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.
“Until there is a definite unanimity of belief among physicians as
to when the treatment of syphilis shall be begun, and some concert
of action as to what constitutes the adequate treatment of
syphilis, we cannot hope to make any considerable progress in the
prevention of syphilis of the nervous system, save by educating
the individual toward infection.”
=TABETIC NEUROSYPHILIS may produce symptoms chiefly if not entirely
in the region supplied by the CERVICAL plexus (“cervical tabes”).=
=Case 31.= Paul Halleck, 35, was a salesman who had begun to find it
hard to carry his sample case, since he was unable to tell whether or
not he had it in his hand. There was not only an anesthesia of the
hands, but they felt numb and there was often a tingling sensation. Of
late it had become hard for Halleck to dress himself or to write, and
these symptoms had been slowly growing worse. There was no other
complaint. There was, however, a history of a chancre about 7½ years
before, which had been followed by a rash and a sore throat. There had
been treatment with mercury and potassium iodid alternating for a period
of two years.
=Physically=, there was no evidence of disease except =neurologically=.
The pupils were unequal (the right larger than the left) and reacted
slowly to accommodation and not at all to light. A marked ataxia of the
hands was shown in coat-buttoning. The finger-to-nose test showed a
marked dysmetria. Arm-jerks as well as knee- and ankle-jerks were
absent. There was a slight swaying in the Romberg position but no true
Romberg sign. There was no difficulty in locomotion. Both blood and
spinal fluid proved positive to the W. R.; globulin and albumin were
increased. The gold sol reaction was syphilitic, and there were 85 cells
per cmm.
This case is probably not a pure example of CERVICAL TABES, since the
knee-jerks are also absent, and we may suppose a degree of lumbar spinal
cord changes in addition to the cervical changes. It well illustrates,
however, that the tabetic involvement of the cord may be quite
generalized and that it may strike high as well as low.
=ERB’S SYPHILITIC SPASTIC PARAPLEGIA.=
=Case 32.= Margaret Neal, a maid-of-all-work, 36 years of age, was
committed to a home for inebriates on account of her excessive
alcoholism, but she was shortly transferred to the Psychopathic Hospital
on account of difficulty with locomotion. We found a very marked
spasticity in walking, with a characteristic scissors gait. The pupils
were somewhat irregular, and although both reacted to light, the left
reacted far more slowly than the right and the reaction failed to hold
well. The arm reflexes were very active, and the knee-jerks and the
ankle-jerks were particularly exaggerated. There was a double Babinski
reaction, as well as Oppenheim and Gordon reflexes and a bilateral ankle
clonus. There seemed to be tenderness over the nerve trunks in the back
of the leg, below the knee. There was no evidence of incoördination, no
Rombergism, no disturbance of sensation, no disorder of the special
senses, and not even a tremor of the tongue or hands.
=Mentally=, the patient was entirely negative.
=Diagnosis=: Symptomatically, it is entirely clear that the patient was
suffering from SPASTIC PARAPLEGIA. One would have to consider besides
spinal syphilis, also amyotrophic lateral sclerosis, syringomyelia, and
spinal cord tumor. However, there appeared to be no definite wasting of
muscles, and the fact that the sensations were intact seems to rule out
also syringomyelia. There was none of the characteristic pain associated
with a cord tumor. There was, in fact, a strong clinical premonition
that the case was one of spinal syphilis, simply because syphilis is the
most common cause of spastic paraplegia in the adult. The pupillary
anomalies were also highly suggestive.
The serum W. R. proved to be weakly positive, as was also the gold sol
reaction in the zones characteristic of syphilis. The spinal fluid
examination yielded 14 cells per cmm. There was a positive globulin test
and a moderate increase in albumin. The W. R. of the spinal fluid was
negative.
1. Why was the spinal fluid W. R. negative in this case of spinal
syphilis? The explanation of negative W. R.’s in spinal syphilis
is not easy. Possibly, however, in the course of years the
intensity of the process has been reduced and possibly the W. R.
has been one of the first tests to disappear.
2. How shall we explain the nerve trunk tenderness? We might
consider this to be due possibly to an inflammation about the
posterior roots. On the whole, partly on account of the situation
of the pains below the knee, it seems probable that the nerve
trunk tenderness of this case is the residuum of an alcoholic
neuritis.
=Treatment=: Under injections of mercury salicylate, there was a rapid
improvement. In fact, in the course of several months, the patient
regained an ability to walk long distances. There still remains a
certain spasticity, but the abnormal spinal reflexes above mentioned are
no longer present.
=SYPHILITIC MUSCULAR ATROPHY, probably due either to spinal
parenchymal lesions, or to root neuritis, or to both.=
=Case 33.= Joseph Graham, now 50 years of age, seemed no longer to be
able to do good work as a teamster. His arms had become weak and the
muscles had become tremulous and apparently wasted. There was also pain
in the left leg and hip. It appears that this latter symptom had been
thought to be rheumatism, having begun about 8 years before with a
sudden sharp shooting pain in the left hip, about the region of the
sciatic notch. Graham had rubbed the hip with liniment, but without
reducing the so-called rheumatism. The trembling of the hands had begun
some years later, but no wasting had been noticed except during the past
year. The pain in the leg had suddenly become so severe that a month
before medical observation he had quit work. The question immediately
arose whether Graham was not suffering from some familial form of
muscular atrophy; but according to his representations, there was
nothing of the sort in the family.
=Physically=, there was little to note. =Neurologically=, there was
more. The pupils were somewhat irregular in outline, and the right was
larger than the left. The left pupil failed to react to light, and the
right pupil reacted very slowly and with but a slight excursion. There
was no tremor of the tongue and no evidence of facial palsy nor was
there smoothing of the nasolabial folds. It was somewhat remarkable,
that in the absence of these signs, there was a marked speech defect.
