defect.

Soon the hallucinatory phase passed, and the patient remained in a cloudy and disoriented state, inaccessible, rarely speaking, and gradually failing physically. Death occurred about three months after admission (pulmonary symptoms).

In estimating the duration of the process in John Baxter, we must take into account that he left the grocery business and became a hard-working but poorly-paid boat tender at about 35 years, at the same time that the alcoholic habit began.

The =autopsy= showed that death was due to bronchopneumonia with pleurisy. There were in the body a variety of chronic lesions, such as gastritis, colitis, epididymitis, splenitis, parietal and valvular endocarditis, prostatitis, chronic appendicitis, and some mesenteric lymphnoditis. The heart was somewhat hypertrophied. There was a slight diffuse nephritis with cysts, emaciation, and decubitus. The calvarium was thick and somewhat dense. The dura was thickened and adherent, and the pia mater,—as above stated, the most thickened and altered pia mater in our series,—is described as everywhere thickened, of a brownish gray and white color, especially over the vascular lines, and as showing small white areas of deeper thickening scattered over the surface, but most markedly over the sulci, and not as a rule over the crowns of the gyri. There were also yellowish brown spots with a suggestion of fibrin over the lateral aspects of both hemispheres. The vessels at the base were not remarkable in the gross. The brain weighed 1220 grams, and appeared to be of darker color than usual.

=Some cases of PARETIC NEUROSYPHILIS (“general paresis”) have so
much BRAIN ATROPHY that it is not possible to expect much
improvement through antisyphilitic therapy.=

=Case 102.= Theodosia Jewett, dead at 58 years, showed the most remarkably wasted brain in a long series of victims of paretic neurosyphilis. We present her case to emphasize what therapy must face in certain instances, but would recall the fact that exceedingly few such wasted brains have come to our attention in cases dying in the institutions of Massachusetts.

Mrs. Jewett, a housewife, whose parents died of shock, and one of whose two brothers also died of shock, was a normal child and schoolgirl, and worked as dressmaker until she was married, at 24, to a grocer, by whom she had two children. At the age of 46, Mrs. Jewett began to suffer from so-called “nervous prostration.” The attack lasted some two years, but there were no psychotic symptoms beyond worry and insomnia. The menopause occurred at 52, at which time the first signs of psychosis appeared, namely, a forgetfulness concerning familiar matters, such as sewing, cooking, and the like. At 55, this amnesia had become so marked that Mrs. Jewett could neither write nor tell time. She, however, was a perfectly quiet and easily manageable patient, often subject to drowsiness in the day.

Six months before her admission to the hospital, she began to suffer from insomnia, failed to recognize her surroundings, and had a number of crying spells. Restlessness had begun a month before admission; auditory hallucinations developed in the form of imaginary conversations with dead persons. A certain loquacity set in, and for a week before admission, Mrs. Jewett became somewhat resistive.

=Physically=, the patient was sallow, poorly nourished, with pale mucous membranes, peripheral arteriosclerosis, no teeth, muscular feebleness, tremor of hands and tongue, and active knee-jerks. =Mentally=, the patient was depressed, talked to herself, assumed a supplicating position, suddenly altered her attitude, and was very tremulous. Her talk was low, mumbling, and incoherent, for the most part composed of answers to her own questions. Sometimes there was a curious difficulty in speaking, such that the lips moved but no sound emerged; but for the most part there was no difficulty in uttering words. The patient either could or would not write. Only when the attention was secured by speaking to her sharply was she apparently able to understand questions, and the answers to these sharp questions came spasmodically and as if interrupting her own thoughts. Nor was it ever possible to obtain a repetition of the same answer.

The patient died in exhaustion, with pulmonary symptoms three weeks after admission.

The =autopsy= which was performed 3½ hours after death showed the following points of interest:

The heart weighed 210 grams. There was marked thickening of the aortic valve. The coronaries were slightly thickened.

The lungs were slightly adherent to the chest wall at the apices and posteriorly. The right lung was consolidated in the lower two lobes posteriorly and the bronchi exuded pus; the left lung was not remarkable. There was a chronic splenitis.

The liver showed fibrous changes, was a brownish-red in color, mottled with yellow.

Combined weight of the kidneys 195 grams. The capsules were adherent, tearing the cortex when stripped.

