Chapter XII: Introduction (4)

3. What can be expected from treatment in these cases of vascular
cerebral syphilis? The condition offers very little opportunity
for therapeutic results. However, antisyphilitic therapy is
indicated to prevent if possible further progress of the lesions.
Since the lesions are, however, vascular, and since it must remain
a question how far these vascular lesions are due directly to
spirochetal action, and since in any event it may be difficult to
reach the spirochetes thus active, perhaps it is best to place
most reliance on potassium iodid. In any event, potassium iodid
should be given. Salvarsan and mercury are also indicated. It is
common to warn against administration of large doses of salvarsan
in this type of case on the ground that further vascular ruptures
may be produced. (See Friedberg, 108.)

4. If we conclude that the aphasia of the Bartlett case is due to
vascular disease, can we conclude a relation between this vascular
disease and vascular tension? It is not safe to draw such a
conclusion. The Bartlett case itself showed low blood pressure. To
be sure, some cases of neurosyphilis show high blood pressure from
which one draws the _à la mode_ clinical conclusion to the effect
that the kidneys are probably involved in the arteriosclerosis;
but other cases do not show a high blood pressure but may in fact
show a low blood pressure. The vascular disease doubtless
responsible for the aphasia in the Bartlett case is probably not
at all an effect of blood pressure conditions, but is, on the
contrary, an effect of local syphilitic vascular lesions.

=PARETIC NEUROSYPHILIS (“general paresis”) is often marked by
APHASIA.=

=Case 22.= Meyer Levenson, a traveling salesman of 36 years, had for the last two or three years been undergoing a change of disposition, quite interfering with his work. He had begun to take unreasonable aversions to people, had become irritable and emotionally depressed, and often fell to weeping without cause.

About nine months before hospital observation, it seems that a trunk-cover had fallen on Levenson’s head, and there is some question as to whether he did not have a convulsion at that time. However, a month later he had a definite seizure, followed by speech disorder, a slight paralysis, and a staggering gait. Four weeks later, however, he had gotten over these post-convulsive difficulties and had gone back to work.

At his work, he became tired easily, his gait and speech did not seem entirely normal, and there was a considerable memory disorder. After five more months, another attack of a convulsive nature, with twitching of hands and face and tongue-biting occurred, and the attending unconsciousness remained for two days. Again improvement followed, though without ability to return to work. Four (?) months later there were several severe convulsions and Levenson would remain unconscious for a day or two at a time. Restlessness, irritability, and irrational talking followed.

=Physically=, the patient was fairly well developed and nourished; blood pressure 168 systolic, 68 diastolic; pupils reacted very sluggishly to light. There was a marked motor aphasia, which the patient recognized as a speech difficulty. On the whole, however, Levenson was very euphoric and was entirely sure that he was improving and would surely get well.

Shortly after entrance, Levenson had a severe convulsion, with unconsciousness. The movements were mainly on the right side of the body, and there was a post-convulsive weakness of the right side for several days, followed by a slow recovery of strength.

The course of the disease—convulsions followed by improvement—is very characteristic of a paretic onset. The =laboratory findings= were in all respects confirmatory. It was rather striking that a permanent _motor aphasia_ followed the convulsions in this case, since the seizures of paresis do not in the vast majority of cases leave permanent paralyses. The course of the disease continued to show convulsions, which would in each instance leave him at a lower terrace of capacity than had been before shown. The patient died four years after the onset of symptoms of a general asthenia. With the exception of the permanent motor aphasia, this case might be regarded as a fairly typical one of general paresis.

1. What is the general nature of speech disorder in paretic
neurosyphilis? Speech disorder is, along with the pupillary
changes, one of the most important clinical symptoms in paretic
neurosyphilis. There are aphasic and articulatory disturbances.
The aphasia that accompanies paretic seizures is of a transient
nature as a rule. A case with such long-standing motor aphasia as
shown by Levenson is not common. Paraphasia, with incorrect naming
of objects, may last longer. The so-called “sticking” phenomenon
is often observed.

Word-deafness is said to be rarer but is difficult to test on
account of the patient’s dementia. Agrammatism (incapacity to form
correct sentences) is sometimes observed. But the most
characteristic disorder is in the syllabic composition of words.
Syllables are left out (“medaltricity” for medical electricity),
or fused (“exity”), or doubled (“electricicity”). Besides the
central speech disorders of which the above are examples, there
are disorders in articulation, which at first occur as a
consequence of paretic seizures or in states of excitement, but
later become permanent. These are divided into paretic and ataxic
disturbances.

2. What is the structural basis of these forms of aphasia? It is
believed that they are due to microscopic changes, not to coarse
destructive lesions.

BROOKLINE, MASS.
]

BROOKLINE, MASS.

Mss. of Levenson, case 22. Paretic neurosyphilis. Tremor, misspelling.
Metathesis of letters (Bk, not Br) omission of letters (Book).
]

God save the Commonwealth of Massachusetts

Mss. of Safsky, case 48, brain tumor. Tremor not marked. Misspelling,
omission of letters. Wrong letters (h in hweth).
]

Mss. of Halleck, case 31, cervical tabes. No brain disorder.
Pen-holding and bearing on difficulties. Crowding of phrases result
of ataxia.
]

Mss. of Collins, case 61, paretic neurosyphilis. One misspelling
(-chussetts); not psychopathic? Characteristic tremor.
]

=REMISSIONS of identical appearance occur in PARETIC and in DIFFUSE
(non-paretic meningovascular) NEUROSYPHILIS.=

=Case 23.= Thomas Donovan, a merchant 44 years of age, acquired syphilis according to his own story at the age of 31, and he was at that time treated at a well-known watering-place with mercurial injections. Later he continued treatment under his family physician, and at 34 was pronounced cured. However, four years later—that is seven years after his initial infection and in his 38th year—he had his blood examined and it proved positive. He was accordingly treated by salvarsan and his W. R. became negative. The story did not end there, however, for at 43, mental symptoms appeared of the nature of depression and a diagnosis of paresis was made. He was released from the institution against advice at that time, and without treatment, made a partial recovery.