The atrophy of arms, forearms, and hands was well marked, especially the
atrophy of the thenar and hypothenar eminences of the right hand. The
extended hands, especially the right, showed a marked coarse tremor.
Fibrillation was found in the muscles of the hands, forearms, arms, and
pectoral muscles. There was no dysmetria, and the diadochokinesia was
normal. Strength was diminished (dynamometer right hand, 32 kg., left 31
kg.). There was little or no atrophy of the legs, although the left
thigh was perhaps slightly atrophic and the gluteal muscles of the left
side were somewhat flabby. The patellar and Achilles reflexes were
absent on both sides. There was a slight swaying in Romberg position.
Gait was normal. There was a marked tenderness on the left side of the
sciatic notch, as well as over the entire distribution of both external
and internal popliteal nerves. This area of skin was also hyperesthetic.
There were no other neurological signs on systematic examination.
=Diagnosis=: The sensory disorder, the speech defect, and the pupillary
abnormalities seem to render the diagnosis of progressive muscular
atrophy doubtful. Nor was there any dissociation of sensations to
suggest a syringomyelia. Under such circumstances, one must fall back
upon the question of syphilis. Both blood and spinal fluid proved to be
positive to the W. R.; the globulin was increased and the albumin
markedly so; there were 61 cells per cmm., and the gold sol reaction
read 4 4 4 4 3 2 1 0 0 0.
1. Is there a relation of SYPHILITIC MUSCULAR ATROPHY to amyotrophic
lateral sclerosis? Spiller, some years since, claimed such a
relation, and it would seem with some justice.
2. How shall the present case be classified? There is evidence of
root pains (left hip). We may naturally suppose that these root
pains are reasonably good clinical evidence of a meningitic
lesion, of which the spinal fluid clinically gave a confirmation.
The fibrillation in this case somewhat suggests, however, a
central origin for the muscular atrophy. Accordingly, it would be
difficult to definitely classify the present case as either one of
meningovascular syphilis or one of central syphilis. It will be
remembered that Head and Fearnsides classify muscular atrophy
under both these headings.
=The period of SECONDARY SYPHILIS is frequently (over a third of all
cases?) MARKED BY approved signs of NEUROSYPHILIS precisely like
those of full-blown paretic or diffuse (meningovascular non-paretic)
neurosyphilis. These signs occur sometimes in association with
severe clinical symptoms, sometimes without clinical symptoms.=
=Case 34.= John Bennett, 28, was brought to the Psychopathic Hospital
much confused. His brother, who came with him, said that he had been a
very heavy drinker but had given up drinking about four months before.
He had recently had a cold but was otherwise in good health up to the
night before admission. On this night, Bennett had become suddenly
excited and went into his mother’s room, at the common home, and began
to curse her. However, he was put to bed safely, but on the next morning
began to moan continuously. After some hours of moaning, he was brought
to the hospital. Here he remained difficult to manage, being irritable,
noisy, and resistive. Questions he either would not or could not answer,
and there was even no evidence that he understood questions. However,
within a few hours, it was clear that he was slowly coming out of the
confused state. On the following day, it was possible even to rouse him
and get his name. The confusion gradually cleared still further and, by
the end of three days, he had become mentally absolutely well so far as
could be determined.
He then informed us that he had had a chancre about five or six months
before, followed by a secondary skin eruption; that he had received four
injections of salvarsan (the last, a month before admission) and three
injections of mercury. At about the time of the last injection of
salvarsan, he had developed headache with pain and slight stiffness in
the back of his neck; and a fortnight later, he began to have dizzy
spells, followed during the last week by difficulty in hearing. There
was amnesia for everything that happened after his spell of sudden
excitement on the evening before admission, and this amnesia was never
lifted for the four days that followed.
=Physically=, Bennett was very well built and muscular. Nor were there
any evidences of disease outside the nervous system. There was some
slight stiffness of the neck and slight pain on movement of the head,
which probably ought to be attributed to meningitis. The =neurological
examination= showed tendon reflexes all normal, and normal sensations.
There were, in fact, no neurological signs except that both pupils were
dilated; the left was larger than the right. Both pupils reacted to
light but reacted very poorly. They reacted much better to
accommodation.
The W. R. proved to be positive, as might well be expected in a man
whose infection had taken place less than six months before. The
globulin and albumin of the cerebrospinal fluid were in great excess, of
a degree which we clinically express by ++++. The W. R. of the fluid
also was strongly positive down to 0.1 of a cmm. The gold sol reaction
was the “paretic” type, and there were 228 cells per cmm.
1. How early may clinical evidence of neurosyphilis set in after
infection? Craig found one case of “brain syphilis” occurring one
month after infection. Frye claims a case of tabes dorsalis
developing six weeks after infection. Craig states that he has had
three cases of brain syphilis occurring within six months, and six
within a year of infection.
2. What effect did the salvarsan injections have in causing or
preventing the symptoms in this case? Nonne sums up the
neurorecidive question as follows: Since the introduction of
salvarsan therapy for neurosyphilis, paralyses of various cranial
nerves are seen more frequently. This higher frequency is in part
only apparent since more attention has been paid of late to
auditory and labyrinthine disorders. On the whole, however, it
must be considered that salvarsan does mobilize spirochete foci
which without salvarsan therapy would perhaps have remained
latent. Probably we are here dealing in some instances with fresh
infections of neurosyphilis, in other cases with a Herxheimer
reaction. Ehrlich believed that these latent foci occur
particularly in places with stagnant blood current; as, for
instance, in the narrow bony canals. This hypothesis, sufficient
in some instances, is less satisfactory for cases of peripheral
neuritis, for example.