The diploë were well marked. The dura was not adherent. The pia was slightly thickened and raised from the cortex by a large amount of subpial fluid (showing atrophy of the cortex). The pial vessels were injected, more markedly so on the left side. The arachnoid villi were reported as moderately developed, especially along the longitudinal fissure.

The brain was rather soft in all regions. The weight was 1045 grams. According to Tigges’ formula the weight of the brain should be approximately 8 times the body length in centimeters. The length in this case was 158 cm., therefore, according to this formula the weight of the brain should have been 1464 grams. The difference of more than 400 grams is evidently a loss to be accounted for by atrophy, a very heavy loss.

Perivascular exudate (low power) in atrophic cortex from case of
general paresis.
]

Markedly atrophic cortex, but without local perivascular exudate.
]

1. Was the “nervous prostration” at 46 of syphilitic origin? One
cannot give a categorical answer to this question. The high
incidence of shock in the family suggests poor stock in which a
psychoneurosis is not an unusual phenomenon. The presence of
syphilis might act as a debilitating factor or _agent
provocateur_, if it were not to cause any demonstrable brain
lesion. As pointed out in the case of Harrison (9), however, it is
not unusual in neurosyphilis to find a history of symptoms
occurring years before the final breakdown and symptoms frequently
not recognized as of neurosyphilitic nature.

2. Does the fairly long duration of the psychosis (at least 3 years)
explain the marked atrophy? Cases having symptoms even much longer
than three years at times show relatively very little atrophy, so
that this factor in itself cannot be said to explain the
tremendous destruction of tissue.

=The THERAPY OF NEUROSYPHILIS has to face not merely variations in
the degree of brain wasting and in the degree of meningitis, but
also variations in the topographical distribution of lesions.
Autopsy.=

=Case 103.= To bring out this point we may instance the case of Alfred Weed, a victim of PARETIC NEUROSYPHILIS, dying at the age of 48 years after a course of about seven years. The following is an abstract of the clinical history:

A. W. suffered from lues some 24 years before his death at Danvers Insane Hospital in 1907. There is no account of insanity in his family. The patient had been undergoing mental changes for six years before death. At the age of 42 began to take interest in socialism and spiritualism. Would become excited at times and was observed to talk to himself. At times it seemed that he was reacting to visual hallucinations. After eight months he became depressed and apprehensive and developed delusions of poisoning.

On admission to the Danvers Insane Hospital in June, 1902, the subject was found to be ataxic, falling in the Romberg position. Pupils were equal but of pin-point size. There was tremor of the facial muscles. The knee-jerks were absent. Speech was ataxic. Memory defective. Depressed. Thought he was to be punished. Refused to eat.

Later in the year of admission, patient became more negativistic. He refused to have his clothes brushed. His answers were slow. Mental arithmetic was correctly but slowly done. During January, 1903, the patient was apt to be active and talkative for a time, and then his attitude would suddenly change to one of silence, resistivement and untidiness. From time to time he would be querulous and sulky. In August, 1903, the patient became weaker and could walk with assistance only. Paralysis developed in the left facialis region and in the left external rectus. Pupils were still small, but the left had become smaller than the right. Light reaction tests unsatisfactory. Knee-jerks could not be obtained.

In December, 1903, the patient was untidy and helpless, lying with his thighs and legs flexed. The limbs were spastic on passive motion. In 1905, the pain sense of the legs was found lost and the pupils were small and stiff. The protruded tongue was deflected to the right. The right labial fold was more prominent than the left. Knee-jerks remained absent. Ataxia was extreme.

The =Neurological Findings= may be summed up as follows:

1. Ataxia of the legs.

2. (Probable) Diminished sensibility in the legs.