A sudden outburst of violence brought Mr. Donovan to the Psychopathic Hospital; he was very surly, combative, and difficult to manage, standing 6′ 2″, and weighing 210 pounds. He was oriented only fairly well and his surliness was streaked with humor. He facetiously said that the Psychopathic Hospital was the largest hospital in the country, and that it was, in fact, a horse hospital; that he had come because he liked the surroundings, not to make money; that he was the healthiest man in the world, never having been sick; that the Psychopathic Hospital was a club, for which you have to get somebody to propose your name. There was amnesia and no knowledge of current events. He regarded the food as poisoned, refused to eat, and was very irritable and untidy.

=Physically=, there were few abnormalities, but the pupils failed to react either to light or accommodation, and the knee-jerks and ankle-jerks were absent. There was a slight Rombergism. There was a marked speech defect to test phrases. Both serum and spinal fluid W. R.’s were positive; the fluid showed 41 cells per cmm., there were large amounts of globulin and albumin, and the gold sol reaction was of the “paretic” type.

=ATAXIA OR INCOÖRDINATION=

NEUROSYPHILIS
LESION OF PERIPHERAL SENSORY NERVES
DIVISION OF POSTERIOR ROOTS
TUMORS OR CHRONIC SCLEROSIS OF POSTERIOR COLUMNS
SUBACUTE COMBINED DEGENERATION
VESTIBULAR ATAXIA
FRIEDREICH’S ATAXIA
FAMILY PROGRESSIVE HYPERTROPHIC NEURITIS
THROMBOSIS POSTERIOR INFERIOR CEREBELLAR ARTERY
MARIE’S HEREDITARY CEREBELLAR ATAXIA
LESIONS OF CEREBELLUM, TUMORS, ETC.
WRITERS’ CRAMP
PREHEMIPLEGIA
MULTIPLE SCLEROSIS
PSEUDO-SCLEROSIS
HYSTERIA

CHART 18

=CONDITIONS IN WHICH VERTIGO IS FOUND=

NEUROSYPHILIS
HEAD TRAUMA
CEREBRAL ANEMIA AND HYPEREMIA
MENOPAUSE
ARTERIOSCLEROSIS
RENAL DISEASE
CEREBRAL HEMORRHAGE AND THROMBOSIS
INTRACRANIAL TUMORS
MULTIPLE SCLEROSIS
EPILEPSY (AURA)
TOXIC CONDITIONS:
alcohol, tobacco, constipation
PSYCHONEUROSIS
OCULAR DISTURBANCES
EAR DISEASE
MÉNIÈRE’S DISEASE
MIGRAINE

CHART 19

Salvarsanized serum was injected intraventricularly through a trephine opening in the right frontal region. Injections were made through the corpus callosum into the third ventricle. There was progressive symptomatic improvement after each of four injections. In fact, after the fourth injection the patient was allowed to leave the hospital despite the fact that there was only a slight improvement in the spinal fluid findings. The speech defect had entirely disappeared. (Speech defect, according to many authorities, including Kraepelin, is of very grave diagnostic significance.) His memory returned. Mr. Donovan is now able to handle figures rather extraordinarily well. He now has a good insight into his delusions and tells stories about them with great humor.

1. What is the definition of a remission in general paresis?
Remissions form a foil to seizures; just as seizures mark a sudden
advance in the severity of the disease or may even lead to death;
so remissions may cause a sudden cessation of both mental and
nervous phenomena in the disease. Whereas the seizures occur most
often, according to Kraepelin, in the demented types of paresis,
the remissions occur in all cases except in the terminal phase.
Kraepelin quotes Hoppe as observing pronounced remissions of long
duration in 17% of male and 15% of female paretics. Gaupp observed
marked improvement in less than 10%, and very marked improvement
indeed in only 1% of his cases. Kraepelin states that such
improvements are most frequent in agitated and especially in
expansive forms of paresis, and that they are rarer and less
complete in the depressive and demented forms. Sometimes the
improvement occurs over night, although the full extent of the
remission becomes complete only gradually, perhaps in the course
of months. The sensorium clears, the disorientation disappears,
the delusions retreat, and the former delusions are treated as
dreams and imaginations. There is often a good deal of persistent
uncertainty as to events during the height of the disease. The
nervous disorders are far more obstinate than the mental. Still,
both speech and writing may often greatly improve.

Cotton in New Jersey found, among 127 cases of paresis
diagnosticated by modern methods during seven years, that
remissions occurred in but five, or about 4%, lasting from a half
to three years.

2. Does a remission ever amount to a cure? The classical case quoted
in this connection is one observed by Tuczek. This case developed
a picture of paresis in 1876, at the age of 36; and a remission,
or cessation, of symptoms, occurred in 1878; but in 1883, at 43
years, the patient developed a tabes without any trace of mental
disorder, which tabes gradually advanced. By the middle of 1898,
when the patient was 58, certain symptoms of excitement and
confusion occurred, which led to death with dementia, 22 years
after the beginning of the disease. Nissl pronounced the cortex to
be undoubtedly the characteristic cortex of a paretic. This
observation seems to indicate that a clinical remission tantamount
to a clinical recovery may occur without the death of the
spirochetes engaged. This observation is to be held in mind in
connection with all therapeutic work with neurosyphilis.

Nonne states that during his clinical experience of 19 years he
had followed 10 cases of paresis with apparent recovery; but of
these ten cases, four had to be thrown out by Nonne because the
apparent recoveries turned out to be only long and almost complete
remissions, finally issuing in characteristic dementia. Of the
remaining six cases, perhaps two should hardly be counted as
paretic and Nonne rather preferred to term them cases of
syphilitic dementia in the sense of a non-paretic cerebral
syphilis. At the end, therefore, of his review of observations,
Nonne found himself with four cases of true recovery from paresis.

Spielmeyer holds that there is no theoretical reason why paresis
might not be cured, since all the different changes that have been
described in the disease can be halted, and many of them can be
repaired. In particular, he reminds us that the acute infiltrative
process, the neuroglia reaction, and the phagocytic action of the
large mononuclear cells are distinctly removable processes. (See
discussion below under Section V, for apparent cures and
remissions occasionally secured under treatment.)