3. What treatment is indicated? Intensive antisyphilitic treatment
is strongly indicated. Whatever may be the truth concerning the
production of neuro-recurrences (“neurorecidives”) it is certain
that the symptoms usually vanish with a continuance of salvarsan
therapy. The important point is to give efficient treatment, and
in a case like Bennett’s improvement is fairly certain unless some
serious insult occurs before the remedial efforts have been given
time. It is still an open question whether intraspinous treatment
is more efficient in such cases than intensive intravenous
injections of salvarsan. In Bennett’s case diarsenol was injected
intravenously twice a week in 0.6 gm. doses, reënforced with
intramuscular injections of mercury salicylate and potassium iodid
by mouth. Under this treatment improvement began slowly and in a
few months he was symptomatically well and after three months his
tests were practically negative.
=JUVENILE PARETIC NEUROSYPHILIS (“juvenile paresis”) with OPTIC
ATROPHY.=
=Case 35.= Mary Coughlin, a blind girl of 16 years, was brought to the
hospital in a state of great excitement, laughing and crying
alternately. The neurologist is entitled to think of blindness, and
particularly of the optic atrophy which Mary showed, as probably due to
syphilis. However, there was no history of syphilis in the father, who
died in an accident at the age of 40, or the mother, who died at 45, of
heart trouble. An elder sister was married and well; two younger sisters
were living and well. The fifth sibling, a boy, had died in infancy.
There had been no miscarriages. In fact, the only point in favor of
syphilis was the somewhat far-fetched point that the younger brother of
the patient had died in infancy.
The patient’s history was rather suggestive of some other diagnosis. Her
birth had been normal, she walked and talked at 13 months, was at school
from six to twelve, reaching the seventh grade, and was considered
bright. At three years of age, she had been run down by a car and
dragged under the fender for a considerable distance. Her head was hurt
but the patient did not lose consciousness in the accident. Fainting
spells began at 11, in which spells the patient would lose consciousness
for a minute or two. About this time, the patient’s eyesight had begun
to fail, and for some four years she had been entirely blind. Headaches
had come on of late.
The Coughlin case, except for the above-mentioned suspicion of
syphilitic optic atrophy, might be regarded as an unusual example of a
post-traumatic disease.
We found her to be fairly well developed and nourished; there was a
deformity of the lower half of the sternum and of the third and fourth
ribs on the right side. There were no other physical phenomena found
upon systematic examination. The left pupil still reacted to light; the
right failed to react, but this lack of reaction could not be regarded
as of Argyll-Robertson nature on account of the finding of optic atrophy
with the ophthalmoscope.
=Mentally=, it appeared that the patient’s retention of school knowledge
was poor, though her blindness for four years had doubtless given her
little opportunity to keep such information fresh. Rather strangely,
Mary gave utterance to many delusions: first, expecting to receive her
sight by an operation on the head; second, to write a book of her
doings; third, to buy a house for the children; fourth, would pay $3000
for the house, earning the money by working at a tailor’s or as a
trained nurse; fifth, to go on the stage to earn money by dancing;
sixth, will have lots of money.
One of Mary’s characteristic statements is as follows: “Won’t it be
lovely when I can see Dr. H.’s face in heaven or some other lovely
place? Dr. H. was a grand doctor to me, and when we get together again
we are going to Tremont Temple and keep us together. I am going to do
some dancing and play the piano. I am going to graduate at the high
school and go to Trinity College in Washington, and I hope I shall be a
faithful keeper of mother’s tomb.”
The patient was at times euphoric and expansive.
At this stage, what with optic atrophy, euphoria, and expansive
delusions, we should perhaps be entitled, had Mary been an adult, to
offer the diagnosis GENERAL PARESIS. In fact, on the whole, any other
than a syphilitic cause for the optic atrophy was exceedingly doubtful.
Brain tumor of a nature to produce optic atrophy might very improbably
last so long as five years. There was no evidence of any intoxication at
the time when the blindness occurred.
The W. R. was positive in the blood and spinal fluid; there was a
positive globulin test, and an excess albumin as well as 15 cells per
cmm.
1. What is the significance of Mary’s trauma at three years? So far
as we are aware, none.
2. What light could be thrown by a W. R. study of the family? In
some instances, much light is thrown; in the present case all
three living sisters of the patient have been examined and their
serum W. R.’s have been found negative.
3. What is the prognosis of juvenile general paresis? Death within a
few years, as in general paresis in adults. The patients live
rarely more than four or five years after the onset of symptoms.
Mary Coughlin died a year and a half after the above examination,
namely, in her eighteenth year, some seven years after the onset
of symptoms.
4. What can be said of treatment? A few favorable results have been
reported after intraspinous therapy (Swift-Ellis). Too little work
has been done with systematic treatment of juvenile neurosyphilis,
both paretic and non-paretic, to permit important conclusions at
this time.
5. How can we explain the infection of this sibling whereas the
others, both younger and older, escaped? It would seem that we
would have to discard the hypothesis of a congenital infection and
consider that it was acquired accidentally during the lifetime of
the patient. Considering the prevalence of syphilis it is rather
to be wondered that more such cases of “innocent” infection do not
occur in children. We may recall how many instances of juvenile
gonorrhea occur. In a case as this where the symptoms calling
attention to syphilis necessarily occur so long after the original
infection it is practically impossible to trace the origin of the
infection.
=The diagnosis of JUVENILE PARESIS is often easy.=
=Case 36.= Theresa Mullen, an under-sized girl of 12 years, presented a
remarkable appearance due to congenital amputations of the fingers and
toes. She lay in bed, drivelling and making unintelligible cries. It
appeared that the patient weighed about 12 pounds at birth and was very
fat; that she had been fed on condensed milk, had survived cholera
infantum, whooping cough, and, as the parents said, “two kinds of
measles.”
