2. What is the prognosis? The prognosis of juvenile paresis is

currently regarded as entirely grave. There is probably less hope

for improvement in juvenile paresis than in the acquired paresis

of adult life, since it seems to be a general principle that

congenital syphilis is always more difficult to cure than acquired

syphilis.

This case had seizures a few months after initial observation, and

the seizures were followed by a transient right hemiplegia. This

right hemiplegia was shortly followed by a left hemiplegia, which

remained permanently. Moreover, a few weeks later, a right

hemiplegia again developed, leaving the patient with complete

paralysis and aphasia. Death followed in six weeks.

3. What effects were shown in the parents? Following up the parents

was rewarded by the discovery that the mother was suffering from

nerve deafness, probably of syphilitic origin, and that the father

had recently begun to suffer from what he considered rheumatism,

but which on examination was shown to be tabetic neurosyphilis

(“tabes dorsalis”). This family again supports the hypothesis that

there is a strain of spirochetes especially prone to attack the

nervous system. Here it would seem that the syphilis acquired by

the father had infected the mother and been transmitted to the

son. In all three infected by the same strain or strains of

organisms the nervous system was involved. It is difficult,

nevertheless, to explain on this hypothesis why in one case the

disease took the form of tabes dorsalis, in the second, eighth

nerve involvement and in the third, paresis. This question of

strains is really more than academic because it enters deeply into

the question of treatment, as well as that of the suggested

increased viability of the neural strain.

=Is there a relation between epilepsy and juvenile neurosyphilis?=

=Case 79.= John Doran fell off the rear of an ice-wagon, at six years of

age, and shortly afterward developed fits. It appears that John was not

unconscious at the time of his fall, but that he complained of headache.

Although the convulsions were fairly frequent at first, it appears that

they later became rare and occurred only when the patient got into a

temper. At the stage of exhaustion after violent excitement, John would

fall.

=Physically=, at 9 years a fair development and nutrition were evident.

There was a great exaggeration of the frontal bosses; the nose could not

be said to be typically saddlebacked, yet there was a suggestion of a

sinking of the bridge. The teeth slightly suggested the Hutchinsonian

type, but only slightly. There was a slight roughening of the tibia, and

there was a slight scar over either knee. The patient graded according

to the Binet scale at 9 years, and he was regarded as definitely

feebleminded.

The family physician states that, according to his information, the

father contracted syphilis when the child was between three and four

months of age, and that the mother also was infected at this time.

However, the child had not been suckled except immediately after birth,

and there had been no evidences, according to the family physician, that

John had acquired syphilis.

Ordinarily, one might content himself regarding the case of John Doran

as one of idiopathic epilepsy with mental defect or deterioration.

However, the frontal bosses, suggestive teeth, the flattened bridge of

the nose, the roughened tibiae, and the old scars, though singly not of

great significance, collectively make one suspicious. Despite the family

physician’s belief that John could not have acquired syphilis from the

parents, the infection seems entirely possible despite the fact that no

symptoms developed early thereafter.

The W. R. in this case proved positive in both blood serum and spinal

fluid.

1. What is the relation of trauma to this case of JUVENILE

NEUROSYPHILIS? Probably none.

2. What would be the effect of treatment? For a number of years John

Doran was lost sight of. He was, however, treated, according to

our information, with intraspinous injections of salvarsanized

serum, whereupon his convulsions shortly ceased. He has been

recently examined mentally once more, and still grades as

feebleminded. He still has violent outbreaks of temper.

3. Is such a case as Doran typical? Shanahan has investigated

conditions at Craig Colony. There were 22 out of 886 epileptics

(at Craig Colony) or 2½%, who showed a positive W. R. Nine of

these cases were regarded by Shanahan as cases of epilepsy

actually caused by syphilis. Viet had found 7%, and Bratz and Lüth

5% of constitutional epileptics to be syphilitic, but the data of

these German authors were obtained before the era of Wassermann

tests.

=Adrenal tuberculosis complicating juvenile paretic neurosyphilis

(“juvenile paresis”). Autopsy.=

=Case 80.= When James Arnold appeared at the Danvers Hospital in his 22d

year, he looked as if he were but 12 or 14 years of age. He was

excessively fat but of fair muscular development. The left eye diverged

outward, and the left pupil was smaller than the right. An odd feature

was a high degree of pigmentation of the skin of the genitalia and the

groins (the axilla, the mammillary areas, and the oral mucosæ were free

from pigmentation). =Physically= speaking, the patient was practically

normal. =Neurologically=, however, there was much of interest, in the

light of which the clinical history was of value.

It seems that after an apparently normal early childhood, the boy had

begun, at the age of 11, to experience difficulty in carrying out

every-day school tasks; and after this his mental capacity had slowly

but progressively deteriorated. The deterioration was not merely

intellectual, but the boy became dishonest and untrustworthy and

developed a number of untidy and uncleanly habits, behaving at the age

of 16, as the parents stated, like a child of six.

In his seventeenth year, the boy had been taken with a severe attack of

what was regarded as an “attack of indigestion.” This attack ushered in

a gradually developing muscular weakness, especially involving the

limbs. By the age of 21 he had become irritable and the paresis was so

extreme that the patient was unable to get in or out of a carriage.

This generalized muscular weakness was plain upon admission to the

hospital though there seemed to be no actual paralysis. The patient was

unable to walk in a straight line and Romberg’s position could not be

maintained. Marked tremor was present in the hands and lips. There was

bilateral impairment of vision and nystagmus. Reflexes and sensations

normal. Speech was markedly affected, all syllables being very much

slurred. School knowledge and memory for both recent and remote events

very poor. The patient’s habits were very untidy. He was very emotional,

easily made to laugh or cry; and in behavior, extremely childish.

Two months after his admission to the hospital, the weakness suddenly

became extreme. He was constantly nauseated, refusing food. The face and

hands were cyanosed and the heart’s action rapid, weak, and irregular.

This attack lasted for a week and was followed by a period of

improvement, during which, however, he still remained very weak and

apathetic.

One month later he again became so feeble that he was unable to raise

himself in bed. He complained persistently of feeling very “sick.” His

temperature was elevated and there occurred the same train of

circulatory symptoms observed previously, _viz._, rapid and tumultuous

action of the heart, with cyanosis of face and extremities. He soon

became unconscious, remaining so until his death, which occurred on the

seventh day of the acute attack.

This case was under observation before the days of the W. R., yet

clinically the case had been diagnosticated JUVENILE PARESIS. There was

no history of the acquisition of syphilis or any likelihood of its

acquisition. Considered clinically, many of the classical features

described by Addison were present, _viz._, marked asthenia and apathy;

severe and frequent gastro-intestinal symptoms (the disease probably

commencing with the attack of so-called “acute indigestion” six years

prior to patient’s death); attacks of extreme cardiac weakness with the

characteristic small, low-pressure pulse. On the other hand,

pigmentation of the skin (usually the most striking clinical feature)

was limited to the external genitalia, these being colored a deep brown.

The most striking feature found at autopsy was a bilateral adrenal

tuberculosis (caseation, giant cells, lymphocytosis, tubercle bacilli).

The thymus gland was persistent (7×5×.5 cm.), whereas the thyroid gland

was smaller than usual. The brain showed macroscopic and microscopic

features entirely consistent with the diagnosis of general paresis,

including lymphocytosis, plasmocytosis, irregular degrees of nerve cell

destruction, and gliosis, with an especially characteristic microscopic

picture in the frontal regions.

It may be of note to consider the degree of change undergone by a brain

in 11 years or more of deterioration, and the following description of

the head findings is therefore included:

=Head=: Hair abundant, dark. Scalp normal. Calvarium, weight 435

gm., transparent in bregmatic region only, elsewhere thick and

dense. The average thickness of the vertical plate of the frontal

bone is 7 mm. The frontal bone shows a moderate thickening and

hardening of the inner table with obliteration of diploë. Dura mater

moderately adherent to the bregmatic region of calvarium.

Arachnoidal villi moderately developed. Sinuses not remarkable. Pia

mater shows a moderate focal thickening with opacity, especially

along sulci. Vessels well injected. =Brain=: Weight, 1200 gm. The

brain shows marked focal variations in sulcation and consistence.

Spread on a board, the right hemisphere is obviously somewhat bigger

than the left. There is a difference of only 0.5 to 0.75 cm. on

measurement of the greatest circumference of the cerebrum, taken

from the median line superiorly to the median line inferiorly, but

the right hemisphere is throughout slightly more convex than the

left. Both postcentral gyri are much narrowed in their superior

portions, and the sulci posterior thereto are deeper than the other

sulci of the hemispheres. The sulci of the orbital surfaces are

asymmetrical and, on the left side, show a tendency to microgyria.

The cerebral hemispheres as a whole show a remarkable tendency to

slight protrusion of the border gyri; especially those of the two

poles, of the free edges along the great fissure, and most

strikingly the gyri at the boundary line between the inferior and

lateral surfaces. This _marginal prominence_ is slight but obvious

and is emphasized by a slightly paler color in some regions. The

cerebrum shows a general induration which is greatest in the frontal

tips and along the inferior borders of the lateral surfaces of the

hemispheres, especially right. The orbital surfaces are firm,

especially anteriorly and externally (prefrontal); the tips of the

temporal lobes are firm, and the superior temporal gyri are firmer

than adjacent gyri. The postcentral gyri are indurated more than the

other gyri of the superior surface. The hippocampal gyri are

likewise firmer than adjacent gyri.

=Cerebellum and pons=: Weight, 145 gm. The inequality of the two

hemispheres is more marked than in the case of the cerebrum.

Greatest lateral diameter; left, 4.5 cm., right, 5.5 cm.

Anteroposterior diameter adjacent to notch: Left, 5.8 cm., right,

5.5 cm.

There is no appreciable difference in depth. The diminution in

volume appears to be chiefly at the expense of the right clivus. The

inferior surface is firmer than the superior. The laminæ adjacent to

the horizontal fissure are firmer than the remainder of the

cerebellum. The pons is small.

There was also a lateral curvature of the spinal column, as well as

characteristic adhesions between spinal dura and pia mater which are

always suggestive of syphilis. For the rest, there were few findings of

note: some adhesions of the left pleura, hypostatic congestion of the

lungs, tracheitis, and chronic gastritis. There were four lobes of the

right lung but it is doubtful whether this should be regarded as a

stigma.

1. Can we separate the symptoms of Addison’s disease from those of

paresis in this case? The extreme cardiac weakness with a

characteristic, small low-pressure pulse is in point. The asthenia

and apathy are consistent enough with Addison’s disease as well as

with paresis itself. It would also be possible to ascribe the

gastro-intestinal symptoms to either disease.

2. Of what significance is the persistent thymus? Persistent thymus

has been observed in a few cases of Addison’s disease, but that it

plays any part in the symptomatology thereof is a matter of doubt.

3. How can the obesity be explained? It is of course of note that

the thyroid gland was small, but microscopically there were no

peculiar features in this gland.

4. Was the adrenal tuberculosis actually primary? Minute search

failed to reveal evidences of tuberculosis elsewhere unless we

regard the few adhesions binding the lower half of the lung to the

chest wall as indicative of an old tuberculosis. In particular,

the mesenteric lymph nodes were normal.

=Neurosyphilis? Secondary stage of syphilis.=

=Case 81.= Florence Fitzgerald, a woman 25 years of age, applied at the

police station to be taken care of. She said she had been a prostitute

for the last few months, was now ill, and wanted to reform. She appeared

physically ill and was sent to the Psychopathic Hospital, where she

remained at first almost mute, making answers chiefly by nodding the

head. She gave the impression of daze or stupor, and in fact her

condition was at first regarded as catatonic. This reaction, after a few

days, changed and Florence became quite normal, giving a full account of

her condition.

It seems that four months before going to the police station, she

developed a chancre, which was locally treated. A careful physical

examination showed a fine red macular eruption which was without much

question a syphilitic roseola. The spinal fluid yielded a positive W. R.

although other tests of the fluid were negative. Curiously enough, no

physical sign of involvement of the nervous system could be discovered.

We were inclined to regard the mental symptoms as partly due to the

syphilitic intoxication, and partly due to a psychic reaction of the

nature of defense. As for the positive W. R. in the spinal fluid, in

early secondaries various observers differ as to the frequency both of

the W. R. and of other changes, percentages being given that range from

25 to 90%. See case Caperson (45). It is of note that clinically there

were symptoms referable to a syphilitic involvement of the nervous

system; namely, marked headache and malaise. The headaches of the

secondary period are frequently the result of meningeal involvement.