=REMISSIONS of identical appearance occur in PARETIC (“general
paresis”) and in DIFFUSE (non-paretic) NEUROSYPHILIS.=

=Case 24.= Michael O’Donnell, a laborer of 48 years, came home, one day, at 5:30, complaining of severe headache. His wife told him he should lie down and, taking him by the arm, tried to help him to the bed. At this moment, O’Donnell lost control of both left arm and left leg, and fell, unable to move but with consciousness preserved. The wife noted that the left side of his face was drawn up and that he drooled. He was at once carried to a general hospital, remaining there for about three weeks, talking at random in a delirious manner and tied in bed. Two intraspinous injections of salvarsan were given, and O’Donnell showed considerable improvement and went home.

However, upon his return from the hospital, he became very wilful, would not remain in bed, and on one occasion actually took the mattress from the bed, carried it to another room, and then returned to his own room and slept upon the springs. He became irritable and emotional, insisted upon going to the hospital, did not go there but upon returning home insisted that he had been there. That night, O’Donnell left the house only partly dressed.

It appears that O’Donnell had been excessively alcoholic, but that before August 15, when he sustained the left-sided hemiplegia above mentioned, there had been no symptoms except that in February he had once been very dizzy. It appears that there had been another dizzy spell, three nights before the paralysis, accompanied by a fall and unconsciousness for about 15 minutes.

=TRANSIENT OR FLEETING PARALYSES=

NEUROSYPHILIS
MYASTHENIA GRAVIS
MYOTONIA CONGENITA (THOMSEN’S DISEASE)
PARAMYOTONIA CONGENITA
MYOTONIA ATROPHICA
INTERMITTENT CLAUDICATION
OCCUPATION NEUROSES
FAMILY PERIODIC PARALYSES
ETANY
EPILEPSY MINOR
HYSTERIA
MULTIPLE SCLEROSIS
APOPLEXY
CEREBRAL THROMBOSIS

CHART 20

O’Donnell was brought to the Psychopathic Hospital some six weeks after the paralysis, complaining merely of a slight headache and desirous of treatment. There were no mental symptoms of any sort. =Physically=, O’Donnell was in general not abnormal (there was a slight pre-systolic murmur and a blood pressure of 190 mm. systolic). The pupils were slightly irregular, the left larger than the right; both reacted sluggishly. Both ears were moderately deaf; the tendon reflexes of the left arm and leg were somewhat more lively than those on the right. The systematic =neurological= examination otherwise revealed no abnormalities. The urine was negative. The serum W. R. was positive but the spinal fluid reaction was negative. There were but 2 cells per cmm., and there was a very slight trace of albumin.

1. How shall we account for O’Donnell’s transient paralysis? We
might invoke brain tumor, alcoholic pseudoparesis, or some form of
neurosyphilis. The diagnosis of brain tumor seems quite untenable
in view of the absence of premonitory symptoms and in the absence
of intracranial pressure. As for alcoholic pseudoparesis it is
true that the patient was excessively alcoholic.

However, against these two diagnoses and in favor of the diagnosis
of NEUROSYPHILIS, are the positive serum W. R. and the pupillary
reactions (although these are short of the true Argyll-Robertson
phenomenon). Dizziness with retention of consciousness and
associated with the paralyses mentioned suggests rather a
subcortical than a cortical lesion. We are inclined to regard this
lesion as probably THROMBOTIC, and to place it possibly in the
region of the internal capsule. We are inclined to regard the
phenomenon as purely vascular and as not in this case associated
with an encephalitis. We are, however, not entirely satisfied with
the diagnosis.

2. What shall be said as to treatment? A full-blown left-sided
hemiplegia may be produced even when the thrombotic lesion is
itself exceedingly small. It is common to explain this on the
basis that there is an area of collateral edema about the small
necrotic, thrombotic, or hemorrhagic area responsible for the
lesion. In short, numerous neurones are functionally rather than
structurally affected, or at all events capable of early
restitution of function.

3. What is the prognosis in such cases? It appears that now and
again patients run for several years without further trouble, both
with and without treatment. We are inclined, however, to advocate
treatment rather than absence of treatment for a variety of
reasons. In the first place, vascular lesions may at any time
become associated with meningitic lesions, and treatment by
salvarsan may perhaps be counted on to head off this process;
secondly, the treatment with iodids may possibly aid in the
resolution of a local thrombotic process.

4. What are the prodromal symptoms of cerebrospinal syphilis?
According to Nonne, headache, dizziness, sleeplessness, mental
symptoms of the irritability group, loss of capacity as to mental
work, whether severe or not, and loss of capacity for difficult
thinking; also impairment of memory. Nonne does not regard these
phenomena as characteristic of syphilitic vascular disease, and
calls attention to the fact that in every organic disease the same
subjective symptoms occur. The triad—headache, dizziness, and
impairment of memory—is for example now counted as a prodromal
symptom complex for arteriosclerotic apoplexy (Cramer). Of course,
apoplectic attacks occur without such preliminary symptoms:
particularly, according to Nonne, the nocturnal attacks.

5. Can the fleeting paralysis be of service in differentiating the
diffuse from the paretic form of neurosyphilis? Probably not. In
both forms transient paralyses occur as well as the permanent
ones. In general, however, the transient paralyses are more
frequent in paretic neurosyphilis, whereas the permanent ones
occur more often in diffuse neurosyphilis.

=There are cases of NEUROSYPHILIS in which the laboratory signs are
positive but in which there are no clinical signs or symptoms
(PARESIS SINE PARESI?).=

=Case 25.= Richard Lawlor[6] was admitted to the Psychopathic Hospital, October 29, 1914, being sent there from a general hospital where he had gone on account of a self-inflicted wound of the wrist, apparently made in a period of depression with suicidal intent. Routine notes follow.