Theresa had gone to school at 5 years, reaching the third grade at the
age of 9; but at this time, she began to lose ground and was put in a
class for backward children. Moreover, at about this time, the teachers
noticed spells of causeless laughter and meaningless twisting back and
forth. Theresa would also scream at night, looking about the room; once,
rising and crying, “Take him away, that black thing,” though no
appropriate object was present. There had been little or no complaint of
headache. Theresa had been deteriorating for some time, and for a year
past had been having increased difficulty in walking. For two months the
child had not spoken intelligible words; for the last week, she had been
incontinent.
The =diagnosis= was almost obvious from the manual and pedal deformities
taken in connection with the saddle-back deformity of the nose. It was
interesting in connection with the contentions of W. W. Graves, that the
scapulae were scaphoid in type.
Accordingly, the history given by the parents seemed consistent enough.
The parents were both 36 years of age, having married at 23. The first
pregnancy was a miscarriage at two months, of unknown cause. Theresa
came next; thirdly, came a miscarriage at three months; fourthly, a
girl, who is not strong or well physically, has suffered much from
headaches and sore throat, but is fairly bright. The fifth pregnancy
resulted in a boy, who is bright but of under-size. Three more
pregnancies resulted in miscarriage.
Taking into account the above-mentioned physical characteristics, the
personal history, and the family history of Theresa, the diagnosis could
hardly be in doubt even in the absence of a lack of pupillary reaction
to light on the right side, infantilism of genitalia, positive W. R.’s
of serum and spinal fluid, positive globulin, and excess albumin, 34
cells per cmm. and the paretic type of gold sol reaction which were
found.
The =prognosis= of this case appears to be rapid deterioration,
terminating in death within a few months. Now and again, however, some
such cases spontaneously improve. Such a case as that of Theresa Mullen
is always disheartening in itself but suggests the social value of
Wassermann tests in the other members of the family. The other children
of the Mullen family proved to be suffering also from syphilis, since
their blood sera all showed a positive W. R.
1. What is the characteristic age of onset in JUVENILE PARESIS? An
impression has prevailed in some quarters that the typical onset
of juvenile paresis is in the adolescent years, and Clouston’s
first case (1877) developed in a boy of 16. Thierry’s 58 cases,
developing from the 8th to the 20th year, averaged 14 years of age
at onset. Mott’s 22 cases from the 8th to the 23d year, averaged
17 years at onset. According to Clouston, juvenile paresis
develops most often at puberty (15 to 17 years). It is sometimes
claimed that cases developing symptoms early live longer, and that
juvenile cases developing symptoms after the 20th year run a short
course. For a case developing in the 5th year, see John
Friedreich, Case No. 77.
2. What may be concluded from the physical signs (congenital
amputations) present in this case before the development of mental
symptoms? Some cases of juvenile paresis appear to show no
physical signs whatever in childhood. While these amputations
might be the accidental result of a difficult delivery, it is more
probable that they are due to a syphilitic process.
[Illustration:
Juvenile paresis—congenital amputation of digits. This case reached
fourth grade in school before deterioration.
]
=CONGENITAL SYPHILIS is apparently capable of producing simple
FEEBLEMINDEDNESS (that is, a form of disease non-paretic,
non-tabetic, without special tendency to progression, and without
tendency to vascular insults).=
=Case 37.= Isaac Goldstein was a small boy of six years and seven
months, with a father known to be suffering from general paresis. The
child was very irritable and nervous and very difficult to manage, but
would hardly have been the subject of medical attention except in a
family study suggested by the paresis of the father.
The child had been born at term and had apparently undergone a normal
development. Physically, he showed no definite signs of congenital
syphilis. In fact, the physical examination was to all intents and
purposes negative. The W. R. of the serum, however, proved to be
positive. Mental tests showed that his mental age was that of a child of
a little over five years. Taking all things into account, it is probable
that he should be regarded, therefore, as somewhat retarded mentally.
1. Is syphilis answerable for the mental retardation in this case?
Provided that the family is free from feeblemindedness and mental
disease, it would seem that the retardation of a congenital
syphilitic should perhaps be regarded as syphilitic in origin. Of
course, the institutions for the feebleminded have not shown
exceedingly high percentages of syphilitic children in various W.
R. surveys; still, the percentage of positive reactions in
institutions for the feebleminded is clearly higher than the
incidence of congenital syphilis shown in the population at large.
Hence, we may conclude that syphilis is one of the etiological
factors in the production of feeblemindedness. Dr. W. E. Fernald,
of the Waverley School for the Feebleminded, has recently pointed
out that the syphilitic cases belong rather in the lower grades
(idiots and imbeciles) of feeblemindedness than in the higher
(morons).
2. Can we guess what the pathological anatomy and histology of the
brain may be in such cases? The Waverley studies now in process
seem to indicate that some cases have little or no gross
alterations, but show a few slight traces of lymphocytic
accumulations discovered upon extended search, and a certain
tendency to the appearance of rod cells in various foci. But the
whole matter is still _sub judice_. It is a question whether these
traces of chronic inflammation are the residuals of a more active
process or the beginnings of a process that is about to be more
active.
3. How characteristic is a positive W. R. in the serum of a child
without physical stigmata of congenital syphilis? If we limit the
term _stigmata_ to the major and more important signs, we must
reply that it is not unusual to find positive W. R.’s in sera of
physically normal-looking children. Except in family studies, such
cases will often escape notice, either because there are no
stigmata whatever, or because such stigmata as exist are of a
minor nature and regarded as unimportant anomalies. Some of these
cases occur in the clinics later in life as so-called _syphilis
hereditaria tarda_. If one wishes to discover these cases with
late development of symptoms before their full bloom, the most
obvious method is to examine carefully the children of known
syphilitics.
[Illustration:
Scaphoid Scapulae.
]
=JUVENILE TABETIC NEUROSYPHILIS (“juvenile tabes”); TREATMENT.=
=Case 38.= The point in presenting Archibald Sherry, a JUVENILE TABETIC
of 12 years on admission, is perhaps to exhibit pride in therapeutic
results.
