Chapter XVII: Introduction (9)

1. Could the diagnosis be rendered in this case without a lumbar
puncture? In the first place, the emaciation is not entirely
characteristic. The pupils react normally to light. Probably such
a case might well have been regarded as one of tabes dorsalis in
former days, or even at the present time, if a lumbar puncture had
not been resorted to.

2. Could this case possibly have been one of tabes dorsalis with
negative spinal findings? Such cases have been reported
frequently, but, unlike the present case, are apt to be of
long-standing and non-progressive, in which the active
inflammation is no longer present. The negative findings would not
be consistent with tabes, in which the symptoms are of short
duration and of sudden onset.

3. If the serum W. R. had been positive would the diagnosis have
been neurosyphilis? We are loath to make the diagnosis of spinal
syphilis when the spinal fluid is normal. Syphilis may produce a
marked anemia, however, and thus produce symptoms such as shown by
Mrs. Brown. It is even possible that such is the explanation of
this case, taking into consideration the suggestive findings in
the husband. However, there is insufficient evidence to make such
an hypothesis rock firm, and we do not more than suggest it.

=Atypical case of CONGENITAL NEUROSYPHILIS—peculiar mental state.=

=Case 76.= James Seabrook, 20 years of age, obviously showed a number of signs of congenital syphilis. The =physical examination= disclosed an old scar and indentation in the right mastoid region, another on the right side of the neck, another on the posterior surface of the right forearm, and two on the outer surface of the right upper arm. The lesions were about the size of half a dollar. There was a saddle-shaped nose and a perforation of the palate and uvula; there were palpable cervical and axillary glands, small but numerous. There was a dulness in the region of the right scapula, and slight dulness on both sides behind. There were loud whispering and piping râles and bronchial breathing throughout the chest, more marked on the left; there was much coughing, and the sputum was at times blood-stained. The pupils were irregular but reacted perfectly. The left knee-jerk was slightly more active than the right. The W. R. in blood and fluid was negative; the gold sol, globulin and albumin tests were negative. There were, however, 56 cells per cmm. in the fluid.

We learned that the patient had had several spells of great excitement, with pounding on the door and a desire to fight bystanders. There were spells of headache and vertigo. =Mentally= the tests showed him to be subnormal.

The =diagnosis= Of CONGENITAL SYPHILIS seems established; possibly the pulmonary condition is syphilitic. The mental subnormality as well as the abnormal traits and episodes are probably to be accounted for on the basis of syphilitic involvement of the brain.

1. Are the headache and vertigo connected with syphilis? This is
perhaps suggested by the pleocytosis in the spinal fluid.

2. How shall we explain the negative W. R.? This patient had
received, shortly before his entrance to the hospital, salvarsan
and mercury. Possibly the drug treatment has little or nothing to
do with the negative W. R.’s since they not infrequently grow
weaker as congenital syphilitics grow older.

3. What is the explanation of the spells of excitement? Compare the
spells of excitement in a form of neurosyphilis described by
Kraepelin, namely: syphilitic paranoia, discussed in the case of
Bridget Collins (59).

4. Is treatment indicated considering the W. R.’s to be negative in
blood and fluid? Despite the negative W. R.’s in this case
treatment is strongly indicated on account of the pleocytosis.
This would seem to indicate that there is an active inflammatory
process in the cerebrospinal axis, and it is more than probable
that this process is syphilitic. How much real improvement of the
symptoms would result from antisyphilitic treatment it is
impossible to prophesy. Every case is a special problem, and this
case is very unusual in showing a pleocytosis in the absence of
other indications of syphilitic nervous disease, _viz._, globulin,
albumin and W. R.’s.

=CONGENITAL NEUROSYPHILIS resembling an undifferentiated case of
FEEBLEMINDEDNESS—actually PARETIC.=

=Case 77.= John Friedreich, a 7–year old boy, was brought to the Psychopathic Hospital by agents of a charitable society, who found him a neglected child and quite evidently a subnormal one.

The dominance of syphilis in the situation was clear. The boy’s father had died but a few months before of syphilitic heart disease, from which he is said to have suffered for five years. The boy’s mother (the parents were first cousins) had also been treated for syphilis and was excessively alcoholic. The first child of this union—a girl—had died at 6 years, of a disease diagnosticated spinal meningitis. The history indicates that syphilis was acquired after the birth of this first child; but in any event it is possible that the meningitic condition of which the first child had died was syphilitic. The second pregnancy terminated in a stillbirth; the third issued in a girl, who died two weeks after birth of what was termed “inward convulsions.” The fourth pregnancy resulted in a miscarriage; the fifth in our patient, John Friedreich. The sixth pregnancy resulted in a girl, now 5 years of age, who is apparently normal. (Her W. R. was negative and she shows no stigmata of syphilis.)

The patient, John Friedreich, at some very early age had a rash on his body diagnosticated as syphilis. He also had many seizures called fainting spells. Ever since birth he had been taking mercury pills. He had not learned to talk until his third year, and was able then to say only a few disconnected words. In fact, John has never been able to talk in complete sentences, mumbling much that is quite unintelligible. However, he walked at 15 months in a normal fashion and nothing peculiar in his gait was noted until he was 5 years old, when he began walking on his toes, particularly those of his left foot. Shortly thereafter, the seemingly inevitable trauma appeared; John fell out of a window and severely injured his left leg, whereupon the peculiarity of toe-walking became more pronounced and associated with a limp.

Juvenile paresis. 7 years.
]

The patient strikes one =physically= as having the development of a child of about five years (actual age, 7). There are a few lymph nodes palpable in the anterior triangles of the neck. The dilated and slightly unequal pupils react neither to light nor accommodation. There is practically complete deafness; loud sounds are not at all noticed.