=Family History.= Paternal grandparents both died of heart disease. Maternal grandfather died at seventy-two of dropsy. Moderately alcoholic. Maternal grandmother died of shock at fifty-six. Father died at age of forty, after an illness of eight years, from heart disease. Father all his life was subject to fainting spells and headaches. The only paternal cousin died at thirteen months of brain fever. Mother, aged forty-seven, is, to say the least, eccentric. Says “she has several times been given up from tuberculosis.” Two maternal uncles died of tuberculosis, one from rupture, one from heart disease. One uncle who “doesn’t know anything after he has a teaspoonful of liquor.” Several other uncles and aunts whose history is not obtained. Patient is mother’s only child. Mother was twice married. There were several miscarriages by both husbands; patient child by first marriage.

=Past History.= Patient born thirty-two years ago, full term, normal delivery and development. Measles, mumps, and chickenpox in childhood. Subject to headaches since seven or eight years old. Kicked in the face by horse at seventeen or eighteen, not considered serious. Hit by a baseball three or four years ago, leaving him hard of hearing on left side. Married ten years ago; no children because he says his wife needed an operation. He denies venereal disease by name and symptoms. For past ten years has had attacks of depression lasting but a short time, but quite severe. Never caused him to quit work as a barber and he felt better when working. His married life he says was fairly happy except for his wife’s extravagances, and on this account he left her a little over a year ago, and she has applied for a divorce, which he is willing that she should have, but does not wish to give her alimony. He admits moderate alcoholism.

=Present Trouble.= Patient states that since he left his wife a year ago he has felt sorry a number of times. He has wished he had her back. He has felt lonely. He has had six or eight periods of depression in that time similar to those he has had for many years, lasting two or three days, and sometimes a week. These were always precipitated by some cause for worry. In these attacks he feels nervous, sleeps poorly, has little or no appetite, sweats during his work and everything looks black. Several times in these attacks he has had suicidal ideas. Ten months ago he considered taking corrosive sublimate. For a little over a week before entrance to hospital he had been out of work and had been “sporting.” The day before entrance he had a telephone message from his lawyer which upset him somewhat and he walked the floor all night. He had just been shaving when the idea of suicide came to him. He sat down a minute when suddenly the thought “to hell with the world” came to him; he took the razor and slashed his wrist. He does not remember drawing the razor across his wrist. As soon as he saw the blood he felt sorry, called his mother, and was taken to an emergency hospital and then sent to the Psychopathic Hospital.

=Physical Examination.= Patient is a well developed and nourished man thirty-two years of age. Head is normal as to size and shape; there are no scars or marks of injury. Hair and skin not remarkable in any way. Ears negative to external examination. Teeth well kept; two missing, several gold fillings. Tongue very slightly coated. Throat negative. Tonsils easily visible without evidence of inflammation or exudation. Neck, no thyroid enlargement, no abnormal pulsations, no adenopathy. Chest, symmetrical, expansion good, resonant throughout. Breath sounds transmitted normally. No râles or rubs heard. Heart, no enlargement or cardiac dulness. Sounds of good quality, no murmurs heard. Rate regular. Pulses equal, regular and synchronous, and of good volume and tension. Systolic blood pressure 130, diastolic 65. Abdomen, flat, soft and tympanitic throughout; no masses; no tenderness. Liver edge not felt, below costal margin. Spleen not palpable. Extremities negative, except for incised wound on left wrist.

=Neuromuscular Examination.= Pupils are large, round, regular, equal and react readily to light and accommodation. No nystagmus, strabismus or ptosis. No weaknesses or paresis of facial muscles. The tongue projects medially and shows no tremor. The triceps and biceps reflexes are readily elicited, and are quite active, as are the knee-jerks and ankle-jerks. On one occasion it was thought that the tendon reflexes were slightly more active on the left than on the right. This was never confirmed; always afterwards found equal. There was no tremor of extended hands. Abdominal reflexes not elicited. Cremasteric present on both sides. The plantar response is flexor. There is no Babinski, Gordon or Oppenheim. No Romberg. Coördination tests well performed. No speech defect. No sensory disturbances. Urine examination negative.

Wassermann reaction in the serum: Positive, with cholesterinized antigen; negative, with syphilitic fetal liver antigen.

Wassermann reaction in fluid positive on two occasions. Examination of spinal fluid, November 4: globulin +++, albumin ++, 100 cells per cubic millimeter; large lymphocytes, 8 per cent; small lymphocytes, 90 per cent; plasma cells, 0.7 per cent; endothelial cells, 1.3 per cent. November 11, globulin +++, albumin +++, cells 18 per cubic millimeter. November 26, globulin ++, albumin ++, cells 92 per cubic millimeter; large lymphocytes, 13.1 per cent; small lymphocytes, 82.1 per cent; plasma, 1.2 per cent; endothelial, 3.6 per cent.

Gold sol, November 4, 5555432100.

Gold sol, November 26, 3332100000.

=Mental Examination.= On entrance to hospital patient seemed slightly depressed and a bit irritable. This condition lasted two days, after which he was agreeable and apparently entirely over his depression. Even during his mild depression, however, he talked freely. There was no evidence of retardation. He told his story readily. Orientation was intact. Memory excellent. Educational knowledge well retained. There was no evidence of any hallucinations or delusions.

1. Was Richard Lawlor insane?

There was, then, on the mental and physical examination nothing to
make a definite suggestion of a psychosis, and the most one could
think of was a psychoneurosis or a cyclothymia of at least ten
years’ duration. The findings in the cerebrospinal fluid and the
Wassermann reactions, however, give us material for thought.
Certainly one cannot call the man insane; all who saw him agreed
on this point.

2. If Richard Lawlor should some day develop mental symptoms, what
would be the genesis of the new psychosis? Though writers such
as Fildes and McIntosh, and Swift, have suggested an
anaphylactic or hyperallergic explanation for the development of
symptoms after a normal interval; such a hypothesis could hardly
obtain in the present case. The hyperallergic hypothesis for the
development of tertiary neurosyphilis would run to the effect
that in the secondary stages there had been a definite disease
of the nervous system, which, however, absolutely cleared up,
leaving no inflammatory vascular or parenchymatous relics of its
existence. Nothing would on this hypothesis remain except a
hypersensitisation of the tissues. In some later period of the
now clinically normal person, one or more spirochetes from a
lesion outside the nervous system are carried into the nerve
tissues and there set up an anaphylactic or hyperallergic
reaction. It is obviously difficult to prove the correctness or
incorrectness of the hyperallergic theory without numerous
examinations of the spinal fluid, in clinically normal persons
after the secondaries have passed. The present case, so far from
demonstrating a normal fluid, demonstrates a highly pathological
fluid, even though there are absolutely no clinical symptoms
which could be regarded as of nervous origin. The burden of
proof at the present time would seem to lie with those who claim
hyperallergy in neurosyphilis. We prefer on present evidence to
think that at the conclusion of the secondaries a disease
process often remains in the nerve tissues despite clinical
quiescence.