There was little or no doubt of the diagnosis; in an adult, the
phenomenon would be called tabes dorsalis with a question of general
paresis. The right pupil was larger than the left and reacted neither to
light nor to distance. There was a slight tremor of the tongue and of
the outstretched hands. The knee-jerks and ankle-jerks could not be
obtained, nor could the periosteal reflexes in the legs. There was a
slight unsteadiness in the gait and in various finer movements, and a
slight ataxia of the legs. There was not a classical Romberg sign but
there was slight swaying in Romberg position. The teeth were
Hutchinsonian. For the rest, the physical examination was practically
negative.
The family history was of interest. On the paternal side there was
nervousness as well as alcoholism and degeneracy. The maternal
grandmother had cancer. Archibald’s father was immoral and alcoholic.
There was a girl four years older than Archibald, who, though nervous
and unstable, has shown no signs or symptoms of syphilis and does not
yield a W. R. in blood or spinal fluid.
Archibald himself was born at term, a large child, who, however, lost
weight rapidly, developing a marked skin eruption on head and back three
weeks after birth. This skin disease lasted for a month and a half and
then spontaneously disappeared. Archibald remained weak and sickly, not
walking until three years of age. However, he did well in school up to
the end of his 11th year, when he failed to keep up with the children.
He had been an amiable child and had gotten on well with his playmates.
Some time in his 10th year physical disability had begun; there was
numbness in the legs with weakness; at times, actual inability to walk.
The right pupil was noticed by the mother to have increased in size; the
eyelashes had turned white. There was pain over the left eye and a
feeling of weight on top of the head. Speech became difficult or even
confused.
Consistently enough, the W. R. both in blood and spinal fluid was
positive. Globulin and albumin were present in large amounts; there were
150 cells per cmm.
Granting that this be in some sense a case of juvenile tabes we may
raise a doubt whether the case is one of congenital syphilis. The W.
R.’s of the blood of both father and mother are negative. Syphilis is
denied by them. The nervous and unstable older sister failed to show
definite symptoms of syphilis or a positive W. R. There had been no
miscarriages or stillbirths. The question arises whether the
Hutchinsonian teeth do not indicate congenital syphilis. It appears,
however, that it is possible to develop Hutchinsonian teeth if syphilis
is acquired before the teeth are formed. We have no data as to how or
why this particular baby should have acquired syphilis, if he did so
acquire it, at the age of three weeks. On the whole, sceptics may doubt
our suggestion that the case is one of acquired juvenile tabes. Possibly
the question is academic so far as treatment is concerned.
=Prognosis=: The rarity of juvenile tabes is such that little can be
said as to prognosis. Three and a half years have passed since a few
injections of salvarsan were made. The pains above mentioned rapidly
disappeared, the gait became steadier, the attacks of confusion ceased,
and the speech improved. Unfortunately, on account of a lack of
coöperation on the part of Archibald’s mother, we have been unable to
continue treatment. However, we have from time to time followed the
patient in his home and he seems to have shown no falling back after the
initial improvement. It would be of great value could we know the
situation in the spinal fluid at the present time.
1. Is there any explanation why paresis should occur in some
juveniles and tabes in others? There is no available explanation
for this difference nor any for the characteristic early optic
atrophy of juvenile tabetics.
Be frustrate, all ye stratagems of Hell,
And, devilish machinations, come to nought!
Paradise Regained, lines 180–181
III. PUZZLES AND ERRORS IN THE DIAGNOSIS OF NEUROSYPHILIS
This part of the case collection, dealing with puzzles and errors, is
ushered in by six cases (39–44) drawn from a group of errors in
diagnosis made some years since at the Danvers Hospital. These six are
autopsied cases. Attention is called to the fact that modern methods of
diagnosis might have prevented the errors.
=DIFFUSE NEUROSYPHILIS (“cerebrospinal syphilis”) versus PARETIC
NEUROSYPHILIS (“general paresis”). Autopsy.=
=Case 39.= Caroline Davis, dead at 49 years, was a case of error in the
diagnosis of general paresis. Like Cases 40 to 44, Case 39 was
diagnosticated by the full Danvers staff as a case of general paresis;
however, it must be added, before the days of the W. R. and the modern
methods of systematic diagnosis. As will transpire in the sequel, there
is a large question whether Case 39 is not after all really a case of
neurosyphilis, possibly not of the paretic group. The details are as
follows:
Caroline Davis was a normal school girl till 15, apt in studies, mill
worker till marriage at 18; one child, dead (cause unknown). Habits
good. Moderate deafness set in in the forties and in 1901 patient became
completely deaf in three months’ time. In 1905 she became unable to take
care of her house and had a shock in which the right leg was affected.
On commitment patient showed good development and nutrition with slight
enlargement of capillaries of cheeks, redness and roughening of skin of
right ankle. Teeth absent. Slight radial and brachial arteriosclerosis.
Urine negative. Sluggish pupil reactions to light both directly and
consensually. Deafness absolute, bone conduction defective. Arm reflexes
brisk, knee-jerks equal, brisk. Bilateral Babinski reaction more marked
on the right side, tremor of tongue, Romberg’s sign, gait defective.
Speech stumbling, writing clear, without tremor.
Communicated by writing only. Consciousness normal, disorientation for
day of month, for place (misnames hospital) and for persons (recognizing
nurses, not patients).
Patient wrote many letters complaining of pain, headaches and especially
of pain in the abdomen and side. The patient was thought to show a
slight defect of memory, but her deafness rendered diagnosis difficult.
The patient died suddenly on May 23, 1908, shortly after supper, falling
backwards, and dying in five minutes with marked respiratory distress.
=Post Mortem Findings.= The =cause of death= was not clear. The heart’s
blood and cerebrospinal fluid were sterile. There was a small hemorrhage
in the anterior part of the right ventricle derived from a small artery
of the caudate nucleus. There was about 400 cc. of blood between the
dura mater and the pia mater. There was a slight sclerosis of the basal
and Sylvian arteries. The brain substance was uniformly softer than
normal.