Withal, the child in a general way presents a somewhat attractive appearance, being very playful and mischievous, lying about on the floor and playing with whatever comes to hand, talking to himself or making a few indistinct remarks to the bystanders. He walks awkwardly, on the toes of the left foot. He pays little or no attention to his toilet and needs to be dressed and cared for in all ways. He is quick-tempered and at times very difficult to manage.

There was, of course, little doubt of the =diagnosis= of CONGENITAL SYPHILIS and of FEEBLEMINDEDNESS. The W. R. was positive both in the blood and in the spinal fluid. The gold sol reaction of the fluid was of the “paretic” type; there were 44 cells per cmm. and there was a large excess of albumin and much globulin.

As to prognosis, there is doubt.

1. Is, or is not, this a case of juvenile paresis?

2. Is it, perhaps, a relatively permanent case of feeblemindedness
due to congenital syphilis? On the whole, on account of the spinal
fluid symptoms, we should be inclined to give the case a
relatively poor prognosis, namely, of death in a few years.
However, we may perchance be later surprised to learn that the
patient has lived on, at least into early adult age.

Note: Mercury tablets in some cases of congenital syphilis do not
seem effective. John Friedreich was treated most intensively by
syphilographers from birth.

Dr. W. E. Fernald in a personal communication stated that
syphilitic cases of feeblemindedness are rather those of the
imbecile and idiot groups than of the higher levels. This
statement emphasizes again that the true hereditary cases of
feeblemindedness are rather those of the higher group, whereas the
cases in which special causes have operated in the uterus or in
early life eventuate in idiocy and imbecility. However, such a
case as that of Friedreich shows that now and then a case of
feeblemindedness without evidence of neurological disorder and
looking in almost all respects like an hereditary case may be at
times produced by syphilis.

3. How often is the central nervous system involved in hereditary
syphilis? An interesting table bearing on this point is presented
by Veeder.[16] The table concerns the lesions in various parts and
systems of the body in 100 cases of late syphilis. It appears that
in 47, or approximately one-half of Veeder’s series of 100 late
cases, the infection developed some form of lesion of the nervous
system. As Veeder remarks, this result runs counter to the common
statements of pediatricians, notably of Holt.

Bones:
Periostitis tibia 4
Periostitis skull 1
Osteomyelitis 1

Joints:
Acute arthritis knee 8
Acute arthritis ankle 1

Skin:
Macular eruption 1
Condyloma anus 3
Gummata 3
Alopecia 3

Eye:
Interstitial keratitis 24
Choroiditis 1

Ulcerations:
Nasal 2
Laryngeal 1
Pharyngeal 1

Central Nervous System:
Mental deficiency 23
Cerebrospinal syphilis 14
Hemiplegia 6
Epilepsy 5
Spastic paraplegia 4
Chorea 2
Hydrocephalus 2

Miscellaneous Conditions:
Ozena 1
Enlarged spleen (only symptom) 1
Torticollis 1
Aortitis 1
Obscure abdominal pain 1
Obscure pain in legs 2
Endarteritis obliterans 1
Paroxysmal hemoglobinuria 1
Raynaud’s disease 1
Hutchinson’s teeth 4

=Juvenile paretic neurosyphilis. Quadriplegia.=

=Case 78.= Gridley Ringer, 15 years of age, had the facies of a congenital syphilitic, including Hutchinsonian teeth, rhagades of the face, and the so-called Olympic brow. No secondary sexual characteristics had developed. There was a marked speech defect. =Mentally=, Ringer was a low-grade imbecile. He had been born at full term, and delivery had been normal. There had never been other pregnancies. He had never developed normally.

The father admitted syphilis 23 years before, namely, 8 years before the birth of his son, but the father had been treated for several years and had been declared cured.

1. What would be expected in the spinal fluid of this case? Without
the history, it would perhaps be impossible to say whether the
case was one of a quiescent imbecility or one of juvenile paresis.
The spinal fluid of the juvenile paretic gives a picture identical
with that in the adult. The spinal fluid in this case showed a
positive W. R. (as did also the serum), a marked increase of
albumin and globulin, 115 cells per cmm., and a “paretic” gold sol
reaction. Accordingly, the diagnosis of GENERAL PARESIS was made.

2. What is the prognosis? The prognosis of juvenile paresis is
currently regarded as entirely grave. There is probably less hope
for improvement in juvenile paresis than in the acquired paresis
of adult life, since it seems to be a general principle that
congenital syphilis is always more difficult to cure than acquired
syphilis.

This case had seizures a few months after initial observation, and
the seizures were followed by a transient right hemiplegia. This
right hemiplegia was shortly followed by a left hemiplegia, which
remained permanently. Moreover, a few weeks later, a right
hemiplegia again developed, leaving the patient with complete
paralysis and aphasia. Death followed in six weeks.

3. What effects were shown in the parents? Following up the parents
was rewarded by the discovery that the mother was suffering from
nerve deafness, probably of syphilitic origin, and that the father
had recently begun to suffer from what he considered rheumatism,
but which on examination was shown to be tabetic neurosyphilis
(“tabes dorsalis”). This family again supports the hypothesis that
there is a strain of spirochetes especially prone to attack the
nervous system. Here it would seem that the syphilis acquired by
the father had infected the mother and been transmitted to the
son. In all three infected by the same strain or strains of
organisms the nervous system was involved. It is difficult,
nevertheless, to explain on this hypothesis why in one case the
disease took the form of tabes dorsalis, in the second, eighth
nerve involvement and in the third, paresis. This question of
strains is really more than academic because it enters deeply into
the question of treatment, as well as that of the suggested
increased viability of the neural strain.

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

=Case 79.= John Doran fell off the rear of an ice-wagon, at six years of age, and shortly afterward developed fits. It appears that John was not unconscious at the time of his fall, but that he complained of headache. Although the convulsions were fairly frequent at first, it appears that they later became rare and occurred only when the patient got into a temper. At the stage of exhaustion after violent excitement, John would fall.