3. What is the prognosis in the case of Richard Lawlor? The
prognosis _re_ neurosyphilis is doubtful. We have, however, boldly
termed the condition _PARESIS SINE PARESI_, meaning thereby to
suggest that the patient is in considerable danger of the
efflorescence of a true diffuse or paretic neurosyphilis. We have
no means of telling, however, whether the positive symptoms would
be those of a paretic or a non-paretic neurosyphilis. As data
accumulate regarding these cases of _paresis sine paresi_, we may
be able finally to come upon some case in which trauma shall bring
out the clinical symptoms of neurosyphilis. For discussion of this
matter, see the case of Bessie Vogel (52) in Part III of this
book.

4. Should Lawlor have been brought to a psychopathic hospital? It is
a safe working rule to have any person who attempts suicide
observed. A large percentage of suicides occur in psychotic
individuals and a suicidal attempt is not infrequently the first
recognized abnormality. Immediate observation is a necessary
safeguard against another more successful attempt.

=Demonstrates SYMPTOMS and LESIONS of PARETIC NEUROSYPHILIS
(“general paresis”). Autopsy.=

=Case 26.= John Morrill, 49, an operative in a mill town in Essex County, Mass., was described as a “Saturday night and Sunday drinker,” with a history of very serious long sprees at the age of 43. It seems that he had had what was called “sciatica” at 35, and was treated in hospital for seven weeks at that time. The nature of this sciatica is in doubt, but there was a history of syphilitic infection at 36 years (scar of glans).

Morrill had been married twice, and two of the children were dead; one daughter was described as “very nervous,” but there were four children under ten years of age, all regarded as perfectly healthy.

Morrill had been a mill operative of average capacity, was industrious, and had supported his family despite alcoholism. The syphilis had been treated with reasonable thoroughness.

Aside from alcoholism, there had been no symptoms up to two months before admission to Danvers Hospital. Then there had been insomnia, fatigue, agitation, eruption on foot, loss of ten pounds in weight, hypochondriacal fears, apprehensiveness for the future of the children, incoherent talk; and just before admission, his talk was described as foolish. He had taken to running away and hiding in bushes by a pond and in the cellars of other people’s houses.

The patient was of medium height and weight, with thin grayish hair and grayish irides; musculature was slender. The face was blank in expression, the teeth poorly preserved with atrophy of gums, the tongue coated, and the breath foul. There was a gummy secretion of the eyelids, an area of brownish branny eruption over both clavicles, a number of depressed scars over the limbs and back, and another area of scaly eruption on the right heel and the sole of the foot. The heart area was increased, and the sounds were faint at the base, with the first sound accentuated at the apex. The urine showed a trace of albumin.

=Neurologically=, the Romberg position was maintained with a general tremor and fluttering of the eyelids. In complicated movements, the patient was slightly ataxic. The pupils were irregular, the left being much larger than the right. There were no light reactions to be obtained in window light. The reaction to accommodation was present, though slight. Vision was poor, ¼-inch capitals could not be read by left eye at reading distance. The knee-jerks were diminished equally; the Achilles jerks were absent; the other reflexes were normal. Upon the sensory side, the patient gave a history of pains in the legs at irregular intervals for several years. These pains he described as of a darting character. There was little or no sensory disorder, although the outer surface of the right leg required a deeper pressure to elicit sensation. There were no disorders of muscle sense.

If Morrill was to be trusted, he had been born in Ireland, and had come to the United States at the age of 17. He married at 18; there had been seven pregnancies by the first wife, with one stillborn child; one child had died at five weeks. The four children by the second wife were healthy. The first signs of neuritis had occurred at 45 and had received the diagnosis neuritis, although no connection between the neuritis and the syphilis had been noted.

The patient entered the hospital July 26, 1904, and was discharged, improved, January 5, 1905. He returned a little more than a year later, January 15, 1906, and died March 21, 1906. The total duration of the disease from the onset of mental symptoms may therefore be stated as somewhat under two years. When the patient appeared at the hospital the second time, he showed a positive Romberg sign, an unsteady gait, an ataxia that still was moderate, and somewhat more marked tremors, involving fingers, tongue, and face. He was now unable to read ½-inch type with the left eye. The knee-jerks, formerly diminished, were both exaggerated, the left slightly more so. The Achilles reaction, not obtained formerly, now appeared on the right side. The pupils reacted as before. The sensory loss had become more marked, since sharp and dull points could hardly be distinguished. Deep pinpricks were not felt in the leg, and heat could not be told from cold.

The speech in 1904 had been somewhat defective (“truly rural” rendered as “tooly lualal,” “sifted soft thistles” as “thoft thsistles”), and there had been little further development of the speech defect. The handwriting had lost appreciably in legibility and had become much more tremulous. During the first period of hospital observation Morrill had what might possibly have been visual hallucinations, but it was impossible to tell whether his story of seeing his wife and children trying to get in through the window was hallucinatory or a matter of fabrication. Memory was decidedly imperfect and few details of recent events could be produced. The association of ideas was almost a so-called “flight” of apprehensive, fearful ideas, loosely connected, incoherently expressed, and dealing chiefly with his work and his children. Judgment was imperfect; the height of the room was estimated as 24 feet, but the height and weight of persons were estimated with fair accuracy, and also the length of small objects, whose lengths were doubtless remembered rather than estimated. The estimate of time elapsing during a medical examination was accurate, but the estimate of longer durations involving over-night memories was hopelessly imperfect. Emotionally, there was a dulling of sensibility, an appearance of suspicion and apprehensiveness; the patient fancied himself to be in a hopeless condition as a result of syphilis, but at the same time accompanied his statement of his hopelessness with laughter. A sample of his hypochondriacal ideas: “I am all gone; I am good for nothing; I am all gone now; I can’t drink now; can’t write or talk at all; worse than when you saw me first; nothing in my inside; all wrong through me again; I aint got no swallow now; I can’t die even; my heart aint much good; I can’t hear it beat; I don’t think it flutters; no life in these hands; they are all cold and dead” (pointing to his arms and moving them about). During such a portrayal the patient laughed in a silly way.