It is possible that the hemorrhage had taken place some time before the
patient’s fall and that the brain substance had swollen in consequence.
Just before the fall she had a weeping spell.
The =anatomical diagnoses= were as follows:
Obesity, unequal pupils, fresh wound near left ear, edema of legs,
slight focal adhesive pleuritis, hypostatic congestion of lungs, chronic
endocarditis, chronic myocarditis, congestion of kidneys, congestion of
pancreas, subacute splenitis, chronic adhesive pelvic peritonitis,
hematoma and cystic condition of Fallopian tubes, =calvarium dense= and
thick, subdural hemorrhage, slight =chronic leptomeningitis=, general
=cerebral atrophy=, marked in tips of =frontal lobes=, old =cyst of
softening= between left corpora albicantia and optic chiasm, small
punctures of left ear drum, drums opaque, =chronic spinal
leptomeningitis=; brain weight, 1190 grams.
There were marked firm interadhesions between dura and pia throughout. A
lumbar puncture soon after admission in 1907 had shown:
Per cent
Endothelial cells 10
Lymphocytes 30
Plasma cells 0
Phagocytes 0
Polymorphonuclear cells 51
Unclassified 9
Fibroblasts 0
Cells in 100 fields 125
It will be noted that the lumbar puncture yielded no plasma cells and
yet showed 30% of lymphocytes. Alzheimer, in 1904, attempted to
distinguish the histology of the cerebral syphilitic from that of the
general paretic, maintaining that _lymphocytosis was the characteristic
feature of the ordinary neurosyphilitic_, _whereas plasma cells were
associated with the lymphocytes in the paretic_. This case showed
=lymphocytic= deposits. To be sure, they were decidedly subordinate in
the cerebral cortex, cerebellum, and basal ganglia, to the marked
evidences of nerve cell destruction, although there were perivascular
infiltrations about a few of the larger vessels in the white matter of
the cerebral cortex.
The spinal cord, however, showed a most severe infiltration, especially
in the gray matter, where the infiltration accompanied severe nerve cell
changes and arterial changes. The pia mater of the spinal cord was also
packed with mononuclear elements, among which, however, no plasma cells
could be found.
But although the inflammatory changes in the shape of lymphocytosis were
relatively more prominent in the spinal cord than in the cortex, yet the
cortex yielded evidence of an exceedingly marked destructive process.
Perhaps no layer of any of the areas of the cortex examined failed to
show some atrophic alteration. The upper layers of the cortex were
everywhere more severely diseased than the lower layers. Here we are
dealing with an instance of an active meningomyelitis and subcortical
encephalitis. It is, of course, probable that the W. R., had it been
performed, would have been positive in this case. On the basis of the
histology, we are inclined to regard the clinical picture in this case
as belonging among cases of NON-PARETIC DIFFUSE NEUROSYPHILIS.
This case, as also the next several, is especially instructive in
teaching the difficulty in differentiating paretic and non-paretic
neurosyphilis. Not only is this difficulty met in clinical diagnosis,
but in pathological diagnosis as well.
The histological diagnosis depends in large part on the work of the
Nissl-Alzheimer school, which has received great recognition. At the
present time, however, there is beginning to be considerable doubt as to
the entire validity of this teaching. At any rate there are many
borderline cases in which the differentiation is well nigh impossible.
In this case note chronic meningoencephalitis, with cortical
degeneration, in the absence of plasmocytosis.
From the clinical standpoint the intensity of the W. R., the character
of the gold sol reaction, and the result of therapy have added new
points in differentiation. Much more work controlled by autopsies is
still needed, however, to put us on sure ground in borderline cases.
=VASCULAR NEUROSYPHILIS(?) versus PARETIC NEUROSYPHILIS (“general
paresis”). Autopsy.=
=Case 40.= Case 40 like Case 41 was an error in the diagnosis of general
paresis which might be regarded as academic rather than practical. Both
were cases of arteriosclerotic brain disease with severe cerebellar
involvement. Case 40 had a spinal cord that was not quite normal. There
was a tabetiform lesion in the cervical spinal cord (not elsewhere),
together with a unilateral degeneration suggesting in some respects a
radicular origin. The most striking feature, however, of Case 40 as in
Case 41, was a lesion of the cerebellum. In Case 40 the dentate nuclei
were in large part destroyed by cysts of softening, although the
cerebellar cortex was fairly well preserved on both sides. The details
of Case 40 are as follows:
H. F., male, gear maker, born 1850.
=Heredity.= Maternal grandmother insane. Mother insane at 52, became
demented and lost use of limbs, died at 71. Aunt insane.
=Personal History.= Common school education. Capable workman till within
a few months. Early in life alcoholic. Drunk almost every week until
1899 or 1900. Irritable, nervous, selfish, loose in relations with
women. Venereal disease denied by wife. Married in 1883. Three frail
children. No miscarriages. Neuralgia in 1901 or 1902.
January, 1904, patient left carriage shop on account of mistakes in
work, became more pleasant, childish, fearful, talkative, did funny
things, later became vagrant, stole from fruit stores, smoked cigarettes
picked up in the street, and became restless and irritable.
Committed to Danvers, June 24, 1904, with slightly enlarged heart,
somewhat heightened blood pressure, and a slight sediment of epithelial
cells in urine.
Romberg’s sign was present, but there was little or no demonstrable
incoördination otherwise. Very slight tremor of fingers. Left knee-jerk
absent, right obtained on reinforcement. Achilles jerk absent. Triceps,
wrist and normal plantar reflexes present. Pupils react to
accommodation, but very slightly, if at all, to light. Sensations normal
except in legs. The legs show preservation of tactile and temperature
senses, but abolition of pain sense except over dorsum of foot.