=Physically=, at 9 years a fair development and nutrition were evident. There was a great exaggeration of the frontal bosses; the nose could not be said to be typically saddlebacked, yet there was a suggestion of a sinking of the bridge. The teeth slightly suggested the Hutchinsonian type, but only slightly. There was a slight roughening of the tibia, and there was a slight scar over either knee. The patient graded according to the Binet scale at 9 years, and he was regarded as definitely feebleminded.

The family physician states that, according to his information, the father contracted syphilis when the child was between three and four months of age, and that the mother also was infected at this time. However, the child had not been suckled except immediately after birth, and there had been no evidences, according to the family physician, that John had acquired syphilis.

Ordinarily, one might content himself regarding the case of John Doran as one of idiopathic epilepsy with mental defect or deterioration. However, the frontal bosses, suggestive teeth, the flattened bridge of the nose, the roughened tibiae, and the old scars, though singly not of great significance, collectively make one suspicious. Despite the family physician’s belief that John could not have acquired syphilis from the parents, the infection seems entirely possible despite the fact that no symptoms developed early thereafter.

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

1. What is the relation of trauma to this case of JUVENILE
NEUROSYPHILIS? Probably none.

2. What would be the effect of treatment? For a number of years John
Doran was lost sight of. He was, however, treated, according to
our information, with intraspinous injections of salvarsanized
serum, whereupon his convulsions shortly ceased. He has been
recently examined mentally once more, and still grades as
feebleminded. He still has violent outbreaks of temper.

3. Is such a case as Doran typical? Shanahan has investigated
conditions at Craig Colony. There were 22 out of 886 epileptics
(at Craig Colony) or 2½%, who showed a positive W. R. Nine of
these cases were regarded by Shanahan as cases of epilepsy
actually caused by syphilis. Viet had found 7%, and Bratz and Lüth
5% of constitutional epileptics to be syphilitic, but the data of
these German authors were obtained before the era of Wassermann
tests.

=Adrenal tuberculosis complicating juvenile paretic neurosyphilis
(“juvenile paresis”). Autopsy.=

=Case 80.= When James Arnold appeared at the Danvers Hospital in his 22d year, he looked as if he were but 12 or 14 years of age. He was excessively fat but of fair muscular development. The left eye diverged outward, and the left pupil was smaller than the right. An odd feature was a high degree of pigmentation of the skin of the genitalia and the groins (the axilla, the mammillary areas, and the oral mucosæ were free from pigmentation). =Physically= speaking, the patient was practically normal. =Neurologically=, however, there was much of interest, in the light of which the clinical history was of value.

It seems that after an apparently normal early childhood, the boy had begun, at the age of 11, to experience difficulty in carrying out every-day school tasks; and after this his mental capacity had slowly but progressively deteriorated. The deterioration was not merely intellectual, but the boy became dishonest and untrustworthy and developed a number of untidy and uncleanly habits, behaving at the age of 16, as the parents stated, like a child of six.

In his seventeenth year, the boy had been taken with a severe attack of what was regarded as an “attack of indigestion.” This attack ushered in a gradually developing muscular weakness, especially involving the limbs. By the age of 21 he had become irritable and the paresis was so extreme that the patient was unable to get in or out of a carriage.

This generalized muscular weakness was plain upon admission to the hospital though there seemed to be no actual paralysis. The patient was unable to walk in a straight line and Romberg’s position could not be maintained. Marked tremor was present in the hands and lips. There was bilateral impairment of vision and nystagmus. Reflexes and sensations normal. Speech was markedly affected, all syllables being very much slurred. School knowledge and memory for both recent and remote events very poor. The patient’s habits were very untidy. He was very emotional, easily made to laugh or cry; and in behavior, extremely childish.

Two months after his admission to the hospital, the weakness suddenly became extreme. He was constantly nauseated, refusing food. The face and hands were cyanosed and the heart’s action rapid, weak, and irregular. This attack lasted for a week and was followed by a period of improvement, during which, however, he still remained very weak and apathetic.

One month later he again became so feeble that he was unable to raise himself in bed. He complained persistently of feeling very “sick.” His temperature was elevated and there occurred the same train of circulatory symptoms observed previously, _viz._, rapid and tumultuous action of the heart, with cyanosis of face and extremities. He soon became unconscious, remaining so until his death, which occurred on the seventh day of the acute attack.

This case was under observation before the days of the W. R., yet clinically the case had been diagnosticated JUVENILE PARESIS. There was no history of the acquisition of syphilis or any likelihood of its acquisition. Considered clinically, many of the classical features described by Addison were present, _viz._, marked asthenia and apathy; severe and frequent gastro-intestinal symptoms (the disease probably commencing with the attack of so-called “acute indigestion” six years prior to patient’s death); attacks of extreme cardiac weakness with the characteristic small, low-pressure pulse. On the other hand, pigmentation of the skin (usually the most striking clinical feature) was limited to the external genitalia, these being colored a deep brown.

The most striking feature found at autopsy was a bilateral adrenal tuberculosis (caseation, giant cells, lymphocytosis, tubercle bacilli). The thymus gland was persistent (7×5×.5 cm.), whereas the thyroid gland was smaller than usual. The brain showed macroscopic and microscopic features entirely consistent with the diagnosis of general paresis, including lymphocytosis, plasmocytosis, irregular degrees of nerve cell destruction, and gliosis, with an especially characteristic microscopic picture in the frontal regions.