During the second hospital stay, Morrill was at first restless, sleepless, profane, imperfectly oriented for time, possibly for place, and also for the attendants. A few weeks later he became stuporous and confused, and his feebleness and physical exhaustion were finally ended by death, March 21, 1906. Death was preceded by a semi-comatose condition; a left otitis media had developed.

At the =autopsy=, it appeared that death was due to an early bronchopneumonia associated with acute splenitis and doubtless related to the otitis media of the left side. The body at large showed, aside from these acute lesions, a few chronic lesions, including slight scars of the left apex, and chronic adhesive pleuritis, chronic diffuse nephritis, and aortic and coronary syphilis. The aorta showed slight linear and nodular markings, with a single small dark ulcer in the upper thoracic region, but the aorta did not show the characteristic scarring which syphilitic aortas often show. The femoral marrow was of a dark red chocolate color. The thyroid appeared to be smaller than normal. A slight sacral decubitus had developed.

The description of the head (E.E.S.) is given in full on account of the encephalitic lesions shown. These encephalitic lesions may be summed up as follows:

Local cerebral =atrophy= and =sclerosis= of the frontal, orbital,
and central regions, especially of the left operculum and left
supramarginal gyrus.

Extension of sclerosis to hippocampal gyri with effacement of
substantia reticularis alba.

Slight chronic internal =hydrocephalus=.

Granular =ependymitis= (especially of floor of 4th ventricle).

Compensatory edema of frontal and central pia mater.

Cerebellar sclerosis (culmen monticuli, lobus culminis, lobus
cacuminis).

Spinal sclerosis (grossly evident in the posterior columns of the
upper thoracic region and of the lumbar enlargement).

The details are as follows:

Head:—Bald on top. Hair =gray=. Scalp normal. Calvarium thin, deeply
excavated by arachnoidal villi to right of vertex. Diploë absent.
Dura closely adherent in bregmatic region. Dura of usual thickness.
Sinuses contain cruor clot. Arachnoidal villi slight. Pia mater hazy
and over sulcal veins porcelain white over all of vertex except
occipital poles and over flanks (notably left). Thickened also
around circle of Willis, over culmen monticuli and in posterior
cerebellar notch. Edema of pia corresponding to atrophy of frontal
and central regions. Cerebral atrophy most marked in orbital
surfaces of both frontal lobes, in left area of Broca, and in left
supramarginal region. The ascending branch and the ascending ramus
of the posterior limb of the left Sylvian fossæ both readily admit
the thumb by reason of atrophy of adjacent substance. Induration
corresponds closely with atrophy, but is not more marked about the
left Sylvian fossa. There is sclerosis of both hippocampal gyri,
with loss of the substantia reticularis alba. The culmen monticuli
and lobus culminis are firmer than the clival regions, and the lobus
cacuminis is again slightly firmer than the clival region.
Cerebellum a little softer than usual. Pia strips with usual
readiness from all regions. The subpial region of the frontal lobes
is a trifle grayer than that of the rest of cerebrum. Ventricles
slightly dilated. Surfaces evenly sanded. Floor of fourth ventricle
shows numerous coarse, closely set granules. Brain wt. 1200 grms.
Cord shows a slight increase of consistence over one or two upper
thoracic segments and in lumbar enlargement corresponding with a
slight graying out of posterior columns. In places there is a
suggestion of graying out also in lateral columns. A few calcified
plaques in posterior lumbar pia.

Analysis of these details shows a number of lesions that characterize paretic neurosyphilis (among others, granular ependymitis, frontal atrophy, chronic leptomeningitis), but the lesions are more than merely frontal, extending as they do back as far as the postcentral regions on both sides, and even as far as the left supramarginal gyrus. The cerebellar involvement although frequent, can hardly be said to be characteristic in paretic neurosyphilis. The spinal involvement is characteristic of a case which is probably to be regarded as one of taboparesis; that is, of paretic neurosyphilis following a number of years after the establishment of tabetic neurosyphilis. The aorta is almost constantly affected by sclerosis in paretic neurosyphilis. The absence of diploë in the skull is not infrequent and the adherent dura mater is often found.

Microscopically, the tissues showed the characteristic lesions of PARETIC NEUROSYPHILIS; nerve cell destruction, fibrillar and cellular gliosis, lymphocytic and plasma cell deposits about the small vessels.

1. What are the clinical evidences of syphilis outside the nervous
system? The brownish branny eruptions of the skin, the depressed
scars and the scaly eruption on right heel and sole are very
suggestive of syphilis. Such clinical evidences of syphilis are
very important in systematic examination. Although the laboratory
tests are of the utmost assistance in the diagnosis of syphilis,
the clinical signs should not be neglected, and no physician
should rest satisfied with laboratory signs alone. X-ray diagnosis
of bone conditions sometimes succeeds when all other methods have
failed.

=GUMMA of cerebral cortex verified by operation; death.=

=Case 27.= The presenting picture in the case of David Tannenbaum was that of deep dementia, in which condition the patient was brought to the hospital. There was a meagre history to the effect that about four months before admission, he had lost his job in a hotel through lack of further work. We heard that at this time he had begun to suffer with excruciating pains in the head; at first, worse at night, later, worse by day. It appeared that this pain, though it came and went, was chiefly localized on the left side of the head. For a fortnight, Tannenbaum had been dragging his legs, until finally he had become unable to walk at all.

_Pari passu_ with these developments, Tannenbaum had become mentally confused and irritable, and his memory had become untrustworthy. For several days before admission, an appearance of marked dementia was presented, with slow incoherent, or at all events, irrelevant words, and a complete disorientation for person. However, his vision had become so poor that it would have been hard for him to have recognized any one.