=TABOPARETIC NEUROSYPHILIS (“taboparesis”); death from TYPHOID

MENINGITIS. Autopsy.=

=Case 82.= Frederick Estabrook was a salesman, who, be it noted, had

never had typhoid fever or any disease remotely resembling typhoid

fever. He had acquired syphilis at 19; had married at 22; was the father

of two healthy children (no miscarriages); had had a certain disturbance

of bladder and rectum, but remained a successful salesman to the age of

28, when advancing tabes confined him to bed for a time. At 30, mental

signs of PARETIC NEUROSYPHILIS developed, and death followed at 32,

after an acute illness of a week.

The details of the history after the first symptoms at 28 are as

follows:

At twenty-eight patient lost control of limbs and was confined to the

house about two months, under medical care. Three months later he had

regained partial control of his limbs but had lost all control of his

sphincters. After another month he had returned to work, but did not

work steadily and seemed to have lost ambition. In the summer of 1905,

his mind became obviously altered. He grew indolent and extravagant and

given to buying expensive and useless articles. Loss of interest in

things followed, together with loss of memory for recent events, lack of

insight into illness, delusions of persecution by wife, irascibility

followed quickly by crying. Before admission to hospital, he was

euphoric, drawling and tremulous in speech, sprawling in penmanship,

alternately depressed and exalted in manner. Knee-jerks were absent,

gait ataxic, pupils stiff to light.

The family history was negative with respect to insanity. All the family

were reported as nervous. A brother died of peritonitis at twenty-eight,

a sister of pneumonia under twenty. Another brother and sister are

living. Father and mother died of heart trouble at about sixty-seven and

sixty respectively.

The patient was at high school one year and was a fair student.

Considerable tobacco was used, and some alcohol. Intoxication denied.

There was no history of typhoid fever or other acute disease.

The patient on admission was sallow, poorly nourished, and flat-chested,

with a slight lateral curvature. There was slight dulness over right

apex in front and in right upper back. Voice sounds were increased over

right apex in front and over whole right back. The right chest showed

bronchial respiration throughout. The respiration in front of right

chest was of an interrupted character. The liver seemed moderately

enlarged. The urine showed a very faint trace of albumin. There were a

few small nodes in right groin and a scar on dorsum of penis.

=Neurological Examination.= Slight swaying in Romberg position. Slight

tremor of protruded tongue and extended fingers. Pupils irregular, left

slightly larger than right. Left pupil reacted to light consensually,

but not directly. Right pupil reacted very slightly to direct light, not

consensually. Knee-jerks and Achilles jerks absent. Ankle clonus absent,

abdominal and cremasteric reflexes brisk. Sharp and dull points were

recognized in the legs with numerous mistakes. Vocal and facial tremor.

Speech slow and drawling. Test phrases repeated well if care was taken.

Consciousness clear. Orientation perfect. Calculating ability preserved.

Many words omitted in writing. Penmanship clear but shaky.

Hallucinations absent. Memory of recent events poor. Associations of a

logical or defining type. Patient denied various statements in

commitment papers and had little or no insight into the mental side of

his disease—slight euphoria.

After a month’s observation the patient was removed to a quiet ward and

set to work a few days in the scullery. One night he began to yell as if

assaulted and said later that he had an idea that he was going to die.

Before three months had passed he had become untidy, disorderly, and

imperfectly oriented.

The general degeneration continued rapidly. One week before death the

temperature rose to 103 degrees F., and the patient succumbed to what

seemed clinically like a bronchopneumonia. Unconsciousness two days

before death.

Note with respect to history of typhoid.—Inquiries of his physicians,

wife, employer, and brother tend to show conclusively that the patient

never had a disease even remotely resembling typhoid fever.

The =autopsy= findings were as follows:

Acute conditions:

Hypostatic pneumonia, with early serofibrinous pleuritis and without

lymph node swelling; =enlargement of mesenteric lymph nodes=; =acute

cerebrospinal leptomeningitis=; multiple small hemorrhages of spleen.

Other findings:

=Scar of penis=; =sclerosis of aortic arch= (Heller’s type?) and slight

coronary arteriosclerosis; =calvarium= thin and =dense=; =dura mater

thickened= and adherent to calvarium; calcified arachnoidal villi;

=chronic= cerebral and cerebellar =leptomeningitis=; =atrophy of frontal

lobes=; =granular ependymitis=; =sclerosis of posterior columns= of

spinal cord; emaciation; unequal pupils; slight parietal fibrous

endocarditis, slight mitral sclerosis; gastro-intestinal atrophy;

chronic cystitis; chronic abscess of prostate.

The description of the head findings is as follows:

Skin exceedingly loose, and the whole skull cap thinned. The diploë

are absent. Adhesion with dura easily separated. The dura somewhat

thickened, but not distended. Along the longitudinal sinus extensive

calcareous granulations adhere to it. The longitudinal sinus does

not contain blood, and the inner surface is normal in color. The pia

is extensively thickened and opaque and a general subpial exudate

exists which is more marked over the vertex where it lifts the pia

from the brain surface to the extent of three centimeters in

Rolandic, superior frontal, intraparietal, and mesial precentral

sulci on each side. The arteries at base are free from atheroma. The

temporal lobes are much bound down by adhesions, as is the

cerebellum. Post mortem softening is evident. The hemispheres show

no asymmetry, but the frontal convolutions are markedly atrophic.

The corpus callosum is united to the cortex by old adhesions and has

to be dissected away from it. Lateral ventricles contain some slight

amount of cloudy fluid, and the pia along the vessels is opaque.

Some granulations in ependyma. Brain weight, 1305 grams. Pons and

cerebellum, 195 grams.

Cord.—Dura much thickened, and the pia corresponds to its appearance

in brain with a like exudate. Cross sections of cord show sclerosis

of posterior columns.

Bacteriologically the _typhoid bacillus_ was cultivated _from the

meninges and from the swollen mesenteric lymph nodes_. The blood was

negative; the intestines were negative so far as lesions were

concerned.

The microscopic examination confirmed the clinical diagnosis of GENERAL

PARESIS and of TABES, since there was not only an extensive chronic

encephalitis, with the usual lymphocytic and plasma cell deposit and

irregular gliosis, but also a well marked posterior column sclerosis,

not unusual save in its extreme degree.

It might be surmised that some difficulty would arise in distinguishing

the effects of paretic meningoencephalitis from those of the more recent

typhoidal process. The well-known tendency of typhoidal processes to

escape polynuclear exudation, at least until frank necrosis has set in,

gave rise to the idea that the two mononuclear pictures—that of general

paresis and that of typhoidal processes—might be confusing.

The picture presented by the meninges was scarcely what might be

expected. Although numerous mononuclear phagocytic cells are everywhere

found, yet the predominant picture is that of a polynuclear exudation.

The polynuclear leucocytes occur in greatest numbers in the tissue

spaces, especially in the meshes of the lumbar arachnoid and in the

spaces of the frontal and paracentral pia mater. In the lumbar region of

the spinal arachnoid wide fields occur in which the cells are almost one

hundred per cent polynuclear leucocytes. In places phagocytic cells

occur, and in a few fields, even in the open tissue spaces, the number

of phagocytic cells may arise to fifty per cent. Edema is a considerable

feature in the meninges. Fibrin is found chiefly in the cerebral

meninges and appears in numerous delicate strands in the tissue spaces.

Moloch, horrid king, besmeared with blood

Of human sacrifice, and parents’ tears;

Though, for the noise of drums and timbrels loud,

Their children’s cries unheard that passed through fire

To his grim idol.

Paradise Lost, Book I, lines 392–396

IV. MEDICOLEGAL AND SOCIAL

=Neurosyphilis in a public character: eloquence, reformatory

efforts, notoriety.=

=Case 83.= Major Isaac Thompson, M.D., was a character. He had been

regarded as eccentric for many years prior to his death at 63. In fact,

it seems that there had been more or less definite symptoms and signs

about his fortieth year. The doctor himself had a ready explanation for

his Argyll-Robertson pupils; he explained that he had had a peculiarly

heavy smallpox at about the age of 27 (which would be about 1872).

The doctor had a good secondary education, he had gone through the Civil

War as a hospital steward, went into business after the war, married,

and then went to the medical school, graduating at the age of 34. He

continued in practice for a dozen years, and then gave it up. For years

he had been especially interested in certain literary lines and he had

published any number of pamphlets, all of a somewhat striking

description, often with a political color and intended to stir up reform

measures. The doctor never bore a very good reputation, and years later

it was recalled that certain books disappeared from libraries and their

loss was almost certainly traced to Dr. Thompson. In general, however,

he was considered to be a rather worthy local figure.

It is possible that a fall on the ice in his 61st year actually started

the fatal process, since after that time the patient had difficulty in

walking, and a few months later developed periods of excitement with

peremptory insistence on obedience to his wishes. Whereas formerly the

doctor had finished up one literary piece of work after another, he now

began to do very scattering work. He appeared in public to denounce

certain financial schemes with great force and unusual eloquence. His

eloquence was greatly complimented, and these compliments induced the

doctor to a remarkable crusade against a certain corporation; there was

so much truth mixed with the fiction of his eloquence that he obtained a

considerable following in his campaign. He wanted to start a bureau of

information for the instruction of the public on these matters, and he

planned to put up a building adjoining his own home for the

accommodation of the various clerks and writers in this bureau. However,

before the building had been actually started, an outbreak occurred.

One morning the doctor was very excitable and noisy over the telephone,

ordering typewriters and giving directions to mechanics. He repaired to

Boston in connection with certain resources that he supposed (and gave

others reason to believe) had been supplied by the Government and by a

large newspaper. One evening he returned very late. It appeared that he

had had a fracas at a hotel and had knocked down one or two colored

porters, acting as though drunk. Upon being put to bed, the doctor

talked incessantly of religious matters, proposing to undertake a Sunday

School class. His interlocutor did not exhibit a particular interest in

this scheme, whereupon Dr. Thompson threatened him with violence. Police

and doctors were called in and a constant stream of conversation lasted

for hours. The patient was finally brought to Danvers Hospital upon

representation by physicians, to whom he told that his luck had turned,

that he was about to be made senator from the district, and that he and

Roosevelt were going to break up the trusts, and that, as a matter of

fact, he was a relative of Mr. Roosevelt.

Upon admission, the patient was a well preserved and well groomed man

with gray hair and beard. He was somewhat pallid but his teeth were well

preserved and well cared for, and there was little or no physical change

except a slight hypertension. He claimed that he had suffered from

kidney disease for some years, and there was in fact a trace of albumin

in the urine.

=Neurologically=, the plantar and Achilles reactions could not be

obtained, but there were no other reflex disorders except the bilateral

Argyll-Robertson pupil. The doctor’s explanation for these stiff pupils,

which he described as existing for many years, was frank and

circumstantial, so that the unlikelihood of Argyll-Robertson pupils due

to smallpox was rather frowned upon by him. Without entering upon a

detailed description of the clinical symptoms and course of the disease

which led to death a little over a year after admission, it may be said

that the differential diagnosis lay between the expansive form of

general paresis and a maniacal condition, presumably the maniacal phase

of manic-depressive psychosis. From the data of a special staff meeting

held upon the case, we learn that the diagnosis of manic-depressive

psychosis was entertained more strongly than that of general paresis.

Thus, for general paresis alone was the somewhat gradual onset with

increasing excitement, accompanied by expansive delusions concerning

unlimited finance, personal over-importance, and Argyll-Robertson

pupils. Dismissing the Argyll-Robertson pupils from consideration, the

diagnosticians were led to see in the constant motor activity displayed

in conveying an enormous number of thoughts on paper, inconsistent

talking with digressions, a manic-depressive psychosis. There was no

amnesia and no other sign of mental deterioration. There was a certain

improvement early in the hospital stay of the patient. Consciousness was

clear and orientation perfect. The delusions themselves, though

extravagant, were not inconsistent or fantastic. The hallucinatory

disorder was hardly characteristic either of manic-depressive psychosis

or of paresis.

The patient might be described as “interesting.” A good preliminary

training with years of travel and variety of occupation, furnished him

with a fund of knowledge. An excellent memory, prompt replies and

repartee, endless digressions with voluntary return to the original

topic, caused him to be an amusing and even instructive interlocutor.