It may be of note to consider the degree of change undergone by a brain in 11 years or more of deterioration, and the following description of the head findings is therefore included:

=Head=: Hair abundant, dark. Scalp normal. Calvarium, weight 435
gm., transparent in bregmatic region only, elsewhere thick and
dense. The average thickness of the vertical plate of the frontal
bone is 7 mm. The frontal bone shows a moderate thickening and
hardening of the inner table with obliteration of diploë. Dura mater
moderately adherent to the bregmatic region of calvarium.
Arachnoidal villi moderately developed. Sinuses not remarkable. Pia
mater shows a moderate focal thickening with opacity, especially
along sulci. Vessels well injected. =Brain=: Weight, 1200 gm. The
brain shows marked focal variations in sulcation and consistence.
Spread on a board, the right hemisphere is obviously somewhat bigger
than the left. There is a difference of only 0.5 to 0.75 cm. on
measurement of the greatest circumference of the cerebrum, taken
from the median line superiorly to the median line inferiorly, but
the right hemisphere is throughout slightly more convex than the
left. Both postcentral gyri are much narrowed in their superior
portions, and the sulci posterior thereto are deeper than the other
sulci of the hemispheres. The sulci of the orbital surfaces are
asymmetrical and, on the left side, show a tendency to microgyria.
The cerebral hemispheres as a whole show a remarkable tendency to
slight protrusion of the border gyri; especially those of the two
poles, of the free edges along the great fissure, and most
strikingly the gyri at the boundary line between the inferior and
lateral surfaces. This _marginal prominence_ is slight but obvious
and is emphasized by a slightly paler color in some regions. The
cerebrum shows a general induration which is greatest in the frontal
tips and along the inferior borders of the lateral surfaces of the
hemispheres, especially right. The orbital surfaces are firm,
especially anteriorly and externally (prefrontal); the tips of the
temporal lobes are firm, and the superior temporal gyri are firmer
than adjacent gyri. The postcentral gyri are indurated more than the
other gyri of the superior surface. The hippocampal gyri are
likewise firmer than adjacent gyri.

=Cerebellum and pons=: Weight, 145 gm. The inequality of the two
hemispheres is more marked than in the case of the cerebrum.

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

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

There is no appreciable difference in depth. The diminution in
volume appears to be chiefly at the expense of the right clivus. The
inferior surface is firmer than the superior. The laminæ adjacent to
the horizontal fissure are firmer than the remainder of the
cerebellum. The pons is small.

There was also a lateral curvature of the spinal column, as well as characteristic adhesions between spinal dura and pia mater which are always suggestive of syphilis. For the rest, there were few findings of note: some adhesions of the left pleura, hypostatic congestion of the lungs, tracheitis, and chronic gastritis. There were four lobes of the right lung but it is doubtful whether this should be regarded as a stigma.

1. Can we separate the symptoms of Addison’s disease from those of
paresis in this case? The extreme cardiac weakness with a
characteristic, small low-pressure pulse is in point. The asthenia
and apathy are consistent enough with Addison’s disease as well as
with paresis itself. It would also be possible to ascribe the
gastro-intestinal symptoms to either disease.

2. Of what significance is the persistent thymus? Persistent thymus
has been observed in a few cases of Addison’s disease, but that it
plays any part in the symptomatology thereof is a matter of doubt.

3. How can the obesity be explained? It is of course of note that
the thyroid gland was small, but microscopically there were no
peculiar features in this gland.

4. Was the adrenal tuberculosis actually primary? Minute search
failed to reveal evidences of tuberculosis elsewhere unless we
regard the few adhesions binding the lower half of the lung to the
chest wall as indicative of an old tuberculosis. In particular,
the mesenteric lymph nodes were normal.

=Neurosyphilis? Secondary stage of syphilis.=

=Case 81.= Florence Fitzgerald, a woman 25 years of age, applied at the police station to be taken care of. She said she had been a prostitute for the last few months, was now ill, and wanted to reform. She appeared physically ill and was sent to the Psychopathic Hospital, where she remained at first almost mute, making answers chiefly by nodding the head. She gave the impression of daze or stupor, and in fact her condition was at first regarded as catatonic. This reaction, after a few days, changed and Florence became quite normal, giving a full account of her condition.

It seems that four months before going to the police station, she developed a chancre, which was locally treated. A careful physical examination showed a fine red macular eruption which was without much question a syphilitic roseola. The spinal fluid yielded a positive W. R. although other tests of the fluid were negative. Curiously enough, no physical sign of involvement of the nervous system could be discovered. We were inclined to regard the mental symptoms as partly due to the syphilitic intoxication, and partly due to a psychic reaction of the nature of defense. As for the positive W. R. in the spinal fluid, in early secondaries various observers differ as to the frequency both of the W. R. and of other changes, percentages being given that range from 25 to 90%. See case Caperson (45). It is of note that clinically there were symptoms referable to a syphilitic involvement of the nervous system; namely, marked headache and malaise. The headaches of the secondary period are frequently the result of meningeal involvement.

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

=Case 82.= Frederick Estabrook was a salesman, who, be it noted, had never had typhoid fever or any disease remotely resembling typhoid fever. He had acquired syphilis at 19; had married at 22; was the father of two healthy children (no miscarriages); had had a certain disturbance of bladder and rectum, but remained a successful salesman to the age of 28, when advancing tabes confined him to bed for a time. At 30, mental signs of PARETIC NEUROSYPHILIS developed, and death followed at 32, after an acute illness of a week.

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

At twenty-eight patient lost control of limbs and was confined to the house about two months, under medical care. Three months later he had regained partial control of his limbs but had lost all control of his sphincters. After another month he had returned to work, but did not work steadily and seemed to have lost ambition. In the summer of 1905, his mind became obviously altered. He grew indolent and extravagant and given to buying expensive and useless articles. Loss of interest in things followed, together with loss of memory for recent events, lack of insight into illness, delusions of persecution by wife, irascibility followed quickly by crying. Before admission to hospital, he was euphoric, drawling and tremulous in speech, sprawling in penmanship, alternately depressed and exalted in manner. Knee-jerks were absent, gait ataxic, pupils stiff to light.