It appeared that the family history was entirely negative; that the patient was without education but had been physically very strong, and had been fairly successful at first in the junk business, and later in the clothing business; but latterly he had been less fortunate in the clothing business, and finally had to resort to work as a laborer around a hotel.

His wife had had eleven pregnancies with but one miscarriage. Nevertheless, out of the eleven pregnancies, there were now but four living children.

=Physically=, Tannenbaum was a rather small man; he was flabby and looked as if he had recently lost weight. The skin showed areas of pigmentation on the face and sides of the neck, and some dark copper-colored circular areas, marble-size, in the neck (syphilitic?). There was a slight radial arteriosclerosis. The heart was slightly enlarged with distant and indistinct sounds. There was a small pedunculated growth on the right side of the abdomen.

The pupils failed to react to flash-light but they reacted to sunlight. They both were slightly irregular but were equal in size, and reacted in accommodation. There was apparently almost complete blindness and extreme deafness. Arm-jerks and knee-jerks were absent; there was an occasional slight response of the left ankle-jerk, but the right ankle-jerk was absent; the left abdominal reflex was very feeble; the right absent; the cremasteric reflexes were absent, but there were no other abnormalities in the systematic examination. Hand grips weak; gait awkward, with right leg held somewhat flaccidly.

It was significant that percussion over the left frontal and parietal regions was able to elicit great pain. Either through the patient’s deafness or through sensory aphasia, spoken language was not understood. The serum W. R. was positive, the fluid W. R. negative.

=Diagnosis=: The clinical symptoms seem clearly to indicate syphilis. The local skull tenderness and impairment of vision might well suggest intracranial pressure. Uniting these suggestions, we might automatically arrive at a diagnosis of cerebral gumma. We have learned to be rather cautious of making a diagnosis of gumma of the brain through its mere rarity.

Decompression was suggested and executed. A deep growth resembling a GUMMA, in the view of the surgeon, was discovered. No attempt could be made to remove it. The patient died without recovering consciousness.

1. What is the significance of the negative fluid W. R. in this case
of cerebral gumma? The W. R. producing substances not infrequently
fail to appear in the spinal fluid from a gumma of the brain. The
serum W. R. was positive in this case, but even the serum W. R.
may be negative in cases of gumma, both of the brain and of the
body at large. It must be remembered that the serum W. R. may be
negative in paretic neurosyphilis (general paresis); the serum W.
R. is even more apt to be negative in cases of gumma.

Gummatous meningitis. Compression of hemisphere. Tissue destruction of
underlying cortex.
]

2. Is operative procedure to be advised in cerebral gumma? There are
cases in which the acute and threatening symptoms of heightened
intracranial pressure require operative treatment simply because
the therapeutist cannot wait for the effect of antisyphilitic
treatment. Moreover, antisyphilitic treatment of cerebral gumma is
not always as successful as that of most syphilitic lesions.

3. Could the intracranial pressure be caused by other syphilitic
lesions than gumma? A heavy meningitis may cause symptoms such as
produced by an intracranial tumor. In such a case one will usually
find evidences of inflammation in the spinal fluid. Cysts caused
by syphilitic lesions may also produce identical symptoms.

4. What is the significance of cranial tenderness? Where
sensitiveness to cranial percussion is not due to a scalp lesion
it is very suggestive of a tumor underlying this point. A
gummatous lesion of the cranium itself, may occur without causing
pain or increased sensitiveness.

=CRANIAL NEUROSYPHILIS (focal syphilitic extraocular palsy) without
mental symptoms.=

=Case 28.= A chef, Paolo Marini, 28 years of age, reported that on awaking one morning, everything appeared double to him and that his right eyelid had begun to drop. In the following month Marini had begun to feel weak and to have difficulty in swallowing, as well as at times difficulty in breathing. The diplopia was found to develop when Marini looked to the right. Mentally, the patient was in all respects normal, and no other physical signs were found except the diplopia and ptosis above mentioned. The W. serum test was positive, but the tests of the spinal fluid were negative.

=Diagnosis=: “CEREBRAL SYPHILIS.”

1. What is the anatomical cause of this condition? It is thought to
be due in a number of cases to a small diffuse gummatous lesion at
the _basis cerebri_. In the case of Marini this lesion appears to
have been a little more extensive and to have interfered with the
tenth and twelfth nerves also.

2. Why is the spinal fluid negative in such a case as that of
Marini? Head and Fearnsides believe that intracerebral lues is
characterized by a negative spinal fluid, under which circumstance
one has always to consider the possibility of brain tumor or
migraine in addition to the suspicion of syphilis.

3. What other causes besides syphilis should one consider for the
sudden diplopia? Brain tumor, multiple sclerosis, cerebral
arteriosclerosis, tuberculous meningitis, trauma and migrainous
ophthalmoplegia, are not infrequently at the bottom of this
condition. Cases also occur in which the etiology remains obscure,
even at autopsy.

Under antisyphilitic treatment, Marini slowly improved.

=The SIX TESTS in TABETIC NEUROSYPHILIS (“tabes dorsalis”) may run
milder than in paretic neurosyphilis (“general paresis”) and
characteristically run somewhat like those of diffuse
(meningovascular) neurosyphilis; in particular, the fluid Wassermann
Reaction and the gold sol reaction are apt to run milder. The
clinical course of tabes dorsalis is protracted and the prognosis as
to life is good.=

=Case 29.= Mario Sanzi, 55 years of age, had been having what he called rheumatism since his 43d year. This rheumatism affected only the hips and legs, had at times been very severe, and for two years past had been almost constant. Before that time, pains had come at intervals, lasted a variable period, and suddenly disappeared. They were of knife-thrust character, and could probably be called “lancinating.” In a given attack, these pains would come at intervals of seconds or more. There was also a certain unsteadiness in locomotion and inability to control the vesical sphincter.