However, his commitment and confinement in the institution seemed always

entirely wrong, and he expressed mixed feelings about the family, now

being bitter against them, and again condoning their mistakes. The

patient’s conduct was good and he was tidy in habits, and tried as far

as possible to conform to the requirements of the hospital. The doctor

showed a marked antipathy toward a certain male attendant, who had

removed articles from his clothing upon admission and had reclaimed a

book on rules and regulations. The doctor prepared a list of 327

different acts of abuse, lack of care, and insubordination which he said

he had observed in the hospital.

In the last weeks of the patient’s illness, his ideas became more

expansive and extravagant, dealing with a grapevine system of wireless

communication and delusions of unlimited wealth. He would at times keep

his room flooded with urine and water for the purpose of keeping down

the plague which he said was infecting the hospital. Later he mixed food

with urine and other ingredients, claiming that he was constructing an

elixir of life.

The =autopsy= showed few changes of the calvarium or of the dura mater,

nor was the pia mater more than slightly thickened and milky over the

frontal poles, along the longitudinal fissure and over the sulci. There

were fairly firm adhesions of the pia mater to the dura mater along the

longitudinal fissure and over the frontal poles and at the temporal

tips. The hemispheres were firmly interadherent, and the

cerebello-pontine tissues were covered with a firm leptomeningitis. The

floors of the ventricles were smooth and the basal vessels showed little

beyond a few spots of sclerosis. There was a generalized increase of

consistence. The frontal gyri were rather prominent with wide sulci, but

upon section no very marked atrophy of the gray matter could be shown.

The rest of the brain failed to show any flaring of sulci or any special

evidence of cortical atrophy. The brain weighed 1250 grams; a possible

diminution of 100 grams, considering the patient’s body length. However,

it must be remembered that he was at this time 63 years of age.

=Microscopically=, the diagnosis of GENERAL PARESIS was confirmed on the

basis of plasmocytosis, lymphocytosis, gliotic changes and nerve cell

destruction. There was an unusual variation in the degree of the

destructive process, which picked out, for example, certain regions of

the right side for maximal lesion (cornu ammonis, gyrus rectus, and

superior frontal gyrus).

If the patient’s own estimate of 35 years’ duration for his

Argyll-Robertson pupils can be trusted (and in general his memory was

extremely good), we may well conceive an unusual duration for the

process in his case. There was, however, in the body at large no very

marked degree of changes. There was a slight old tuberculosis. There was

a slight interstitial nephritis, with cardiac hypertrophy and fibrous

myocarditis. There was also a sclerosis of the mitral and aortic valves;

there were chronic changes in the spleen, liver, and bladder; there was

generalized arteriosclerosis of mild degree; there were two round

gastric ulcers near the pylorus. The liver weighed but 800 grams, and

its left lobe was somewhat rough.

This case is placed among the medicolegal and social cases because the

phenomena that ushered in his last illness were mistaken by the local

public for meritorious social reform measures. They were regarded as not

markedly different from the variety of steps taken by the very active

doctor in previous years; indeed the public eloquence that he displayed

a year before his death was quite in line with previous habits, despite

the suspicious over-brilliance of language. It is an important question,

how far the eccentricity and literary overactivity of the latter half of

the doctor’s total life can be explained on the basis of a mild

syphilitic irritation of the nervous system. In this connection we are

tempted to recall the suggestions of Mœbius concerning a portion of the

literary products of Nietzsche. Our doctor was by no means so brilliant

an exemplar of syphilitic literature as was Nietzsche, if we grant the

hypothesis of Mœbius to cover our doctor’s case as well as that of

Nietzsche. In the future, important studies of character change under

the influence of syphilis will doubtless be made. With modern diagnostic

methods, of course, the diagnosis would have been rendered almost at

once in the case of Major Isaac Thompson, M.D., and much of his past

life would have been brought under special review in connection with the

syphilis which doubtless the blood serum or at any rate the

cerebrospinal fluid would have shown.

This case illustrates but one of the many social complications arising

as the result of paresis. When one recalls that the onset is often

insidious and not correctly understood for a period of time, it is

readily seen that many unfortunate acts may be committed by a patient.

As hypersexual desire is not an infrequent early symptom and as judgment

is early disturbed, loose morals may ruin the patient’s reputation. The

poor judgment and expansive delusions often lead to foolish business

deals wherein the patient’s family is left destitute. At other times the

onset is sudden and then the danger of false commands or acts by a

person in a responsible position, as a steamship captain, an engineer or

chauffeur, may lead to loss of life and property.

=Sudden grandiosity: debts. PARETIC NEUROSYPHILIS (“general

paresis”): Question of liability.=

=Case 84.= Lester Smith was a salesman, 31 years of age, who, while on a

business trip, accompanied by his wife, suddenly developed grandiose

ideas. He originated a scheme of cornering the phonograph market. His

prospects seemed so certain to him, that he hired an expensive suite of

rooms in a hotel at something over $35 a day. As at the first

presentation of his bill it was found that he had no money to meet these

charges, he was taken into custody and at once transferred to a hospital

for the insane, where it was discovered that he was suffering from

GENERAL PARESIS.

1. What is the patient’s responsibility for these debts? Legally the

patient or his estate is responsible for debts accruing from

services rendered or goods received. As he is adjudged _non compos

mentis_ contracts entered into would not hold, and he would not be

considered liable for criminal acts.

Note: This case shows how dangerous paresis may be not only to the life

and usefulness of a patient, but further how it may ruin a family

financially. Mr. Smith’s little escapade used up all the money that he

had been able to save in his life and when he was taken to a hospital

his wife was left destitute.

=Suicidal attempt (?) by a neurosyphilitic.=

=Case 85.= At first Mrs. Annie Monks, a widowed seamstress, 50 years of

age, did not particularly suggest syphilis. Mrs. Monks was sent to us

from a general hospital. She had been found unconscious in her room,

with gas turned on, and a diagnosis of gas poisoning was made. Mrs.

Monks remained unconscious for 24 hours, and her apparent suicidal

attempt seemed to warrant her being sent to the Psychopathic Hospital.

Mrs. M., however, scoffed at the idea of any attempt at suicide, and

claimed to have had no recollection of any such affair. On the contrary,

she had gone to mass the morning of the day on which she was taken to

the hospital, remembered well enough returning to her room but nothing

of what followed until she woke up.

Mrs. Monks was not coöperative and would reveal few facts about her

history. For years, she had had edema of the feet and palpitation of the

heart (the heart was somewhat enlarged, with a double murmur in the

aortic area, systolic louder, and a blood pressure of 160 systolic and

85 diastolic; clubbed fingers; palpable liver). She had been treated in

the out-patient department of a general hospital for a number of months.

We could obtain no evidence of mental impairment, particularly none of

memory.

Aside from the heart lesions above indicated, the patient was fairly

well nourished, with a slight enlargement of superficial glands, and was

otherwise normal.

=Neurologically=, the slightly irregular pupils reacted poorly to light;

the right knee-jerk could not be obtained, whereas the left knee-jerk

was very active. Systematic examination revealed no other disorder

except that the abdominal reflexes could not be obtained.

Here we have, in a cardiac patient, a possibly or probably accidental

gas poisoning, and little to go upon for a profounder diagnosis than the

sluggish irregular pupils and unilateral absence of knee-jerk.

The routine serum W. R. came through as positive. Following custom, we

examined the spinal fluid, finding the W. R. here again to be moderately

positive (strongly positive to 1 cc., moderately to 0.7 cc., and

negative to 0.5, 0.3, and 0.1 cc.). The gold sol index was 1 2 2 1 0 0 0

0 0 0, which must be interpreted as syphilitic. There were 16 cells to

the cmm., the albumin was 1+, and the globulin stood at 2+.

Here, then, we seem to have evidence of an inflammatory process of the

central nervous system, and it is natural forthwith to be sceptical as

to the accidental nature of the gas poisoning. Perhaps there was an

attempt at suicide based upon a passing impulse, or perhaps there was a

period of confusion in which the cock was not turned off.

In any event, we feel justified in making the diagnosis of cerebrospinal

syphilis on the basis of the neurological and laboratory findings. On

the whole, we are inclined to make a diagnosis of VASCULAR NEUROSYPHILIS

with a moderate involvement of the MENINGES.

1. What is the outcome in such cases as that of Annie Monks? The

case somewhat resembled that of Martha Bartlett, who still

survives. The case of Annie Monks illustrates another outcome. A

few days after her admission, she became unconscious once more,

and upon recovery remained very much confused and aphasic,

moaning, and unable to handle herself well, although without

definite paralysis. Three weeks later the patient died, although

in the meantime strenuous antisyphilitic therapy was practised.

Death was sudden. We thought death due to cerebral embolism.

=Early delinquency and neurosyphilis in a juvenile.=

=Case 86.= Frank Johnson was 21 years of age when he was taken up by the

police for threatening his sister with a revolver. The police thought he

deserved an examination at the Psychopathic Hospital. The patient

protested that he had threatened his sister only to frighten her

because, he said, she nagged him and made him nervous. In fact, they had

always had trouble as she had always nagged him and they had always

fought together. Moreover, their mother always took the sister’s part.

They had been troubling him for days, and at last Frank could stand it

no longer. His sister had complained of the way he treated her dog.

Moreover, Frank said he had not been feeling well; there had been some

trouble with his stomach; and after one of the nagging attacks, he had

taken out an old empty pistol to scare his mother and sister.

In these cases, it is good practice to consult the sister also. She said

that Frank had always been very difficult to manage, unwilling to work,

preferring to loaf about, spending every obtainable cent; he was once in

a reformatory for several years, but not reformed thereby; recently

given to drinking; at times acting somewhat peculiarly (sitting at the

window with his hat on, refusing to move).

Further =mental examination= of Frank showed that he was properly

oriented and in possession of a good memory, although he was quite

obviously a liar. He lay about in bed at the hospital, saying that he

was too weak to be up. He was a bit dull, at times not readily grasping

ordinary questions.

=Physically=, Johnson was rather thin; the teeth were somewhat

peg-shaped although far from typically Hutchinsonian. The pupils were

unequal and irregular, and failed to react to light or even to

accommodation when tested. The deep reflexes of arms and legs could not

be obtained, though the superficial reflexes were present. For the rest

systematic examination proved negative. Serum W. R. negative.

The first thought in such a case would be that the criminological

diagnosis of delinquency would be sufficient. However, the pupillary

disorder and the areflexia are suggestive despite the negative serum W.

R. Resort was naturally had to lumbar puncture, whereupon a positive W.

R. was found, a characteristically “paretic” gold sol reaction,

globulin, excess albumin, and 134 cells per cmm. In short, it would

appear that we must consider a diagnosis of JUVENILE PARESIS, and, in

point of fact, the patient deteriorated rapidly from this time, becoming

demented at the end of a few months.

1. How far are the early difficulties of management (leading to a

reformatory) due to syphilis? We should not dogmatically say that

there is a relation between the early delinquency and syphilis.

Still, it is not unusual to find emotional disorder and

instability as well as delinquency in congenital syphilitics.

2. What suggestion, if any, should be made to the patient’s

intelligent and seemingly normal sister, two years older? We

prevailed upon Miss Johnson to submit to the W. R. of the serum,

which was found, as in the case of Frank, to be negative. Frank’s

sister should undoubtedly submit to a lumbar puncture; but in the

present phase of mental hygiene, she would be difficult to

persuade.

3. How is it possible to find such a marked evidence of congenital

syphilis in a younger sibling with no evidence of syphilis in the

elder? In the first place, there may be a history of entrance of

syphilis into the lives of the parents between the pregnancies.

However, in other instances, there is no evidence of such

intercurrent syphilis, and contrary to the prevailing opinion it

is not so infrequent to find congenital syphilis in the younger

brother or sister of a normal person.

4. What can be said of treatment in such cases? In the first place

it is clear that delinquent cases should be tested far earlier for

the possibility of syphilis. Had this case been examined by a

neurologist or alienist many years earlier, it is probable that

the same pupillary signs and the peg-shaped teeth would have been

found, and that the hypothesis of syphilis might have been raised.

There is no good evidence as yet that these cases can be markedly

benefited by treatment.