The family history was negative with respect to insanity. All the family were reported as nervous. A brother died of peritonitis at twenty-eight, a sister of pneumonia under twenty. Another brother and sister are living. Father and mother died of heart trouble at about sixty-seven and sixty respectively.

The patient was at high school one year and was a fair student. Considerable tobacco was used, and some alcohol. Intoxication denied. There was no history of typhoid fever or other acute disease.

The patient on admission was sallow, poorly nourished, and flat-chested, with a slight lateral curvature. There was slight dulness over right apex in front and in right upper back. Voice sounds were increased over right apex in front and over whole right back. The right chest showed bronchial respiration throughout. The respiration in front of right chest was of an interrupted character. The liver seemed moderately enlarged. The urine showed a very faint trace of albumin. There were a few small nodes in right groin and a scar on dorsum of penis.

=Neurological Examination.= Slight swaying in Romberg position. Slight tremor of protruded tongue and extended fingers. Pupils irregular, left slightly larger than right. Left pupil reacted to light consensually, but not directly. Right pupil reacted very slightly to direct light, not consensually. Knee-jerks and Achilles jerks absent. Ankle clonus absent, abdominal and cremasteric reflexes brisk. Sharp and dull points were recognized in the legs with numerous mistakes. Vocal and facial tremor. Speech slow and drawling. Test phrases repeated well if care was taken. Consciousness clear. Orientation perfect. Calculating ability preserved. Many words omitted in writing. Penmanship clear but shaky.

Hallucinations absent. Memory of recent events poor. Associations of a logical or defining type. Patient denied various statements in commitment papers and had little or no insight into the mental side of his disease—slight euphoria.

After a month’s observation the patient was removed to a quiet ward and set to work a few days in the scullery. One night he began to yell as if assaulted and said later that he had an idea that he was going to die. Before three months had passed he had become untidy, disorderly, and imperfectly oriented.

The general degeneration continued rapidly. One week before death the temperature rose to 103 degrees F., and the patient succumbed to what seemed clinically like a bronchopneumonia. Unconsciousness two days before death.

Note with respect to history of typhoid.—Inquiries of his physicians, wife, employer, and brother tend to show conclusively that the patient never had a disease even remotely resembling typhoid fever.

The =autopsy= findings were as follows:

Acute conditions:

Hypostatic pneumonia, with early serofibrinous pleuritis and without lymph node swelling; =enlargement of mesenteric lymph nodes=; =acute cerebrospinal leptomeningitis=; multiple small hemorrhages of spleen.

Other findings:

=Scar of penis=; =sclerosis of aortic arch= (Heller’s type?) and slight coronary arteriosclerosis; =calvarium= thin and =dense=; =dura mater thickened= and adherent to calvarium; calcified arachnoidal villi; =chronic= cerebral and cerebellar =leptomeningitis=; =atrophy of frontal lobes=; =granular ependymitis=; =sclerosis of posterior columns= of spinal cord; emaciation; unequal pupils; slight parietal fibrous endocarditis, slight mitral sclerosis; gastro-intestinal atrophy; chronic cystitis; chronic abscess of prostate.

The description of the head findings is as follows:

Skin exceedingly loose, and the whole skull cap thinned. The diploë
are absent. Adhesion with dura easily separated. The dura somewhat
thickened, but not distended. Along the longitudinal sinus extensive
calcareous granulations adhere to it. The longitudinal sinus does
not contain blood, and the inner surface is normal in color. The pia
is extensively thickened and opaque and a general subpial exudate
exists which is more marked over the vertex where it lifts the pia
from the brain surface to the extent of three centimeters in
Rolandic, superior frontal, intraparietal, and mesial precentral
sulci on each side. The arteries at base are free from atheroma. The
temporal lobes are much bound down by adhesions, as is the
cerebellum. Post mortem softening is evident. The hemispheres show
no asymmetry, but the frontal convolutions are markedly atrophic.
The corpus callosum is united to the cortex by old adhesions and has
to be dissected away from it. Lateral ventricles contain some slight
amount of cloudy fluid, and the pia along the vessels is opaque.
Some granulations in ependyma. Brain weight, 1305 grams. Pons and
cerebellum, 195 grams.

Cord.—Dura much thickened, and the pia corresponds to its appearance
in brain with a like exudate. Cross sections of cord show sclerosis
of posterior columns.

Bacteriologically the _typhoid bacillus_ was cultivated _from the
meninges and from the swollen mesenteric lymph nodes_. The blood was
negative; the intestines were negative so far as lesions were
concerned.

The microscopic examination confirmed the clinical diagnosis of GENERAL PARESIS and of TABES, since there was not only an extensive chronic encephalitis, with the usual lymphocytic and plasma cell deposit and irregular gliosis, but also a well marked posterior column sclerosis, not unusual save in its extreme degree.

It might be surmised that some difficulty would arise in distinguishing the effects of paretic meningoencephalitis from those of the more recent typhoidal process. The well-known tendency of typhoidal processes to escape polynuclear exudation, at least until frank necrosis has set in, gave rise to the idea that the two mononuclear pictures—that of general paresis and that of typhoidal processes—might be confusing.

The picture presented by the meninges was scarcely what might be expected. Although numerous mononuclear phagocytic cells are everywhere found, yet the predominant picture is that of a polynuclear exudation.

The polynuclear leucocytes occur in greatest numbers in the tissue spaces, especially in the meshes of the lumbar arachnoid and in the spaces of the frontal and paracentral pia mater. In the lumbar region of the spinal arachnoid wide fields occur in which the cells are almost one hundred per cent polynuclear leucocytes. In places phagocytic cells occur, and in a few fields, even in the open tissue spaces, the number of phagocytic cells may arise to fifty per cent. Edema is a considerable feature in the meninges. Fibrin is found chiefly in the cerebral meninges and appears in numerous delicate strands in the tissue spaces.