3. Pupils small and stiff. Left smaller than the right.

4. Paralysis of left facialis.

5. Paralysis of left external rectus.

6. Tongue protruded to right.

7. Right elbow jerk greater than left.

8. Knee-jerks absent.

The cause of death was bronchopneumonia. The walls and valves of the heart showed a few chronic changes. There was a marked splenitis and an atrophy of the liver. The kidneys showed numerous depressed scars. The arch of the aorta was somewhat sclerotic. The following is a full description of the head findings which we present by way of comparison with other cases. Note especially the cerebellar, dentate, and olivary changes. Note also the fact that palpable sclerosis is demonstrable over a far larger area than atrophy, so that we may almost safely conclude that the process of induration sometimes precedes that of atrophy. One gets the impression from the extent of visible atrophy and tangible induration in this case, that a possible therapy would have not merely to clear the perivascular spaces of cells and spirochetes, but would also need to arrest the indurating and wasting process. Nor could any therapy deal effectively with the superior frontal and upper central atrophy of the cerebrum of this case, or with the olivary and cerebellar lesions.

=Head=: Hair thin at vertex. Scalp normal. Calvarium thin and dense. Dura mater slightly adherent to calvarium at vertex. Sinuses normal. Arachnoidal villi well developed. Pia mater of anterior and central regions contains an excess of fluid. The pial veins well injected.

The =pia mater= exhibits one unusual lesion: Faintly yellowish brown spots of miliary and slightly larger size are scattered irregularly in clusters over the vertex. These miliary pial macules are observed especially over the posterior third of the left superior frontal gyrus (a group of twelve or more). Two are seen in the pia mater of the right superior frontal gyrus. One is seen in the upper part of the left post central gyrus. The upper end of the right postcentral gyrus contains three macules.

Besides these brownish macules, the pia mater also shows focal white thickenings which resemble the more frequent appearances of chronic fibrous leptomeningitis. The white thickenings are of irregular size but are, as a rule, larger than the macules above mentioned. They occur, as a rule, over the sulcal veins and are most frequent in the anterior region.

The vessels at the base are normal. There is no evidence of pial thickening at the base of the brain. =Brain= weight, 1265 grams. There is visible atrophy of both superior frontal gyri and of the upper two-thirds of both central gyri. The extent of palpable sclerosis surpasses that of visible atrophy. Palpable increase of consistence is shown by the prefrontal, orbital (more marked on left side), frontal, central, hippocampal and occipital regions. The temporal cortex is of normal or slightly reduced consistence.

Section of the cerebral cortex shows everywhere preservation of the cortical markings. The sclerosed areas show a diminution in depth of the cortex, which is more marked in the left prefrontal region. The white matter of the centrum semiovale of the prefrontal and occipital regions on both sides shows an increase of consistence. The cerebellar cortex also shows variations in consistence. The clivus and lobus cacuminis and the posterior half of the inferior surfaces of both cerebellar hemispheres are firmer than normal. The laminæ of the left clivus are a trifle narrower than those of the right. There is visible extensive atrophy of the laminæ on both sides of a fissure in the middle of the left lobus cacuminis. In the coördinate portion of the right cacumen there is a similar process which is less marked. The dentate nuclei are firm. The olives show an increase of consistence, equal on both sides. The left olive shows on section a crowding together of its folds in the middle part of the upper limb.

Spinal cord was not remarkable.

=Summary=:

Adhesive pachymeningitis Chronic fibrous leptomeningitis Miliary pial macules Cerebral atrophy Cerebral sclerosis Cerebellar atrophy and sclerosis Bronchopneumonia Chronic splenitis Nephritis Aortitis

=It is generally recognized that DIFFUSE NEUROSYPHILIS
(“cerebrospinal syphilis”) frequently is cured through
antisyphilitic therapy. Example. Mental improvement, in one month;
recovery from paralysis, ten months.=

=Case 104.= John Edwards, a man of 28 years, well developed and nourished, with general enlargement of glands and skin lesions, came to the hospital in a stuporous condition, with evidences of a complete hemiplegia.

According to the wife, Edwards had had a chancre of the lip about a year before, for which he had been treated with an intravenous injection, presumably of salvarsan, and also presumably with mercury. The lip lesion had then disappeared. For a month before admission, Edwards had had headache and dizziness, for which he was given pills and drugs. There had also been difficulty with speech and numbness of the left arm as far up as the elbow, but this paresthesia had quickly disappeared. The hemiplegia was of only a few days’ duration. After a feeling of nausea and vomiting, the patient had fallen with left-sided paralysis. Afterwards, he had shown mental peculiarities, eventually becoming noisy, hard to manage, and appropriate for hospital care.

The =physical examination= showed a variety of increased reflexes, including ankle clonus on the left side.