=Neurosyphilis in a “defective delinquent.”=

=Case 87.= Vivian Walker, 22 years of age, was arrested on the streets

of Boston for drunkenness. Upon arrival at the jail, she developed a

series of convulsions, each lasting a very brief time, with loss of

consciousness, frothing at the mouth, and jerky movements of the arms

and legs.

The Walker family was known to the police, since there were police

records in two generations on the maternal side. The father was regarded

as of rather low-grade mentality; a sister had committed suicide. Vivian

herself had been irregular at school, was regarded as vicious, and had

been hysterical. She had been committed to a reformatory at the age of

15 years. In the reformatory she had a number of excited outbreaks, with

resentment of discipline, and these outbreaks presented hysterical

traits. After each outbreak Vivian was depressed. It was during her stay

at the reformatory that her sister committed suicide. Vivian attended

the funeral, and the idea of suicide appears to have taken hold of her

mind, as she constantly spoke of suicide, threatened suicide, and made

several attempts. She claimed at this time to see visions and to hear

her sister’s voice. On that ground she had been committed to a hospital

for the insane at 16.

At the hospital there were many fluctuations in mental condition. Vivian

professed discouragement on account of poor home influences, telling how

her mother had often been in prison, allowing Vivian to come under the

influence of bad girls. Now and then Vivian had outbreaks of profanity

and glass-breaking, and she also made at the hospital for the insane

several half-hearted attempts at suicide. At the age of 19 she was

returned to the reformatory, whence she was placed out on probation and

allowed to return home.

However, she was shortly re-committed to the insane hospital in a phase

of excitement, talking continuously of men and sex relations, and also

of imaginary illicit sex relations with any man whom she happened to

see. Again from time to time she made attempts at suicide. However, she

was allowed to go out on visit, returned to her habits, and at the time

of her arrest was living as a prostitute.

After her convulsions in jail, she was admitted to the Psychopathic

Hospital. At first obstinate and stubborn, later she became tractable.

Special mental tests left her in the subnormal class, but we could

hardly class her as feebleminded. We were able to observe her in a

number of seizures, during which she would drop to the floor, apparently

lose consciousness, writhe about, and assume the position of

opisthotonos, the whole attack lasting but a minute or two.

There was pelvic tenderness, with gonococci in the urethral smear.

Salpingectomy had to be performed, but after the operation Vivian

insisted upon getting up and running about on the second day, tearing

the bandages from her abdomen, and infecting the wound. Outbreaks of

excitement also followed the operation.

In the diagnosis of this case, we must probably separate the convulsive

phase from the remainder of the phenomena. The conduct disturbance,

emotional outbreaks, and suicidal attempts date from early youth, and no

doubt the diagnosis defective delinquent would fit Vivian from the

beginning. The hereditary taint is characteristic enough. The sundry

phenomena in the insane hospital, and particularly the hallucinations,

lead one to wonder whether Vivian is not possibly even suffering from

dementia praecox.

As to the convulsions, it would hardly appear that they are typically

epileptic, although certainly epileptoid. Their onset at 22 is somewhat

unusual. Several features of the seizures together with the opisthotonos

and the previous history of hysteria, lead one to think of making the

diagnosis hysteria.

1. Can cerebrospinal syphilis cause the symptoms? We found the serum

W. R. to be positive though Vivian denied syphilitic infection.

(She also denied gonorrhœal infection despite the clinical and

laboratory findings.) We found that the spinal fluid yielded a

gold reaction of a typical syphilitic nature, showed an excess of

albumin, a slight amount of globulin, and 130 cells per cmm. Even

these findings, however, would perhaps not justify stating that

the convulsive seizures are of syphilitic nature. The seizures

disappeared under the administration of antisyphilitic remedies.

It would seem, therefore, that the seizures should be regarded as

of syphilitic nature. In any event, the diagnosis of cerebrospinal

syphilis is justifiable. This syphilis, however, is of an active

nature and probably of recent production. We should be at a loss

to explain the earlier mental features in Vivian as syphilitic and

are therefore fain to associate the two psychoses, PSYCHOPATHIC

PERSONALITY and DIFFUSE CEREBROSPINAL SYPHILIS.

=NEUROSYPHILIS (“paresis sine paresi”) in an habitual criminal, a

forger.=

=Case 88.=[17] —— was brought to the hospital by the police. He was

charged with having forged a check, and on account of the crudeness of

the work his mental condition was suspected.

=Family History.= The paternal grandfather was considered fast, drank a

great deal and was said to be a thief. The father is said to have been

forced to leave the State when a young man in order to avoid the

reformatory. Paternal cousin murdered a man; the sisters of this cousin

said to have been wild and one brother married a prostitute. Nothing

known of maternal relatives.

=Past History.= Medical history is unimportant. He denies syphilis. His

early childhood is of little significance. He was somewhat dull in

school. At about the age of twelve he began to lie and steal, and has

continued this ever since. His attempts have all been very crude, it is

said, and when confronted he would strenuously deny his deeds, even when

the evidence was overwhelming. He forged checks, borrowed money from all

his friends, and charged things at stores to the family. The family paid

the bills for a time, and then later had him sent to a reform school. He

was married at nineteen, but wife has left him and obtained a divorce.

He has been excessively alcoholic for years, and is suspected also of

taking drugs. He was discharged from the navy dishonorably. He later

joined the army and was discharged therefrom on account of “rheumatism,”

according to his account, but in reality deserted. He had finished a

jail sentence of thirteen months for forgery a little over a year before

entrance.

=Physical examination= shows a well developed and nourished man. The

general physical examination is negative. The lungs show nothing

abnormal. The heart is not enlarged, there are no murmurs or

irregularities; blood pressure, 145 systolic. The alimentary system is

negative. No palpable lymph glands. =Neurological examination=: pupils

equal and react to light and accommodation. Extraocular movements well

performed. Tongue projects in the median line, with no tremor. There is

no evidence of facial paresis or weakness of the muscles. The biceps,

triceps, knee-jerks and ankle-jerks are present and equal on the two

sides. There is no Gordon, Babinski or Oppenheim; no ankle clonus. There

is no tremor of the extended hands. No Romberg sign. There is a little

difficulty in the finger-to-finger test. There is no sensory disturbance

either subjective or objective. No tenderness over nerve trunks.

=Mental examination= shows nothing of a psychotic nature. Patient is

well oriented; memory for remote and recent events is well preserved,

school knowledge well retained, grasp on current events good; no

delusions or hallucinations elicited. Patient is not feebleminded,

according to the intelligence tests of Binet and Simon and

Yerkes-Bridges, but shows poor attention and gives evidence of weakness

in volitional spheres; is very suggestible.

To summarize the case, then, we have a man of thirty years of age who

has shown criminalistic and anti-social tendencies since childhood,

whose general physical and neurological examination is negative

(excepting the laboratory tests), whose mental examination shows no

psychotic symptoms, and who seems not feebleminded. In other words, with

the exception of the serological and chemical findings in the blood and

cerebrospinal fluid, there is nothing to suggest that he is more than a

“criminal type.”

Wassermann reaction in blood serum positive.

Wassermann reaction in cerebrospinal fluid positive. Examination of

cerebrospinal fluid: globulin ++, albumin ++, cells 55 per cubic

millimeter; large lymphocytes, 9.1 per cent; small lymphocytes, 90 per

cent; plasma, 90 per cent. Gold sol reaction, 3321000000.

1. Can the criminalistic tendencies be condoned in this case on the

ground of neurosyphilis? As a matter of fact the delinquencies in

this patient reach back to early childhood and as there is no

evidence of congenital syphilis it cannot be held that syphilis

had any bearing in the causation of symptoms. Even were the

delinquencies only of recent date it is doubtful if the court

would take cognizance of the laboratory findings in the absence of

definite mental symptoms. In this connection it may be stated that

the court takes cognizance only of the acts of a patient at time

of examination, and not of the history or laboratory findings, in

committing a person. We have had several patients who from

history, physical signs and laboratory tests made the diagnosis of

paretic neurosyphilis easy and yet who could not be committed

because they were mentally clear at the time. Such patients may be

of grave potential danger to themselves and families, and present

numerous social problems. See case of Joseph Wilson (95).

=JUVENILE PARETIC NEUROSYPHILIS (“juvenile paresis”) with initial

trauma.=

=Case 89.= Margaret Tennyson was a small girl of six years, described as

having been normal until run down by a double-runner sled about 13

months before her arrival at the hospital. The change was stated to be

remarkable. “She was as unlike her own self as darkness and daylight.”

Once fat and sunny, talkative and demonstrative with her toys, now

Margaret had become silent, sullen, worried, and of a violent temper,

stubborn and unmanageable. It does not appear that the patient was

seriously injured by the double-runner, as she was able to walk a short

distance home. Shortly, however, she began to have trouble with her feet

(diagnosed at the time as flat-foot), and thereafter her whole character

and disposition changed. Upon arrival at the hospital, the patient

walked with a typical scissors gait of spastic paraplegia.

=Physical examination= was very difficult through lack of coöperation

and a screaming and kicking resistance upon every attempt. There was a

suggestion of hydrocephalus in the protrusion of the forehead. The

pupils reacted readily to light and accommodation. The knee-jerks were

active, but there was otherwise no disorder of reflexes. The patient had

great difficulty in getting up from the floor, and for the most part

insisted upon lying in ventral decubitus on the floor, crying when

attempt was made to raise her. An attempt was made to test her by the

Binet scale, by which she was found to rate at 2⅘ years although a

portion of this low-rating was thought to be due to a failure of

coöperation.

The =family history= threw little or no light upon the case. The parents

were living and well; a brother of 16 years was at work in the market

district; two of the other siblings are in the first and second grades

at school and regarded as exceptionally bright by their teachers. The

fourth was the patient, Margaret; a fifth had died at 9 weeks of heart

trouble; the sixth, seventh, and eighth, of 3, 1½ years and 3 months

respectively, appeared entirely well. There were no miscarriages or

stillbirths.

[Illustration:

Juvenile paresis—spastic paraplegia. 5 years.

]

The scissors gait and spasticity seem to point undoubtedly to organic

disease of the nervous system, along with which the mental deterioration

seemed to suggest an active progressive involvement of the cerebrum. The

history seemed to be convincing that the child was not an instance of

congenital feeblemindedness.

A neurologist’s clinical diagnosis would naturally be syphilis. In point

of fact, this diagnosis was borne out by the laboratory tests, which

showed a positive W. R. in the serum and spinal fluid, positive

globulin, a slight excess of albumin, and a syphilitic gold sol

reaction.

1. What is the significance of the trauma in the case of Margaret

Tennyson? The trauma seemed to the family the precipitating cause.

We find cases of general paresis in adults very definitely

following trauma, yet neurosyphilis, both in adults and in younger

patients, mainly occurs without trauma. On the whole, in this

case, it is perhaps safer to regard the trauma as mere

coincidence. A sister older than Margaret was found upon

examination to have a positive W. R. The other children could not

be examined.

=Traumatic form of PARETIC NEUROSYPHILIS (“general paresis”).=

=Case 90.= The point about Joseph O’Hearn was his entire mental

soundness up to the time of an injury at work, when he was blown through

a double window in an explosion, badly bruising his head. Shortly after

the accident, although not immediately, the patient began to show signs

of mental disorder, doing very foolish things, losing his memory, and

becoming unable to work.

It was eight months after the explosion when O’Hearn, at the age of 36,

was admitted to the hospital with general mental impairment. O’Hearn was

confused and disoriented for time and place, although he seemed to

understand that he was in a hospital. He was given to foolish laughter

and a silly manner. There was considerable emotional disorder; judgment

was clearly impaired, and memory was poor.

=Physically=, there was little to be found except upon =neurological

examination=. The right knee-jerk was greater than the left; the tongue

and fingers showed marked tremor, there was a speech defect and writing

disorder.

On the whole, it seemed impossible not to make the diagnosis GENERAL

PARESIS, especially in view of the laboratory tests, with positive W. R.

in both serum and fluid, a “paretic” type of gold reaction, 59 cells per

cmm., excess albumin, and a large amount of globulin.

1. What is the relation of the trauma to the paresis? Trauma is

regarded as a precipitating cause, and Industrial Accident

Commissions have been known to allow damages in such cases. Mott

believes that the symptoms of a post-traumatic paresis must not

develop until after a week’s interval of freedom from symptoms,

since he believes that time is required to destroy or irritate the

brain to the point of producing the paretic picture. Our data are

in agreement with those of Mott. Mott also points out that gumma

sometimes occurs at the site of the trauma.