Speech showed slurring of syllables and “brigrade” for “brigade.”
Disorientation for time, place and in part for persons. Admitted that
his work had been deficient but regarded himself as well. Emotionally
variable, crying at times and suddenly becoming jocular. Eloped July 3
and somehow reached his wife’s house in a neighboring city.
Euphoria persisted. The pupils continued Argyll-Robertson, and the
knee-jerks remained absent. Became oriented for place and partially as
to time (month and day of week correct).
During 1905 failure became rapid, with ataxia of legs, persistent
euphoria, and loss of weight.
Convulsions, regarded as general paretic, developed in 1906. Death
sudden, December 7, 1906.
=Post Mortem Findings.= The =cause of death= was streptococcus
septicemia, probably derived from a gangrenous bronchopneumonia or
related with a small thrombus of the right auricular appendix. There was
also an acute purulent otitis media, mastoiditis and sphenoidal
sinusitis, as well as extensive decubitus. From this decubitus or from
the intestinal tract may have been derived the numerous colonies of
_bacillus coli communis_ which developed on plates from the
cerebrospinal fluid.
=Arteriosclerosis= was little in evidence, being confined to the
coronary, right vertebral and carotid arteries (slight in all). _Cysts
of softening existed in the posterior part of each dentate nucleus_ and
may probably be interpreted as indicating vascular disease.
=Chronic disease outside the nervous system= was prominent and in part
suggestive of senile findings; milky patches of pericardium, adhesions
about liver and gall-bladder, adhesions about spleen, adhesions and
fibrous thickening of parietal peritoneum, adhesions in both pleural
cavities, chronic diffuse nephritis, hypertrophy of bladder wall, dense
calvarium, dural adhesions.
The =nervous system= showed several unexpected features. The _absence of
chronic leptomeningitis_ was striking: the pia mater was everywhere
delicate and transparent except that the walls of the cerebellar and
chiasmal cisternæ were thickened and that there were slight opacities
along the sulcal veins of the convexity. Brain weight 1090 grams. There
was a generalized =sclerosis and pigmentation of the cerebral cortex=.
The sclerosis varied in degree and was most marked in the prefrontal
regions, the anterior halves of the superior frontal gyri, the middle
third of the right precentral gyrus, the region of the splenium on the
left side, and the sagittal rami. If the _bacillus coli communis_ found
in the cerebrospinal fluid had any effect upon the consistence of the
brain, obviously hard to prove in a brain of leathery consistence at the
outset, it was shown only in the right Rolandic area in the vicinity of
the sclerotic part of the precentral gyrus. =Granular ependymitis= of
all ventricles. Weight of cerebellum, pons and bulb, 135 grams.
Perhaps the most remarkable feature of all in the case was the
occurrence of =cysts of softening= in the posterior part of each
=dentate nucleus=. For discussion, see Case 41.
=VASCULAR NEUROSYPHILIS (?) versus PARETIC NEUROSYPHILIS (“general
paresis”). Autopsy.=
=Case 41=, like Case 40, was one of arteriosclerotic brain disease with
severe cerebellar involvement. Here is another case in which the Danvers
staff made a diagnosis of general paresis without dissenting voice.
There were some tabetic symptoms, and the spinal cord at autopsy did
show a moderate lymphocytic infiltration of the meninges, entirely
consistent with the picture in the spinal fluid. In this case, the
dentate nuclei of the cerebellum were not destroyed as in Case 40, but
were affected by cell atrophies of variable degree in different parts of
the nuclei. There was also a severe gliosis of the cerebellar cortex.
The left hemisphere of the cerebellum was more severely diseased than
the right. The cortex showed far more marked and generalized cell
atrophies throughout the layers than did Case 40. The details of this
case, which was that of a colored coachman, Samuel North, are as
follows:
He was born in 1871. Learned to read and write at school. Stableman and
coachman. Alcoholic till 1902. Took much quinine, possibly impairing
hearing thereby. Memory impaired and growing worse since 1902. Gait
unsteady for a longer but unknown period. August 13, 1907, wandered
about, instead of attending boot-black stand, muttered, talked
incoherently. In the next few days talked about religion and apparently
had hallucinations of hearing. Committed August 16, 1907.
On commitment stoop-shouldered, flat-chested. Gait staggering.
Unsteadiness in Romberg’s position. Incoördination of arms and fingers.
Coarse tremor of tongue. Tremor of lower jaw. Exaggeration of left
knee-jerk and diminution of right. Exaggerated Achilles jerks. Spurious
left ankle clonus. Questionable Babinski reaction of left side.
Abdominal and epigastric reflexes present but cremasteric absent. Left
pupil smaller than right and fails to react to light. Reaction of right
pupil sluggish. Moderate defect of hearing of both sides.
During the first week the patient developed hallucinations of sight and
hearing, but of no other senses. Disorientation for time, place, and
persons. Answers to arithmetical problems given with assurance but as a
rule incorrectly (as 17 and 32 are 90; 18 divided by 3 is 88).
Handwriting scarcely legible. Memory poor, especially for recent events
(recalled a lumbar puncture as an exercise in baptism). Impressibility
and attention poor. Euphoria.
Death after gradual failure July 29, 1908.
Lumbar puncture showed: Per Cent.
Endothelial cells 9
Lymphocytes 81
Plasma cells 6
Phagocytes 0
Polymorphonuclear cells 4
Unclassified 0
Fibroblasts 0
Cells in 100 fields 700
=Post Mortem Findings.= The cerebrospinal fluid showed a pure culture of
_Bacillus coli communis_, and the heart’s blood showed many colonies of
an unidentified bacillus. Culture from mesenteric lymph nodes sterile.