Moloch, horrid king, besmeared with blood
Of human sacrifice, and parents’ tears;
Though, for the noise of drums and timbrels loud,
Their children’s cries unheard that passed through fire
To his grim idol.

Paradise Lost, Book I, lines 392–396

IV. MEDICOLEGAL AND SOCIAL

=Neurosyphilis in a public character: eloquence, reformatory
efforts, notoriety.=

=Case 83.= Major Isaac Thompson, M.D., was a character. He had been regarded as eccentric for many years prior to his death at 63. In fact, it seems that there had been more or less definite symptoms and signs about his fortieth year. The doctor himself had a ready explanation for his Argyll-Robertson pupils; he explained that he had had a peculiarly heavy smallpox at about the age of 27 (which would be about 1872).

The doctor had a good secondary education, he had gone through the Civil War as a hospital steward, went into business after the war, married, and then went to the medical school, graduating at the age of 34. He continued in practice for a dozen years, and then gave it up. For years he had been especially interested in certain literary lines and he had published any number of pamphlets, all of a somewhat striking description, often with a political color and intended to stir up reform measures. The doctor never bore a very good reputation, and years later it was recalled that certain books disappeared from libraries and their loss was almost certainly traced to Dr. Thompson. In general, however, he was considered to be a rather worthy local figure.

It is possible that a fall on the ice in his 61st year actually started the fatal process, since after that time the patient had difficulty in walking, and a few months later developed periods of excitement with peremptory insistence on obedience to his wishes. Whereas formerly the doctor had finished up one literary piece of work after another, he now began to do very scattering work. He appeared in public to denounce certain financial schemes with great force and unusual eloquence. His eloquence was greatly complimented, and these compliments induced the doctor to a remarkable crusade against a certain corporation; there was so much truth mixed with the fiction of his eloquence that he obtained a considerable following in his campaign. He wanted to start a bureau of information for the instruction of the public on these matters, and he planned to put up a building adjoining his own home for the accommodation of the various clerks and writers in this bureau. However, before the building had been actually started, an outbreak occurred.

One morning the doctor was very excitable and noisy over the telephone, ordering typewriters and giving directions to mechanics. He repaired to Boston in connection with certain resources that he supposed (and gave others reason to believe) had been supplied by the Government and by a large newspaper. One evening he returned very late. It appeared that he had had a fracas at a hotel and had knocked down one or two colored porters, acting as though drunk. Upon being put to bed, the doctor talked incessantly of religious matters, proposing to undertake a Sunday School class. His interlocutor did not exhibit a particular interest in this scheme, whereupon Dr. Thompson threatened him with violence. Police and doctors were called in and a constant stream of conversation lasted for hours. The patient was finally brought to Danvers Hospital upon representation by physicians, to whom he told that his luck had turned, that he was about to be made senator from the district, and that he and Roosevelt were going to break up the trusts, and that, as a matter of fact, he was a relative of Mr. Roosevelt.

Upon admission, the patient was a well preserved and well groomed man with gray hair and beard. He was somewhat pallid but his teeth were well preserved and well cared for, and there was little or no physical change except a slight hypertension. He claimed that he had suffered from kidney disease for some years, and there was in fact a trace of albumin in the urine.

=Neurologically=, the plantar and Achilles reactions could not be obtained, but there were no other reflex disorders except the bilateral Argyll-Robertson pupil. The doctor’s explanation for these stiff pupils, which he described as existing for many years, was frank and circumstantial, so that the unlikelihood of Argyll-Robertson pupils due to smallpox was rather frowned upon by him. Without entering upon a detailed description of the clinical symptoms and course of the disease which led to death a little over a year after admission, it may be said that the differential diagnosis lay between the expansive form of general paresis and a maniacal condition, presumably the maniacal phase of manic-depressive psychosis. From the data of a special staff meeting held upon the case, we learn that the diagnosis of manic-depressive psychosis was entertained more strongly than that of general paresis. Thus, for general paresis alone was the somewhat gradual onset with increasing excitement, accompanied by expansive delusions concerning unlimited finance, personal over-importance, and Argyll-Robertson pupils. Dismissing the Argyll-Robertson pupils from consideration, the diagnosticians were led to see in the constant motor activity displayed in conveying an enormous number of thoughts on paper, inconsistent talking with digressions, a manic-depressive psychosis. There was no amnesia and no other sign of mental deterioration. There was a certain improvement early in the hospital stay of the patient. Consciousness was clear and orientation perfect. The delusions themselves, though extravagant, were not inconsistent or fantastic. The hallucinatory disorder was hardly characteristic either of manic-depressive psychosis or of paresis.

The patient might be described as “interesting.” A good preliminary training with years of travel and variety of occupation, furnished him with a fund of knowledge. An excellent memory, prompt replies and repartee, endless digressions with voluntary return to the original topic, caused him to be an amusing and even instructive interlocutor. However, his commitment and confinement in the institution seemed always entirely wrong, and he expressed mixed feelings about the family, now being bitter against them, and again condoning their mistakes. The patient’s conduct was good and he was tidy in habits, and tried as far as possible to conform to the requirements of the hospital. The doctor showed a marked antipathy toward a certain male attendant, who had removed articles from his clothing upon admission and had reclaimed a book on rules and regulations. The doctor prepared a list of 327 different acts of abuse, lack of care, and insubordination which he said he had observed in the hospital.

In the last weeks of the patient’s illness, his ideas became more expansive and extravagant, dealing with a grapevine system of wireless communication and delusions of unlimited wealth. He would at times keep his room flooded with urine and water for the purpose of keeping down the plague which he said was infecting the hospital. Later he mixed food with urine and other ingredients, claiming that he was constructing an elixir of life.