=False claim for compensation in neurosyphilis.=

=Case 91.= The facts in the case of Levi Sussman can be brought out by

the following extracts from a report to the Industrial Board: A claim

was made to the Board that the symptoms had developed after a fall from

a building, some _nine months before hospital observation_. No

connection could be found between this accident and the PARETIC

NEUROSYPHILIS found. We introduce the case to emphasize the possibility

that irrelevant accidents may be regarded by ignorant or unscrupulous

persons as setting up a mental disorder for which damages are claimed.

If symptoms are already in existence before the accident and are not

especially increased thereafter, naturally no damages should be

recovered. Unscrupulous persons may falsify about the pre-traumatic

history and claim the development of symptoms immediately after the

accident. Such claims are beyond question to be viewed with the greatest

suspicion. Some days or weeks should elapse before definite symptoms in

post-traumatic paresis appear. Just how long an interval may elapse

between trauma and paretic symptoms and shall entitle the case to be

regarded as one of traumatic paresis, is perhaps a matter of doubt. It

would seem, however, on general grounds that three months is the longest

period in which the post-traumatic effects are likely to be delayed.

The question of traumatic paresis is of great interest on account of the

war. The great strain under which the men at the front live and the

physical injury due to being “buried” is probably responsible for an

increasing number of cases of neurosyphilis. Such at least is the

impression of Canadian medical officers with whom we have spoken. See

Section VI, Neurosyphilis and the War.

=Traumatic exacerbation(?) in PARETIC NEUROSYPHILIS (“general

paresis”).=

=Case 92.= The case of Joseph Larkin was of note from the point of view

of the Industrial Accident Board. This Irish teamster was said to have

been injured in his head two or three months before coming up for

examination at the age of 45. For a week Larkin had had frontal

headaches, had been sleeping poorly, and had been somewhat worried. In

fact, he had stopped work. The W. R. of the serum was positive and a

diagnosis of PARESIS could be made. The case did not come up for

consideration by the Industrial Board until two years after his initial

appearance.

The =physical examination= showed irregular pupils, sluggish pupillary

reactions, Achilles absent, swaying in the Romberg position, enlargement

of the heart to the left, positive W. R. of the blood and of the spinal

fluid.

=Mentally=, the patient’s orientation for place was poor and his memory

defective. Emotionally he was depressed or apathetic and was

apprehensive. His flow of thought was slow, and his insight into his

condition poor.

It is interesting that a variety of causes have been assigned in this

case for the condition: such as, his work, anemia, unhygienic

surroundings, and arteriosclerosis.

This case is not a sharply-defined case of post-traumatic general

paresis, since there had undoubtedly been a variety of mental changes

before the accident. Accordingly, recovery of damages to a full amount

could hardly be expected as in certain cases in which the phenomena of

paresis appear only after the trauma.

[Illustration:

Post-traumatic cranial gumma—developing 13 months after local injury

of skull.

]

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

=Case 93.= The medicolegal interest of Richard Marshall is extreme, as

may be seen from the following brief report by the Psychopathic Hospital

to the Industrial Board.

“As to the case of Richard Marshall, a patient under the provisions

of the temporary care act from December 1 to December 10, inclusive,

this case has proved unusually interesting in that the patient has

proved to be syphilitic by the Wassermann reaction of the blood.

There is no evidence of syphilis in the examination of the

cerebrospinal fluid. The X-ray examination of the skull, taken in

connection with the Wassermann reaction of the blood, warrants the

diagnosis of syphilitic osteitis of the skull at the site of the old

injury. We regard his present condition as shown by the X-ray as a

syphilitic bone condition predisposed to by the injury. We do not

find that the patient has any features of traumatic neurosis.

“Mentally, having an actual age of 30, patient grades at 11.2 years.

It may be that patient has always been a moron. He has earned about

$8.30 a week.

“We regard the patient as deserving treatment and feel that

responsible parties in the case would do well to have such treatment

instituted.”

The principal symptom of which Marshall complained was headache chiefly

felt in the region of the osteitis. There was marked sensitiveness to

percussion in this area. It is of course difficult to decide whether the

headache was entirely due to the gummatous lesions or whether the trauma

had caused contusions of the brain as well. It is also possible that the

dura underlying this area was involved.

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

=Case 94.= David Fitzpatrick was a case referred to the Psychopathic

Hospital by the Industrial Accident Board. He was a granite-cutter of 52

years of age, and had begun to complain of pain in the forearm,

extending back from the elbow, about six months before admission. It

seems that the patient had been growing progressively worse and had

thought he would have to quit work because of difficulty in grasping the

hammer. A physician had told him that he must stop his work at

granite-cutting or else he would entirely lose the use of his arm. He

was in point of fact laid off because of slackness of work and had been

unable to get work again. The pain in the arm, however, had continued

and at times was very severe. Sometimes the pain and the worry led to

insomnia. Fitzpatrick wanted the insurance company to pay certain

accumulated bills, and maintained that he would be able to do work at

$15 a week if work could be found for him. The general situation in this

case can be gathered from the following abstract from the report to the

Industrial Accident Board.

“Secretary Industrial Accident Board,

“Dear Sir:

“_In re_ David Fitzpatrick

referred to us with a copy of an impartial report filed by the

Massachusetts General Hospital,—we concur with said impartial report

that there is now no evidence of paralysis of the arm. We do not

find that the positive Wassermann reaction, although it indicates a

history of syphilis, has affected the patient other than possibly to

have reduced his general mental capacity. Our special tests yielded

a percentage of 62% of what a patient of his age and station should

possess. There seems, however, to be no connection between this

reduction of mental capacity and the difficulty with the arm. We

cannot connect the history of alcoholism with the arm trouble.

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

such effects.

“The patient’s fairly circumstantial account of his difficulty seems

to point to a degree of myalgia or muscular pain in the region of

the forearm when held in a certain position and a feeling of

numbness in the third and fourth fingers. Whether these phenomena

are due to local pressure upon nerves in the upper part of the

forearm due to neuritis, or whether we are dealing with a functional

neuralgic phenomenon is a question.

“We have applied some special tests for faradic sensibility to all

the fingers of both hands and have found that the fingers of the

right hand are still less sensitive than those of the left,

particularly the thumb and the little finger. This test has not yet

been applied in a sufficiently large number of cases to prove any

difficult point, nevertheless the findings are in line with the

patient’s own circumstantial account of former feelings of numbness

in the third and fourth fingers of the right hand.

“Obviously, then, our opinion is that there is still to be found

some effect of the disease, whatever it was, which caused the

patient to knock off work. If we had more experience with such cases

and more data with the new test which we have applied, we should

perhaps be inclined to admit the diagnosis of _occupation neuritis_

and to suppose structural alterations in the nerve trunks

corresponding with the location of the muscular pain and the

anesthesia of fingers and the dulling of electric sense, but in the

present stage of our experience, it is probably wiser to call the

case one of _occupation neurosis_.”

It is clear that the W. R. in this case was of peculiar value in at

least partially clearing up the findings, yet it must be remembered that

it is a principle of the modern administration of industrial accident

boards and similar organizations that it is the employer’s lookout

whether the employee has syphilis. Recovery can be made as if the injury

were due wholly to an accident. It was not possible however definitely

to prove or disprove a relation of syphilis in the form of a syphilitic

neuritis to the condition in this case.

The special tests above referred to are the electric sensory threshold

tests of E. G. Martin.

=Character change: neurosyphilis.=

=Case 95.= Joseph Wilson offered a very serious social problem. He was

the father of two children, and his wife was pregnant at the time of his

admission to the Psychopathic Hospital. He was a husky-looking man of 33

years of age, but for the past four years he had been deteriorating in

his work; he had been drinking heavily, and finally had stolen to obtain

money for liquor. It was on account of his alcoholism and delinquency,

which were taken as an indication of change of character, that he was

sent to the hospital.

Examination on his arrival disclosed at once that there was more to the

case than alcoholism, for the =neurological examination= showed that the

pupils were irregular, the right being larger than the left, both

reacting sluggishly to light, and there was an inequality in the

reaction of the two eyes, the left being better than the right. The

tendon reflexes were exaggerated, with ankle clonus on both sides, more

marked on the right. There was also a marked speech defect. Otherwise

the =physical examination= showed nothing of importance.

The W. R. of the blood and spinal fluid was strongly positive. The

globulin test was strongly positive, the albumin was markedly increased,

there were 74 cells per cmm., and a gold sol reaction of the “paretic”

type.

A formal =mental examination= did not show very much of consequence; his

memory showed no marked impairment, he was not deluded or hallucinated,

and he had a pretty good insight into his failings. However, he was

somewhat childish, and his irritability was quite marked. Were one to

rely upon the mental signs alone, it is probable that a diagnosis of

chronic alcoholism with deterioration would be made; but in the presence

of the physical findings and the laboratory tests, the diagnosis of

neurosyphilis had to be given. It is obvious that, while the patient was

suffering from a progressive brain disease, and while he did show mental

symptoms, there was not sufficient ground on which to commit him, and

therefore he had to be turned out into the community. As a matter of

fact, he was not prosecuted on account of his theft, because, although

legally responsible, it was felt that his disease was at the basis of

the character change which had led him into difficulties. Further

developments of his relations with society had to be considered,

however. It was possible to get him to discontinue the use of alcohol

altogether, and for nearly a year he has taken no alcoholic liquor and

has been self-supporting. However, his irritability has been very great,

making it very difficult for his wife to live with him, and causing his

sister to break off all relations with him.

Here, then, is a man with a marked CHARACTER CHANGE as the result of

neurosyphilis, so that it is difficult for him to maintain the usual

social relations. It does not seem possible to remove him from the

community.

1. May one speak of general paresis without mental symptoms? If one

considers general paresis a mental disease, of course it cannot

exist without mental symptoms. However, if one considers the

disease as a chronic syphilitic meningoencephalitis characterized

by its pathological anatomy, then one may readily speak of general

paresis although no real evidence of mental symptoms can be

discovered. It would seem that we must take this attitude with our

present conception of brain localization, for it is easy to

conceive of a general paretic process affecting areas which do not

definitely relate to psychic function. And further, such a process

may exist but not be of such a grade as to cause mental symptoms.

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

and treatment.=

=Case 96.= The Bornstein family is remarkable. Let us hang the story on

Becky, the mother, an Austrian woman of 43 years, who appears to have

been perfectly well up to within a year. About a year ago, Mrs.

Bornstein began to suffer from severe headaches, which were treated with

apparent success by an osteopath: at all events, Mrs. Bornstein

recovered therefrom in about six months. However, two months later, she

had a convulsion, with foaming at the mouth, blueness of face, and

general muscular stiffening. The convulsion lasted for several minutes.

Again, a fortnight before admission, the patient had five convulsions of

an identical nature in a single night.

Moreover, since the first convulsion, Mrs. Bornstein’s =mental

condition= has altered and become variable, so that at times she is

excited, at times depressed. She would assert inaccurately that there

was some one in the house, and that she had at different times committed

crimes of a heinous nature. Now and then she would seem to see moving

pictures. Her memory was poor and she seemed to believe that events of

five or six years ago had just happened.

The pupils were sluggish, the knee-jerks and ankle-jerks were absent,

there was slight ataxia, and there was speech defect. The suspicion of

neurosyphilis was so strong that it seemed surprising that the W. R. of

the blood serum, even after repeated tests and after the provocative

injection of salvarsan, proved negative. However, the spinal fluid

yielded a positive W. R., and a gold sol reaction of the “paretic” type,

together with 12 cells per cmm., and a marked increase of albumin, with

positive globulin. It would seem warrantable to make a diagnosis at

least of syphilis of the nervous system in this case, but it is a

question whether we should be warranted in making the diagnosis general

paresis.

That the diagnosis is doubtful may perhaps be seen from the variety of

diagnoses in the rest of the family. In the first place, Mrs.

Bornstein’s husband admits syphilitic infection many years before. He

states also that his wife after marriage showed signs of syphilis and

received some treatment, although limited. It is stated also that the

husband himself at this time has a positive W. R. and has stiff pupils

and petit mal attacks. The oldest son, 22 years of age, is confined in

an institution with juvenile paresis. The second son has recently died

at the age of 20 years, receiving a diagnosis of rupture of the aorta. A

third son, 19 years of age, has the appearance of having achondroplasia,

although the proportions of his limbs do not quite correspond with those

of an achondroplast. The fourth son, 17 years of age, is suffering from

caries of the spine. A fifth son, 14 years old, is neurotic and has the

so-called Olympic forehead. The sixth and last son died shortly after

birth of unknown cause.