=Physically=, the patient was entirely normal so far as could be made out except =neurologically=. Argyll-Robertson pupils, absence of knee-jerks, and ankle-jerks, Romberg sign, and characteristic gait, left no cause for doubting the diagnosis of TABES DORSALIS. The blood and spinal fluid both proved positive to the W. R., though the W. R. in the fluid gave a negative reaction with 0.1 cm. and became positive with 0.3 cm. or more. The globulin was somewhat increased though less markedly so than in paresis. The gold sol reaction was “syphilitic” but weak. It is to be noted that the disease had run a 12–years’ course before a doctor had been consulted. The primary infection occurred at 32 years, namely, 11 years before the symptoms began. At the time of his primary infection, Sanzi had received several years of treatment, chiefly in the form of mercury by mouth.

1. What is the value of mercurial treatment of syphilis in the
prevention of tabetic or other forms of neurosyphilis? “Fournier
strove for many years to convince the medical profession that a
syphilitic patient should be treated for at least two years after
his infection, whether the syphilis seemed latent or patent. The
method of treating only the symptoms he characterized as the
opportunist method; treatment in the absence of definite symptoms
the preventive method, as preventing the later manifestations.
That prolonged treatment does prevent is shown by Fournier’s
figures analyzing 2396 cases presenting tertiary signs. These he
divides into three groups: Group I, comprising 1878 cases, or 78
per cent of the whole number, having no treatment or inadequate
treatment—that is mercury for less than one year; Group 2,
comprising 455 cases, or 19 per cent, having moderate
treatment—that is, mercury for one to three years; and Group 3,
comprising the remaining 19 cases which represent only 3 per cent
of the whole number, having treatment for more than three
years.”[7]

In the light of what we now know concerning latent neurosyphilis,
it would seem well for patients to be followed from time to time
with the W. R. on blood and spinal fluid after the supposed
completion of the treatment of primary and secondary syphilis. The
examination of the spinal fluid is not superfluous, as our
experience with the so-called _paresis sine paresi_ abundantly
shows. At the present day it is not good practice to assure a
patient that he is cured after two years of ordinary mercurial
treatment without resort to frequent spinal fluid tests, even
though the serum W. R. be negative.

=TABETIC NEUROSYPHILIS (“tabes dorsalis”) is often quite ATYPICAL
clinically and may even show no single symptom warranting the old
clinical name “locomotor ataxia.”=

=Case 30.= Stephen Green is a case of TABES DORSALIS with active knee-jerks and without locomotor or muscle-sense disorder. When observed at the age of 45, it appeared that there were but two complaints: lack of control of the vesical sphincter and shooting pains in the legs. It appeared that the urinary disorder dated back ten years, when there had been difficulty in passing the urine. Sounds had been passed at the time; occasionally there had been incontinence during after years, ascribed by Mr. Green to the passing of the sound. However, the physician at that time stated that the incontinence was a symptom of tabes dorsalis. The incontinence had recently become worse, especially marked at night, though also occurring in the day; much worse during excitement, and very much worse after taking alcoholic drinks. Besides incontinence, there is also difficulty at times in passing the urine, as well as dysuria.

As for the pains in the legs, they had been first noticed some three or four years ago and considered to be mild rheumatic effects. Now, however, they have grown progressively worse and have been the effective cause of giving up business. The pains are sharp, darting, pinching, and burning, and last, say, about a second with an interval of about the same length. The attack will continue sometimes for many hours.

There is a strabismus of the left eye, ascribed by the patient to an accident with an umbrella (there had been operation without relief). The pupils showed the Argyll-Robertson effect and were markedly irregular. Despite the divergent strabismus with diplopia, the eye movements were well performed although not in parallel axes. Ankle-jerks could not be obtained even on reinforcement, but the knee-jerks were lively, and the other deep and skin reflexes proved normal. The blood and spinal fluid tests were characteristic of tabes dorsalis.

It appears that the syphilis was acquired by this patient 15 years before; that is, 5 years before neurological symptoms began. Three courses of treatment had been taken at a well-known watering-place, and mercury pills had been taken for two years by mouth. The patient is married; has no children; there have been no pregnancies.

1. What causes may be assigned for the absence of children in the
family of a tabetic? There may be lesions of the genital apparatus
(orchitis, or more specialized toxic lesions). But impotence such
as characterized the present case must also be taken into account.

2. What is the therapy for tabetic pains? Pyramidon is nowadays much
in favor; morphine may be used; some authors recommend that the
patients be instructed to chloroform or etherize themselves
slightly for relief of the pain. Surgery of the nerve roots may be
resorted to in extreme cases. Intraspinous therapy, suggested by
various authors, seems to exert beneficial effect in many cases.

3. Is the lack of control of the vesical sphincter an unusual
initial symptom? On the contrary, the more careful the clinical
observation, according to some observers, the more likely is the
examiner to find that vesical symptoms were the earliest or among
the earliest complaints of the patient. Baldwin Lucke found
sphincter disturbances to be initial in 8¼% of his long Blockley
series. He found sphincter disturbance to occur in some stage of
the disease in 67.6%, being exceeded in frequency only by
staggering gait (87.2%) and lancinating pain (71.6%). According to
Lucke, the most frequent _initial_ symptom is lancinating pain in
the lower extremity, which, it will be noticed, occurred also in
our case of Stephen Green as an initial symptom along with vesical
disturbance. Lucke’s figures show that paresthesia of the lower
extremities (17.6%) and weakness of the extremities (16.4%) are
the next initial symptoms in frequency.

4. Could the early treatment in the case of Stephen Green be
considered as adequate? No better answer can be given to this
question than by quoting from Dr. Joseph Collins,[8] who probably
has done more than any other one man in this country in insisting
on the need of proper treatment of syphilis. As to the adequate
treatment of syphilis he says:

“It consists in the proper use of salvarsan and mercury begun at
the earliest possible moment after infection and kept up till all
biochemical evidence of the disease has ceased, while the
metabolism of the individual is maintained as nearly normal as
possible. But the physician does not do his whole duty when he has
accomplished this. He must solicitously watch the individual to
see that no evidence reappears for months and even years after the
apparent cure. As an index of such reappearance the Wassermann
test of the blood serum and of the cerebrospinal fluid is the
safest guide.