The =autopsy= showed few changes of the calvarium or of the dura mater, nor was the pia mater more than slightly thickened and milky over the frontal poles, along the longitudinal fissure and over the sulci. There were fairly firm adhesions of the pia mater to the dura mater along the longitudinal fissure and over the frontal poles and at the temporal tips. The hemispheres were firmly interadherent, and the cerebello-pontine tissues were covered with a firm leptomeningitis. The floors of the ventricles were smooth and the basal vessels showed little beyond a few spots of sclerosis. There was a generalized increase of consistence. The frontal gyri were rather prominent with wide sulci, but upon section no very marked atrophy of the gray matter could be shown. The rest of the brain failed to show any flaring of sulci or any special evidence of cortical atrophy. The brain weighed 1250 grams; a possible diminution of 100 grams, considering the patient’s body length. However, it must be remembered that he was at this time 63 years of age.

=Microscopically=, the diagnosis of GENERAL PARESIS was confirmed on the basis of plasmocytosis, lymphocytosis, gliotic changes and nerve cell destruction. There was an unusual variation in the degree of the destructive process, which picked out, for example, certain regions of the right side for maximal lesion (cornu ammonis, gyrus rectus, and superior frontal gyrus).

If the patient’s own estimate of 35 years’ duration for his Argyll-Robertson pupils can be trusted (and in general his memory was extremely good), we may well conceive an unusual duration for the process in his case. There was, however, in the body at large no very marked degree of changes. There was a slight old tuberculosis. There was a slight interstitial nephritis, with cardiac hypertrophy and fibrous myocarditis. There was also a sclerosis of the mitral and aortic valves; there were chronic changes in the spleen, liver, and bladder; there was generalized arteriosclerosis of mild degree; there were two round gastric ulcers near the pylorus. The liver weighed but 800 grams, and its left lobe was somewhat rough.

This case is placed among the medicolegal and social cases because the phenomena that ushered in his last illness were mistaken by the local public for meritorious social reform measures. They were regarded as not markedly different from the variety of steps taken by the very active doctor in previous years; indeed the public eloquence that he displayed a year before his death was quite in line with previous habits, despite the suspicious over-brilliance of language. It is an important question, how far the eccentricity and literary overactivity of the latter half of the doctor’s total life can be explained on the basis of a mild syphilitic irritation of the nervous system. In this connection we are tempted to recall the suggestions of Mœbius concerning a portion of the literary products of Nietzsche. Our doctor was by no means so brilliant an exemplar of syphilitic literature as was Nietzsche, if we grant the hypothesis of Mœbius to cover our doctor’s case as well as that of Nietzsche. In the future, important studies of character change under the influence of syphilis will doubtless be made. With modern diagnostic methods, of course, the diagnosis would have been rendered almost at once in the case of Major Isaac Thompson, M.D., and much of his past life would have been brought under special review in connection with the syphilis which doubtless the blood serum or at any rate the cerebrospinal fluid would have shown.

This case illustrates but one of the many social complications arising as the result of paresis. When one recalls that the onset is often insidious and not correctly understood for a period of time, it is readily seen that many unfortunate acts may be committed by a patient. As hypersexual desire is not an infrequent early symptom and as judgment is early disturbed, loose morals may ruin the patient’s reputation. The poor judgment and expansive delusions often lead to foolish business deals wherein the patient’s family is left destitute. At other times the onset is sudden and then the danger of false commands or acts by a person in a responsible position, as a steamship captain, an engineer or chauffeur, may lead to loss of life and property.

=Sudden grandiosity: debts. PARETIC NEUROSYPHILIS (“general
paresis”): Question of liability.=

=Case 84.= Lester Smith was a salesman, 31 years of age, who, while on a business trip, accompanied by his wife, suddenly developed grandiose ideas. He originated a scheme of cornering the phonograph market. His prospects seemed so certain to him, that he hired an expensive suite of rooms in a hotel at something over $35 a day. As at the first presentation of his bill it was found that he had no money to meet these charges, he was taken into custody and at once transferred to a hospital for the insane, where it was discovered that he was suffering from GENERAL PARESIS.

1. What is the patient’s responsibility for these debts? Legally the
patient or his estate is responsible for debts accruing from
services rendered or goods received. As he is adjudged _non compos
mentis_ contracts entered into would not hold, and he would not be
considered liable for criminal acts.

Note: This case shows how dangerous paresis may be not only to the life and usefulness of a patient, but further how it may ruin a family financially. Mr. Smith’s little escapade used up all the money that he had been able to save in his life and when he was taken to a hospital his wife was left destitute.

=Suicidal attempt (?) by a neurosyphilitic.=

=Case 85.= At first Mrs. Annie Monks, a widowed seamstress, 50 years of age, did not particularly suggest syphilis. Mrs. Monks was sent to us from a general hospital. She had been found unconscious in her room, with gas turned on, and a diagnosis of gas poisoning was made. Mrs. Monks remained unconscious for 24 hours, and her apparent suicidal attempt seemed to warrant her being sent to the Psychopathic Hospital. Mrs. M., however, scoffed at the idea of any attempt at suicide, and claimed to have had no recollection of any such affair. On the contrary, she had gone to mass the morning of the day on which she was taken to the hospital, remembered well enough returning to her room but nothing of what followed until she woke up.

Mrs. Monks was not coöperative and would reveal few facts about her history. For years, she had had edema of the feet and palpitation of the heart (the heart was somewhat enlarged, with a double murmur in the aortic area, systolic louder, and a blood pressure of 160 systolic and 85 diastolic; clubbed fingers; palpable liver). She had been treated in the out-patient department of a general hospital for a number of months. We could obtain no evidence of mental impairment, particularly none of memory.