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

=Case 97.= Walter Heinmas was a draughtsman 33 years of age when he was

brought to the Psychopathic Hospital suffering from mental disease. This

was diagnosed as general paresis, both on account of the clinical

symptomatology and on account of the laboratory findings. In fact, it

was a case of the classical type with marked euphoria and grandiosity.

As is the routine procedure at the Psychopathic Hospital, in the case of

all syphilitic patients, the family was sent for. This consisted of the

wife and two daughters, aged 9 and 7 respectively. The patient denied

any knowledge of a syphilitic infection. The wife, also, gave no history

of any primary, secondary, or tertiary symptoms; there had been no

abortions, miscarriages, or stillbirths; both children had been born at

term and had been entirely healthy. Examination showed that the mother

had no signs referable to syphilis, and that both the children were

mentally well endowed, with good physique and showing no stigmata of

congenital syphilis. Still the W. R. of all three (the mother and the

two children) was positive in the blood serum. These tests were repeated

several times on the children, with and without injections of salvarsan,

and they remained consistently positive.

1. Are these children to be considered congenital syphilitics

despite the absence of stigmata or symptoms? We must consider

these children as congenital syphilitics and candidates for the

group frequently spoken of as _syphilitis hereditaria tarda_.

2. What is the frequency of syphilitic involvement in the mate and

children of paretics? In our series, we have found that about 15%

of the marriages where one member develops paresis, result in

sterility; that in 18% abortions, miscarriages and stillbirths

occur; and that in 15% positive W. R. is obtained. We have adopted

the motto: “=The families of paretics are the families of

syphilitics.=”

=Neurosyphilis: question of marriage.=

=Case 98.= Mr. Jacobs’ wife was admitted to the hospital with a

diagnosis of general paresis. A few weeks after her admission, she died

as a result of her disease. According to our routine, her husband and

the children were examined for evidences of syphilis.

Mr. Jacobs’ blood serum was found on repeated tests to be positive. He

resolutely denied any knowledge of a syphilitic involvement, but it was

later learned from his brother that about two years before his

marriage—that is, more than 25 years before we saw him—he had acquired

syphilis and had had a very small amount of treatment.

Mr. Jacobs was put upon antisyphilitic treatment in the form of

injections of .3 gram of salvarsan every two weeks with occasional

intramuscular injections of mercury salicylate. After seven months of

treatment, the blood serum still remained positive. At about this time,

the patient came to us to ask about getting married again. He said that

he was living with his sister, who kept telling him that he was the

cause of his wife’s death, and this was so unpleasant that he desired to

start a home for himself again!

1. What advice should be given? It is a general opinion that the

longer the period after the initial infection, the less the

chances of infecting a partner. This chance is further reduced

under antisyphilitic treatment, of which a considerable amount had

been given in the case of Mr. Jacobs. However, when one considers

the trickiness of syphilis and the fact that there is some chance

of infection, which we would apparently overlook if we gave him

permission to marry at this time, the only possible course was to

tell the patient that he should not consider marriage until his

Wassermann had become negative and remained so for some time. The

children in this case were negative.

2. What is the physician’s duty to the family of a syphilitic

patient? It is our firm conviction that it is the duty of every

physician to his syphilitic patient, to the patient’s family, and

to the community, to examine the mate and the children for

evidence of syphilis acquired or congenital and to offer treatment

if it is found to be needed. This is one of the chief means at our

disposal today to prevent the late disasters of syphilis, acquired

or congenital, for by such examinations the syphilitic condition

is discovered before lesions have occurred which are irreparable.

We know that the mate and children of a syphilitic patient have

been exposed to syphilitic involvement, and it is our duty as

physicians in possession of such knowledge, and as guardians of

the public health, to investigate such cases, so that if they be

found to have syphilis, steps may be taken to treat them early.

3. How much danger is there of causing unhappiness and breaking up

families by this procedure? This question offers a chance for many

theoretical answers. The facts are, however, that in doing this as

a routine for nearly three years and examining several hundred

families, there has been no instance to our knowledge in which a

family has been broken up or grave difficulties have been

encountered by this procedure.

4. In what percentage are the mates or children of neurosyphilitics

found to show definite symptoms of syphilis? It is our opinion

that the situation in regard to neurosyphilitics is the same as

for syphilitics in general: That the same laws of attenuation of

virus, and of chance occur here as elsewhere.

Just as this book is going to press, we have learned that the distraught

Mr. Jacobs, still desirous of starting a home for himself and feeling

entirely well, consulted a physician. This physician took a sample of

blood and had it tested at a competent laboratory, which reported the

blood negative.

On the strength of this test, the physician felt himself warranted in

recommending, or at least not advising against, Mr. Jacobs’ marriage,

which has probably now taken place.

Although there is some doubt what ethical relation a state institution

shall maintain with physicians in private practice, we took occasion to

call the attention of our patient’s new counsellor to the fact of the

patient’s neurosyphilis. We noted that the man’s serum had been

constantly positive (Massachusetts State Board of Health findings) in a

score or more of observations. We called attention to the fact that

lumbar puncture had shown positive signs of neurosyphilis, including a

positive W. R., globulin, excess albumin, pleocytosis, and positive gold

sol. These facts, according to a letter received from the private

practitioner in question, have not altered his opinion in the slightest

to the effect that our patient is completely normal and entirely

suitable for marriage. It is clear that he regards the patient as not a

victim of General Paresis.

5. What is the significance o the negative observation eventually

obtained in Jacobs’ serum? One’s first thought is to impugn the

accuracy of the laboratory work, but against this suspicion is the

excellent reputation of the laboratory in question, and the

agreement in the majority of its findings with those of the State

Board of Health. It is more likely, as we assured the private

practitioner at whose request the observation was made, that this

negative test was an exceptional and isolated observation such as

is not infrequent in long series of observations, particularly

those made under therapeutic conditions. In so important a matter,

we are inclined to feel that the physician in question should have

resorted to two more observations at intervals before running

counter to the position taken by the hospital.

——many a hard assay

Of dangers, and adversities, and pains.

Paradise Regained, Book IV, lines 478–479.

V. SOME RESULTS OF TREATMENT

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

to face.

=SPASTIC HEMIPLEGIA in PARETIC NEUROSYPHILIS (“general paresis”),

showing marked degenerative changes, a condition in which therapy

could be theoretically of very little avail. Autopsy.=

=Case 99.= James McDevitt arrived at the Danvers Hospital, July 20, 1906

(saying that he came to be “thawed out”), and died less than six months

later: January 12, 1907. He was 34 years of age. He had been a

shoe-worker after leaving school, had worked eight years with the

General Electric Co., and had then become a bartender. He had, however,

stopped work in September, 1905, and we may safely say that mental

symptoms had begun insidiously at about that time. His symptoms, if

there were any, had been masked by a heavy alcoholism, but an obvious

change had appeared in November, 1905. The patient lost ambition, smoked

and loafed about his room, and developed speech disorder. He denied

venereal disease, nor was there any superficial evidence of such.

=Physically=, the patient showed little or no disorder except acne of

the trunk, patches of eczema on the left lower chest, and numerous

brownish scars along both tibiae.

=Neurologically=, the Romberg position was maintained, but the gait was

very unsteady on attempts to walk a straight line; fingers, tongue, and

face were tremulous, and finer movements were performed with marked

incoördination. No direct or consensual light reactions could be

obtained in the pupils, which were dilated and irregular.

The condition of the reflexes is important on account of the autopsy

findings. The abdominal and cremasteric reflexes were prompt, and the

knee-jerks equal and very lively. Achilles and normal plantar reactions

were present; there was no clonus; the arm reflexes were very brisk.

=COMMON THERAPEUTIC CONCEPTION=

[M]VP = TYPICAL PARESIS

MV[P] = TYPICAL CEREBROSPINAL SYPHILIS

[M]V[P] = TYPICAL SYPHILITIC ARTERIOSCLEROSIS

(M = Membranes, V = Vessels,

P = Parenchyma, [] = not involved)

CHART 21

The =mental symptoms= need not detain us. Consciousness was clear;

orientation for time, place, and to some extent for persons, was

imperfect. Arithmetic had been largely forgotten. Handwriting was

irregular and scrawling, and in places unintelligible. Although the

patient claimed that his memory was intact, it was decidedly imperfect.

He remarked that John D. Rockefeller, a Chicago king, was President; the

General Electric Works had almost 50,000 people at work; and in fact

Lynn was one of the largest cities in the state, having over 12,000

people. The height of patient’s room was estimated at 25 feet. There was

a slight euphoria. There was never any doubt of the =diagnosis= of

PARETIC NEUROSYPHILIS (“general paresis”).

Five months after admission, slight convulsions developed, after which

the patient was more dull and demented; he became bedridden. More

convulsions followed, leaving the right arm and hand useless. There were

clonic spasms of the muscles of both lower legs. Decubitus developed and

death occurred.

We may set the total duration of symptoms in the case of James McDevitt

at a little over a year; nor is there any evidence of previous or

prodromal symptoms beyond a total period of about 15 months, unless we

may regard his leaving the General Electric Works to become a bartender

some nine years before death, as a symptomatic change of character. In

any event, it is of note that the =autopsy= showed singularly few

lesions. Death was due doubtless to complications following decubitus,

and there was a slight acute splenitis. The kidneys showed some

parenchymal change. The aorta showed many patches of sclerosis, with

calcification or ulceration throughout its length. These changes were

not characteristic of syphilitic disease. There was considerable

coronary arteriosclerosis and a slight mitral valvular sclerosis. There

was a brown atrophy of the heart muscle, somewhat surprising in a man of

34 years. The =brain= was practically normal, weighed 1200 grams, and

showed convolutions normal in size, relation, and arrangement. There was

no sclerosis grossly evident in the blood vessels. The pia mater

appeared to contain a considerable excess of clear fluid. The calvarium

was of normal thickness and showed diploë and the dura mater failed to

show adhesions. There were no macroscopic signs of lesion in the spinal

cord.

=Microscopically=, the lymphocytosis, plasmocytosis, and phagocytosis of

the perivascular spaces, (relative?) increase in blood vessels, the

gliosis, and evidence of nerve cell destruction, taken together

warranted the diagnosis of PARETIC NEUROSYPHILIS. It was plain that the

nerve cell destruction was best marked in the _inner layers of the

cortex_. The microscopic study of the spinal cord showed that there was

very possibly a slight sclerosis of the posterior columns in the lumbar

region, but this was so slight that it could hardly be noted in the

myelin sheath stains (Weigert). Very sharply marked, on the other hand,

were the _bilateral pyramidal tract lesions_ in the lumbar and thoracic

regions, less marked at the cervical levels.

Without attempting to analyze carefully all these findings, it is

interesting to note in this case a foil to the usual spinal cord picture

of paretic neurosyphilis. The spinal cord, ordinarily normal, or perhaps

more usually affected by a degree of posterior column sclerosis, in this

case showed such well marked pyramidal tract sclerosis that we may

perhaps place the case in a subordinate group of SPASTIC PARETIC cases

of NEUROSYPHILIS. The source of the pyramidal tract disease lodges,

however, in the cortex cerebri itself, being part and parcel of the

lesions mentioned above as affecting more directly the inner layers of

the cortex. Many of the so-called giant, or Betz, cells had undergone a

complete destruction. It will be remembered that clonic spasms of the

muscles of the legs appeared in the fortnight preceding death, and that

there had been convulsions for about six weeks before death. There was

no evidence at the autopsy why the right arm and hand should have become

useless, whereas the left upper extremity remained normal. This case,

then, forms an exception to the ordinary paretic neurosyphilis group in

that the brunt of the microscopic process was borne by the inner layers

of the cortex. The cells of origin of the pyramidal tract fibres had

been cut in this lesion, and had become subject to partial or complete

destruction. Note, however, that the lesion remained a microscopic one

and that the marked convulsions were not related to gross lesions,

thereby following the rule for paretic seizures.

[Illustration:

Bilateral pyramidal tract sclerosis, secondary to destruction of large

motor (Betz) cells of motor (precentral) cerebral cortex—paretic

neurosyphilis.

]

From the standpoint of possible treatment, it is of course true that few

organs of the body showed grave lesions save in the calcified and

ulcerated aorta, which conceivably might have become quiescent under

appropriate treatment. But, although the brain was almost if not quite

normal in the gross, and although its membranes showed practically no

lesion, treatment would not have been very promising. To be sure, the

exudate might have been cleared away if the spirochetes responsible

therefor had been destroyed by treatment. Yet the destruction of the

giant cells of origin of the pyramidal tract fibres to such an extent as

in this case could hardly have been compensated for by any known

process. So far as we are aware, the destruction of considerable numbers

of the smaller association elements of the brain is subject to the

compensation of other elements of the nervous system, which conceivably

might be re-educated or newly educated to perform certain processes. The

histological picture in a case like that of McDevitt accordingly leads

to the hypothesis that so well marked a spastic paresis, even in the

presence of otherwise favorable signs, would be of especially baneful

portent therapeutically.

=NEUROSYPHILIS with total duration of symptoms twenty-two days. The

comparatively MILD BRAIN LESIONS, INFLAMMATORY AND NOT DEGENERATIVE

in type, suggest the possibility that therapy might have been

successful. Autopsy.=

=Case 100.= Jacob Methuen, 35, was a carpenter from Newfoundland. He was

working upon a certain Thursday with his brother, who noticed that Jacob

was lifting the tools about in an unusual manner and talking strangely

to his fellow workmen. He fell asleep, going home in the street car, and

said afterward that he felt dazed and peculiar. He talked all kinds of

nonsense to his wife upon arrival. Methuen remained in bed next day,

fancying he was going to die, calling his family together, and saying

good-bye to them. He remained in bed all through the next day, but on

Sunday appeared better,—more active, and in fact quite natural. He slept

only an hour Sunday night, calling to his wife that it was time to get

up. On Monday he began to be irritable to his wife, and accused her of

flirting with his brother and intending to elope with him. He struck his

wife several times, and when two brothers came to watch him, accused

them both of trying to steal his wife, and struck them. Tuesday he

remained in bed until late at night, when he arose and tried to assault

the family.

It seems that another brother of the patient had died but eleven days

before his admission to the hospital and five days before the onset of

Jacob’s symptoms. Since his brother’s death he had been dwelling upon

religious matters, and in fact the day after his brother’s death, he

waked up during the night, saying that he was too happy to sleep, that

he heard the Master’s voice, and at times the devil’s voice; that there

was to be a modern miracle and his spiritual life from now on would be

different.

Eleven days after admission to the hospital, Methuen died, making a

total duration of symptoms, beginning at his brother’s death, of 22

days.

=NEUROSYPHILITIC LESIONS=

=LESIONS OF THE SECONDARY PERIOD=

(1) INTERSTITIAL ENCEPHALITIS OR MYELITIS

(“meningitis”)

(2) PARENCHYMATOUS ENCEPHALITIS OR MYELITIS

(“encephalitis,” “myelitis”)

=LESIONS OF THE TERTIARY PERIOD=

(1) CHRONIC INTERSTITIAL ENCEPHALITIS OR MYELITIS

(“gummatous meningitis”)

(2) CHRONIC PARENCHYMATOUS ENCEPHALITIS

(“dementia paralytica”)

(3) CHRONIC PARENCHYMATOUS MYELITIS

(“tabes dorsalis”)

“We have shown that the central nervous system is affected by

syphilis at the same periods and in the same manner as are other

internal organs. In addition the ‘parasyphilitic’ lesions are also

of a typically syphilitic nature, being directly comparable to the

parenchymatous affections found elsewhere in the body. They are

‘tertiary’ lesions differing only from the so-called ‘gummatous’

processes in the central nervous system in that their localization

is in the parenchyma while that of the latter is in the

interstitial tissues.”

McIntosh and Fildes, 1914

CHART 22

=Physical examination= showed a man 5′ 9″ tall, weighing 149 pounds,

rather pale and poorly nourished, with a somewhat enlarged heart and no

evidence of venereal disease.

=Neurologically= there was a slight facial and digital tremor, but

otherwise no symptom or reflex disorder except that the tendon reflexes

were generally increased; the knee-jerks especially were very vigorous.

There was no speech defect. His handwriting was fairly legible.

The patient was very noisy and uncontrollable, tearing clothing and

biting, striking the attendants, refusing food, talking rapidly, loudly,

and incoherently. His manner suggested auditory hallucinations but no

positive evidence of these was obtained. His clothes could not be kept

on him. The following is a sample of his reactions: As the examiner

entered, the patient stood stark naked and glaring. He started to talk

as follows: “Methuen,—I, Saviour, come to life and ought to die—— Now I

lay me—— Now I die—— The heart beats—— No, I ain’t going to die—— I am

going out soon. I want my clothes—— You can’t hold me; I am strong.”

(Struggles violently with the attendants.) “I am God. God. I know you,

you can’t fool me.——I am here——I can do you all. How many doctors are

there here?” (Struggles violently. Looks at examiner.) “He is writing

something. Sir, you can’t fool me in a million years. Do you understand

that, doctor? You can’t fool me. Write all the prescriptions you want

to. Ten thousand years; you hear that, doctor? Ten thousand years. You

can’t fool me; ten thousand years. Ten thousand years are but a day for

the spirit of the Lord,” etc., etc.

The excitement continued unabated. The patient became entirely

disoriented, and finally almost unable to move. He lay in bed trying to

talk and muttering broken gibberish, still attempting to struggle to the

extent of his limited strength.

The =autopsy= showed no sign of lesion (brain weight 1380 grams),

unless, perhaps, the occipital regions were slightly firmer than the

rest of the brain. Death was apparently due to a bilateral pneumonia,

bronchial type. There was an acute splenitis. The only chronic lesions

of the body were a bilateral chronic adhesive pleuritis and a slight

sclerosis of the arch of the aorta.

[Illustration:

Paretic neurosyphilis (“general paresis”) macroscopically normal,

microscopically characteristic. Treatment does not have to face

massive destructive processes already complete.

]

=Microscopically= there was a distinct though mild degree of

lymphocytosis of the perivascular spaces in many regions. Somewhat

extended _search failed to reveal plasma cells_, and it is certain that

if plasma cells existed, they must have occurred in very small numbers.

Here, then, was a case of DIFFUSE NEUROSYPHILIS (with brain picture

consistent) with symptoms lasting but 22 days and with an appearance of

acute mania. It is to be noted that this case arrived at the hospital on

the eleventh day of his symptoms. The case occurred long before the

development of the temporary care system in Massachusetts. It is

probable, or at any rate possible, that he would have been brought to

the hospital far earlier, say, upon the sixth day, had the modern

temporary care system been installed at that time. The routine W. R.

examination would then have been made. With more effective hydrotherapy,

it is possible that the patient’s life might have been prolonged and

that treatment might have been effective. So far as we can see, the case

would have been a singularly good one for treatment despite the

practical unmanageability of the case under ordinary home treatment, and

even under hospital conditions where modern hydrotherapeutic appliances

are not available.

=PARETIC NEUROSYPHILIS showing very MARKED MENINGITIS, suggesting

that therapy might have produced improvement. Autopsy.=

=Case 101.= We report the case of John Baxter, a boat tender of 48

years, because this particular victim of PARETIC NEUROSYPHILIS seems to

have had the most markedly thickened and altered meninges in our whole

series. Of course, the therapeutic theory upon which we now proceed in

the treatment of non-paretic and possibly even of paretic neurosyphilis

is that, other things being equal, the meningitis can be removed by

treatment, or in the course of treatment, so that the degree of ultimate

recovery rather depends upon the condition of the brain substance itself

than upon the condition of the meninges. Here, at all events, is an

example of the most highly meningitic neurosyphilis that we have seen.

Curiously enough, two of Baxter’s brothers were also patients at the

hospital at which Baxter died, and a number of the other members of the

family are reported as “nervous.” It seems that at 35 Baxter began to

drink heavily and had never given over the habit of alcoholism.

Upon admission to the hospital, in fact, he showed a sufficiently

typical picture of delirium tremens. His consciousness was clouded, he

had vivid visual hallucinations and was very apprehensive.

His heart was enlarged to the left; the pulse, 120, was of increased

tension and irregular; there was peripheral arteriosclerosis; the teeth

were poor; the tongue coated; and the mouth foul. The urine showed a

trace of albumin and rare hyalin casts.

=Neurologically=, the gait was somewhat unsteady, there was an extreme

tremor of the whole body, including the tongue and fingers. The Romberg

sign was negative although there was marked swaying. The pupils were

equal and reacted normally; the knee-jerks were markedly exaggerated,

the arm reflexes somewhat exaggerated. The remainder of the reflexes

upon systematic examination were negative.

[Illustration:

A high degree of chronic leptomeningitis. Pia mater thick, opaque,

concealing brain. In paretic neurosyphilis (“general paresis”).

]

Upon arrival, Baxter was put to bed, but he barricaded his door and

fought with the attendants. The tremor increased, the hallucinations

were both visual and auditory. After a few days, Baxter became so weak

that he could not move. He refused to eat for a period of two days,

explaining in whispers that he did not wish to be poisoned; a voice had

told him the food was to be poisoned. The voice was of agreeable tones,

probably belonging to a lady; it did not speak, but sang to him. The

clouding of consciousness failed to clear up, as in delirium tremens, so

that, though patient was admitted March 3d, it was hardly possible to

speak freely with him until more than a month later, April 9th. A

good-natured conversation would run as follows:

“What is your name?” “Baxter.”

“First name?” After long pause, “Don’t know.”

“John?” Pause of 7 seconds, “Yes, I think it is.”

“How old are you?” “There are legs——there is a body——up to here——”

“Say the alphabet.” Term not understood.

“Say the _a_, _b_, “Oh yes; a, b, c, d (long pause), e, f; I cannot

_c_.” say it, I did not have much education; I am not

intelligent.” (In point of fact, the patient had

a good grammar-school education, and had long

worked as a clerk in a grocery store, with good

wages.)

There was some

speech defect.

Soon the hallucinatory phase passed, and the patient remained in a

cloudy and disoriented state, inaccessible, rarely speaking, and

gradually failing physically. Death occurred about three months after

admission (pulmonary symptoms).

In estimating the duration of the process in John Baxter, we must take

into account that he left the grocery business and became a hard-working

but poorly-paid boat tender at about 35 years, at the same time that the

alcoholic habit began.

The =autopsy= showed that death was due to bronchopneumonia with

pleurisy. There were in the body a variety of chronic lesions, such as

gastritis, colitis, epididymitis, splenitis, parietal and valvular

endocarditis, prostatitis, chronic appendicitis, and some mesenteric

lymphnoditis. The heart was somewhat hypertrophied. There was a slight

diffuse nephritis with cysts, emaciation, and decubitus. The calvarium

was thick and somewhat dense. The dura was thickened and adherent, and

the pia mater,—as above stated, the most thickened and altered pia mater

in our series,—is described as everywhere thickened, of a brownish gray

and white color, especially over the vascular lines, and as showing

small white areas of deeper thickening scattered over the surface, but

most markedly over the sulci, and not as a rule over the crowns of the

gyri. There were also yellowish brown spots with a suggestion of fibrin

over the lateral aspects of both hemispheres. The vessels at the base

were not remarkable in the gross. The brain weighed 1220 grams, and

appeared to be of darker color than usual.

=Some cases of PARETIC NEUROSYPHILIS (“general paresis”) have so

much BRAIN ATROPHY that it is not possible to expect much

improvement through antisyphilitic therapy.=

=Case 102.= Theodosia Jewett, dead at 58 years, showed the most

remarkably wasted brain in a long series of victims of paretic

neurosyphilis. We present her case to emphasize what therapy must face

in certain instances, but would recall the fact that exceedingly few

such wasted brains have come to our attention in cases dying in the

institutions of Massachusetts.

Mrs. Jewett, a housewife, whose parents died of shock, and one of whose

two brothers also died of shock, was a normal child and schoolgirl, and

worked as dressmaker until she was married, at 24, to a grocer, by whom

she had two children. At the age of 46, Mrs. Jewett began to suffer from

so-called “nervous prostration.” The attack lasted some two years, but

there were no psychotic symptoms beyond worry and insomnia. The

menopause occurred at 52, at which time the first signs of psychosis

appeared, namely, a forgetfulness concerning familiar matters, such as

sewing, cooking, and the like. At 55, this amnesia had become so marked

that Mrs. Jewett could neither write nor tell time. She, however, was a

perfectly quiet and easily manageable patient, often subject to

drowsiness in the day.