Aside from the heart lesions above indicated, the patient was fairly well nourished, with a slight enlargement of superficial glands, and was otherwise normal.

=Neurologically=, the slightly irregular pupils reacted poorly to light; the right knee-jerk could not be obtained, whereas the left knee-jerk was very active. Systematic examination revealed no other disorder except that the abdominal reflexes could not be obtained.

Here we have, in a cardiac patient, a possibly or probably accidental gas poisoning, and little to go upon for a profounder diagnosis than the sluggish irregular pupils and unilateral absence of knee-jerk.

The routine serum W. R. came through as positive. Following custom, we examined the spinal fluid, finding the W. R. here again to be moderately positive (strongly positive to 1 cc., moderately to 0.7 cc., and negative to 0.5, 0.3, and 0.1 cc.). The gold sol index was 1 2 2 1 0 0 0 0 0 0, which must be interpreted as syphilitic. There were 16 cells to the cmm., the albumin was 1+, and the globulin stood at 2+.

Here, then, we seem to have evidence of an inflammatory process of the central nervous system, and it is natural forthwith to be sceptical as to the accidental nature of the gas poisoning. Perhaps there was an attempt at suicide based upon a passing impulse, or perhaps there was a period of confusion in which the cock was not turned off.

In any event, we feel justified in making the diagnosis of cerebrospinal syphilis on the basis of the neurological and laboratory findings. On the whole, we are inclined to make a diagnosis of VASCULAR NEUROSYPHILIS with a moderate involvement of the MENINGES.

1. What is the outcome in such cases as that of Annie Monks? The
case somewhat resembled that of Martha Bartlett, who still
survives. The case of Annie Monks illustrates another outcome. A
few days after her admission, she became unconscious once more,
and upon recovery remained very much confused and aphasic,
moaning, and unable to handle herself well, although without
definite paralysis. Three weeks later the patient died, although
in the meantime strenuous antisyphilitic therapy was practised.
Death was sudden. We thought death due to cerebral embolism.

=Early delinquency and neurosyphilis in a juvenile.=

=Case 86.= Frank Johnson was 21 years of age when he was taken up by the police for threatening his sister with a revolver. The police thought he deserved an examination at the Psychopathic Hospital. The patient protested that he had threatened his sister only to frighten her because, he said, she nagged him and made him nervous. In fact, they had always had trouble as she had always nagged him and they had always fought together. Moreover, their mother always took the sister’s part. They had been troubling him for days, and at last Frank could stand it no longer. His sister had complained of the way he treated her dog. Moreover, Frank said he had not been feeling well; there had been some trouble with his stomach; and after one of the nagging attacks, he had taken out an old empty pistol to scare his mother and sister.

In these cases, it is good practice to consult the sister also. She said that Frank had always been very difficult to manage, unwilling to work, preferring to loaf about, spending every obtainable cent; he was once in a reformatory for several years, but not reformed thereby; recently given to drinking; at times acting somewhat peculiarly (sitting at the window with his hat on, refusing to move).

Further =mental examination= of Frank showed that he was properly oriented and in possession of a good memory, although he was quite obviously a liar. He lay about in bed at the hospital, saying that he was too weak to be up. He was a bit dull, at times not readily grasping ordinary questions.

=Physically=, Johnson was rather thin; the teeth were somewhat peg-shaped although far from typically Hutchinsonian. The pupils were unequal and irregular, and failed to react to light or even to accommodation when tested. The deep reflexes of arms and legs could not be obtained, though the superficial reflexes were present. For the rest systematic examination proved negative. Serum W. R. negative.

The first thought in such a case would be that the criminological diagnosis of delinquency would be sufficient. However, the pupillary disorder and the areflexia are suggestive despite the negative serum W. R. Resort was naturally had to lumbar puncture, whereupon a positive W. R. was found, a characteristically “paretic” gold sol reaction, globulin, excess albumin, and 134 cells per cmm. In short, it would appear that we must consider a diagnosis of JUVENILE PARESIS, and, in point of fact, the patient deteriorated rapidly from this time, becoming demented at the end of a few months.

1. How far are the early difficulties of management (leading to a
reformatory) due to syphilis? We should not dogmatically say that
there is a relation between the early delinquency and syphilis.
Still, it is not unusual to find emotional disorder and
instability as well as delinquency in congenital syphilitics.

2. What suggestion, if any, should be made to the patient’s
intelligent and seemingly normal sister, two years older? We
prevailed upon Miss Johnson to submit to the W. R. of the serum,
which was found, as in the case of Frank, to be negative. Frank’s
sister should undoubtedly submit to a lumbar puncture; but in the
present phase of mental hygiene, she would be difficult to
persuade.

3. How is it possible to find such a marked evidence of congenital
syphilis in a younger sibling with no evidence of syphilis in the
elder? In the first place, there may be a history of entrance of
syphilis into the lives of the parents between the pregnancies.
However, in other instances, there is no evidence of such
intercurrent syphilis, and contrary to the prevailing opinion it
is not so infrequent to find congenital syphilis in the younger
brother or sister of a normal person.

4. What can be said of treatment in such cases? In the first place
it is clear that delinquent cases should be tested far earlier for
the possibility of syphilis. Had this case been examined by a
neurologist or alienist many years earlier, it is probable that
the same pupillary signs and the peg-shaped teeth would have been
found, and that the hypothesis of syphilis might have been raised.
There is no good evidence as yet that these cases can be markedly
benefited by treatment.

=Neurosyphilis in a “defective delinquent.”=

=Case 87.= Vivian Walker, 22 years of age, was arrested on the streets of Boston for drunkenness. Upon arrival at the jail, she developed a series of convulsions, each lasting a very brief time, with loss of consciousness, frothing at the mouth, and jerky movements of the arms and legs.