Six months before her admission to the hospital, she began to suffer

from insomnia, failed to recognize her surroundings, and had a number of

crying spells. Restlessness had begun a month before admission; auditory

hallucinations developed in the form of imaginary conversations with

dead persons. A certain loquacity set in, and for a week before

admission, Mrs. Jewett became somewhat resistive.

=Physically=, the patient was sallow, poorly nourished, with pale mucous

membranes, peripheral arteriosclerosis, no teeth, muscular feebleness,

tremor of hands and tongue, and active knee-jerks. =Mentally=, the

patient was depressed, talked to herself, assumed a supplicating

position, suddenly altered her attitude, and was very tremulous. Her

talk was low, mumbling, and incoherent, for the most part composed of

answers to her own questions. Sometimes there was a curious difficulty

in speaking, such that the lips moved but no sound emerged; but for the

most part there was no difficulty in uttering words. The patient either

could or would not write. Only when the attention was secured by

speaking to her sharply was she apparently able to understand questions,

and the answers to these sharp questions came spasmodically and as if

interrupting her own thoughts. Nor was it ever possible to obtain a

repetition of the same answer.

The patient died in exhaustion, with pulmonary symptoms three weeks

after admission.

The =autopsy= which was performed 3½ hours after death showed the

following points of interest:

The heart weighed 210 grams. There was marked thickening of the aortic

valve. The coronaries were slightly thickened.

The lungs were slightly adherent to the chest wall at the apices and

posteriorly. The right lung was consolidated in the lower two lobes

posteriorly and the bronchi exuded pus; the left lung was not

remarkable. There was a chronic splenitis.

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

with yellow.

Combined weight of the kidneys 195 grams. The capsules were adherent,

tearing the cortex when stripped.

The diploë were well marked. The dura was not adherent. The pia was

slightly thickened and raised from the cortex by a large amount of

subpial fluid (showing atrophy of the cortex). The pial vessels were

injected, more markedly so on the left side. The arachnoid villi were

reported as moderately developed, especially along the longitudinal

fissure.

The brain was rather soft in all regions. The weight was 1045 grams.

According to Tigges’ formula the weight of the brain should be

approximately 8 times the body length in centimeters. The length in this

case was 158 cm., therefore, according to this formula the weight of the

brain should have been 1464 grams. The difference of more than 400 grams

is evidently a loss to be accounted for by atrophy, a very heavy loss.

[Illustration:

Perivascular exudate (low power) in atrophic cortex from case of

general paresis.

]

[Illustration:

Markedly atrophic cortex, but without local perivascular exudate.

]

1. Was the “nervous prostration” at 46 of syphilitic origin? One

cannot give a categorical answer to this question. The high

incidence of shock in the family suggests poor stock in which a

psychoneurosis is not an unusual phenomenon. The presence of

syphilis might act as a debilitating factor or _agent

provocateur_, if it were not to cause any demonstrable brain

lesion. As pointed out in the case of Harrison (9), however, it is

not unusual in neurosyphilis to find a history of symptoms

occurring years before the final breakdown and symptoms frequently

not recognized as of neurosyphilitic nature.

2. Does the fairly long duration of the psychosis (at least 3 years)

explain the marked atrophy? Cases having symptoms even much longer

than three years at times show relatively very little atrophy, so

that this factor in itself cannot be said to explain the

tremendous destruction of tissue.

=The THERAPY OF NEUROSYPHILIS has to face not merely variations in

the degree of brain wasting and in the degree of meningitis, but

also variations in the topographical distribution of lesions.

Autopsy.=

=Case 103.= To bring out this point we may instance the case of Alfred

Weed, a victim of PARETIC NEUROSYPHILIS, dying at the age of 48 years

after a course of about seven years. The following is an abstract of the

clinical history:

A. W. suffered from lues some 24 years before his death at Danvers

Insane Hospital in 1907. There is no account of insanity in his family.

The patient had been undergoing mental changes for six years before

death. At the age of 42 began to take interest in socialism and

spiritualism. Would become excited at times and was observed to talk to

himself. At times it seemed that he was reacting to visual

hallucinations. After eight months he became depressed and apprehensive

and developed delusions of poisoning.

On admission to the Danvers Insane Hospital in June, 1902, the subject

was found to be ataxic, falling in the Romberg position. Pupils were

equal but of pin-point size. There was tremor of the facial muscles. The

knee-jerks were absent. Speech was ataxic. Memory defective. Depressed.

Thought he was to be punished. Refused to eat.

Later in the year of admission, patient became more negativistic. He

refused to have his clothes brushed. His answers were slow. Mental

arithmetic was correctly but slowly done. During January, 1903, the

patient was apt to be active and talkative for a time, and then his

attitude would suddenly change to one of silence, resistivement and

untidiness. From time to time he would be querulous and sulky. In

August, 1903, the patient became weaker and could walk with assistance

only. Paralysis developed in the left facialis region and in the left

external rectus. Pupils were still small, but the left had become

smaller than the right. Light reaction tests unsatisfactory. Knee-jerks

could not be obtained.

In December, 1903, the patient was untidy and helpless, lying with his

thighs and legs flexed. The limbs were spastic on passive motion. In

1905, the pain sense of the legs was found lost and the pupils were

small and stiff. The protruded tongue was deflected to the right. The

right labial fold was more prominent than the left. Knee-jerks remained

absent. Ataxia was extreme.

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

1. Ataxia of the legs.

2. (Probable) Diminished sensibility in the legs.

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

4. Paralysis of left facialis.

5. Paralysis of left external rectus.

6. Tongue protruded to right.

7. Right elbow jerk greater than left.

8. Knee-jerks absent.

The cause of death was bronchopneumonia. The walls and valves of the

heart showed a few chronic changes. There was a marked splenitis and an

atrophy of the liver. The kidneys showed numerous depressed scars. The

arch of the aorta was somewhat sclerotic. The following is a full

description of the head findings which we present by way of comparison

with other cases. Note especially the cerebellar, dentate, and olivary

changes. Note also the fact that palpable sclerosis is demonstrable over

a far larger area than atrophy, so that we may almost safely conclude

that the process of induration sometimes precedes that of atrophy. One

gets the impression from the extent of visible atrophy and tangible

induration in this case, that a possible therapy would have not merely

to clear the perivascular spaces of cells and spirochetes, but would

also need to arrest the indurating and wasting process. Nor could any

therapy deal effectively with the superior frontal and upper central

atrophy of the cerebrum of this case, or with the olivary and cerebellar

lesions.

=Head=: Hair thin at vertex. Scalp normal. Calvarium thin and dense.

Dura mater slightly adherent to calvarium at vertex. Sinuses normal.

Arachnoidal villi well developed. Pia mater of anterior and central

regions contains an excess of fluid. The pial veins well injected.

The =pia mater= exhibits one unusual lesion: Faintly yellowish brown

spots of miliary and slightly larger size are scattered irregularly in

clusters over the vertex. These miliary pial macules are observed

especially over the posterior third of the left superior frontal gyrus

(a group of twelve or more). Two are seen in the pia mater of the right

superior frontal gyrus. One is seen in the upper part of the left post

central gyrus. The upper end of the right postcentral gyrus contains

three macules.

Besides these brownish macules, the pia mater also shows focal white

thickenings which resemble the more frequent appearances of chronic

fibrous leptomeningitis. The white thickenings are of irregular size but

are, as a rule, larger than the macules above mentioned. They occur, as

a rule, over the sulcal veins and are most frequent in the anterior

region.

The vessels at the base are normal. There is no evidence of pial

thickening at the base of the brain. =Brain= weight, 1265 grams. There

is visible atrophy of both superior frontal gyri and of the upper

two-thirds of both central gyri. The extent of palpable sclerosis

surpasses that of visible atrophy. Palpable increase of consistence is

shown by the prefrontal, orbital (more marked on left side), frontal,

central, hippocampal and occipital regions. The temporal cortex is of

normal or slightly reduced consistence.

Section of the cerebral cortex shows everywhere preservation of the

cortical markings. The sclerosed areas show a diminution in depth of the

cortex, which is more marked in the left prefrontal region. The white

matter of the centrum semiovale of the prefrontal and occipital regions

on both sides shows an increase of consistence. The cerebellar cortex

also shows variations in consistence. The clivus and lobus cacuminis and

the posterior half of the inferior surfaces of both cerebellar

hemispheres are firmer than normal. The laminæ of the left clivus are a

trifle narrower than those of the right. There is visible extensive

atrophy of the laminæ on both sides of a fissure in the middle of the

left lobus cacuminis. In the coördinate portion of the right cacumen

there is a similar process which is less marked. The dentate nuclei are

firm. The olives show an increase of consistence, equal on both sides.

The left olive shows on section a crowding together of its folds in the

middle part of the upper limb.

Spinal cord was not remarkable.

=Summary=:

Adhesive pachymeningitis

Chronic fibrous leptomeningitis

Miliary pial macules

Cerebral atrophy

Cerebral sclerosis

Cerebellar atrophy and sclerosis

Bronchopneumonia

Chronic splenitis

Nephritis

Aortitis

=It is generally recognized that DIFFUSE NEUROSYPHILIS

(“cerebrospinal syphilis”) frequently is cured through

antisyphilitic therapy. Example. Mental improvement, in one month;

recovery from paralysis, ten months.=

=Case 104.= John Edwards, a man of 28 years, well developed and

nourished, with general enlargement of glands and skin lesions, came to

the hospital in a stuporous condition, with evidences of a complete

hemiplegia.

According to the wife, Edwards had had a chancre of the lip about a year

before, for which he had been treated with an intravenous injection,

presumably of salvarsan, and also presumably with mercury. The lip

lesion had then disappeared. For a month before admission, Edwards had

had headache and dizziness, for which he was given pills and drugs.

There had also been difficulty with speech and numbness of the left arm

as far up as the elbow, but this paresthesia had quickly disappeared.

The hemiplegia was of only a few days’ duration. After a feeling of

nausea and vomiting, the patient had fallen with left-sided paralysis.

Afterwards, he had shown mental peculiarities, eventually becoming

noisy, hard to manage, and appropriate for hospital care.

The =physical examination= showed a variety of increased reflexes,

including ankle clonus on the left side.

The question might arise whether this case was one of hemorrhage or

thrombosis, and the facts about the onset of the hemiplegia are

inadequate for a decision. However, at so early an age, the probability

of syphilis is large and the history of labial chancre was quite

suggestive. If we may conclude neurosyphilis, the diagnosis of

thrombosis rather than rupture of blood vessel is likely. The laboratory

tests bore out the diagnosis since the W. R. of serum and fluid both

proved positive; the gold sol reaction was syphilitic; there were 176

cells per cmm.; there was excess albumin, and a positive globulin

reaction.

=NON-PARETIC NEUROSYPHILIS=

=DIFFUSE NEUROSYPHILIS,

MENINGOVASCULAR PARENCHYMATOUS,

CEREBROSPINAL SYPHILIS=

CASES SYSTEMATICALLY TREATED 13

CLINICAL RECOVERY, C.S.F. NEGATIVE 11

UNIMPROVED 1

UNIMPROVED, BUT C.S.F. NEGATIVE 1

MASSACHUSETTS COMMISSION ON MENTAL

DISEASES,

_November, 1916_

CHART 23

The outcome in such a case is dubious. If death does not occur soon,

recovery is not impossible under treatment. At all events, a

considerable improvement is likely.

Edwards was given bi-weekly injections of salvarsan, intramuscular

injections of mercury salicylate, and doses of potassium iodid,

averaging 100 grains, three times a day. Under this treatment, he slowly

recovered and became mentally clear after a few weeks. The paralysis

seemed complete and permanent. Even after three or four months, there

was absolutely no change in the condition, and Edwards was quite unable

to move either arm or leg. Meanwhile, the spinal fluid had become

practically negative to all tests.

_Treatment_ was somewhat optimistically continued and was _rewarded at

the end of ten months_ with marked improvement such that the patient was

able to stand on the paralyzed leg and move the arm to a certain degree.

This improvement is still continuing. The spinal fluid and the serum

have remained negative to laboratory tests.