The =cause of death= is somewhat in doubt. There was an early pneumonic
process with fibrinous pleurisy, and there was an early acute
hemorrhagic ileitis with a very slight overlying peritonitis and slight
corresponding enlargement of mesenteric lymph nodes. There was an
infection of the meninges with _Bacillus coli communis_.
Evidences of =chronic disease outside the nervous system= were: coronary
and pulmonary arteriosclerosis, chronic fibrous endocarditis, mitral
sclerosis, aortic sclerosis with calcification, chronic splenitis,
chronic interstitial nephritis, hepatic atrophy (wt., 900 grams),
thickening of cartilaginous portion of right auricle (old trauma), scars
of apices of lungs.
The =calvarium= was dense and the =dura mater= everywhere adherent. The
=arachnoidal villi= were but slightly developed, but there was one small
focus of cortical herniation through the dura mater of the left middle
cranial fossa. The =pia mater= was delicate except for slight opacities
along sulci. There was some pial thickening over the region of the
interparietal sulci on both sides. There was pial pigmentation
anteriorly and superiorly.
There is no gross evidence of intracranial arteriosclerosis, except (1)
that afforded by the lesions of the dentate nuclei of the cerebellum
mentioned below and (2) the swerving to the right of the basilar artery,
possibly due not to arteriosclerotic lengthening of the artery but to an
unusual shape of the pons (see below).
The =brain= weighed 1245 grams (cerebellum and pons 165 grams). =The
anatomical diagnoses of central nervous system= were:
Slight general encephalomalacia (post mortem imbibition of fluid, 31
hours). Slight gliosis of right prefrontal and frontal gyri. Slight
gliosis of right optic thalamus. Generalized granular ependymitis,
especially near fornix and about foramina of Monro. Anomaly of pons (not
gliotic, but possessing far more white matter on the left side than the
right). Severe arteriosclerosis confined to the dentate nuclei of the
cerebellum.
As we now look over the data in Cases 40 and 41 we are inclined to ask
the question, whether modern systematic diagnosis would not have shown
these cases to be NEUROSYPHILITIC? One is inclined to answer this
question in the affirmative, on the basis that Case 40 showed somewhat
questionable Argyll-Robertson pupils, and Case 41 showed unilateral
Argyll-Robertson effect. Both cases showed Romberg sign, but the dentate
nucleus and other cerebellar disease in each case may in some way have
contributed to or imitated this phenomenon. Whether Case 40 was a
tabetic must remain a question, but Case 41 must be regarded as a case
with spinal and meningeal changes highly characteristic of syphilis.
=VASCULAR NEUROSYPHILIS plus TABETIC NEUROSYPHILIS (“tabes
dorsalis”) simulating paretic neurosyphilis (“general paresis”).
Autopsy.=
=Case 42.= The case of Elizabeth Brown was at one time carefully studied
by Dr. A. M. Barrett in his work on mental diseases associated with
cerebral arteriosclerosis and, like Case 43, was one in which tabes
dorsalis was a factor. Elizabeth Brown’s maternal grandfather and mother
were insane; there had also been insanity in a sister. Mrs. Brown was
struck on the head at 44, and was unconscious for an hour, but there
were no sequelae to this accident. At 48, there was a shock, or
apoplectiform attack, followed by unconsciousness for two hours and by
left hemiplegia, right ptosis, and thick speech. Mrs. Brown began to
walk again after two weeks, but was found to be forgetful and
fabulatory. She seemed at times to be hearing music, and somewhat
repeatedly became helpless and unable to walk. She could not remember
from day to day, showed incontinence of urine and feces, and was brought
to the Danvers Hospital. The physical and mental deterioration was
progressive. There were some signs of organic brain disease. The
musculature was especially flabby on the left side. The left angle of
the mouth drooped, and the left nasolabial fold was smoothed out. The
arm movements were ataxic, the tongue protruded to the left, the right
pupil reacted but slightly to light (eye blind from cataract), the
knee-jerks, Achilles, wrist, and elbow reflexes, were absent. The
patient was unable to stand, and there was a marked tremor of the hand,
tongue, and lips. There was a zone of anesthesia for pain and tactile
stimulation extending round the body, from the 3d to the 6th rib, and
there were symmetrical areas of anesthesia on the inner surface of the
forearms and the legs.
The =autopsy= showed a =general arteriosclerosis= with =chronic= and
=acute meningitis=. The brain weighed 1110 grams; the =pia mater= was
moderately thickened; the basal vessels were highly arteriosclerotic.
The brain itself, however, normal externally, upon dissection, showed a
number of small cysts irregularly scattered in the white substance. The
basal ganglia were porous, and there were several small cysts in the
pons. =Microscopically=, there was evidence of severe vascular disease,
involving not only the arteries but also the veins. It was the
superficial rather than the deep arteries that were more often attacked.
There was a marked =perivascular gliosis=. Extensive search yielded _no
evidence of lymphocyte infiltrations_, either in the brain or in the
spinal cord.
The spinal cord showed degenerations in both the lateral and posterior
columns, of which the explanation may possibly be like that in our
paradigm, Case 1.
Is the case of Elizabeth Brown one of neurosyphilis? We cannot
definitely say on account of the non-availability of the modern
systematic tests, but it may well be that the case, although certainly
not one of paretic neurosyphilis, was one of TABES WITH VASCULAR
COMPLICATIONS.
=TABETIC NEUROSYPHILIS (“tabes dorsalis”) with symptoms of cerebral
origin producing a picture resembling taboparetic neurosyphilis
(“taboparesis”). Autopsy.=
=Case 43.= Robert Allen was the fifth case of error in the diagnosis of
general paresis analyzed some years since from the staff meeting records
of the Danvers Hospital. The Allen case resembles the case of Elizabeth
Brown in that there was a combination of tabetic phenomena with cerebral
lesions of a non-paretic character at autopsy. But although there seemed
to be an utter absence of inflammatory cells (lymphocytosis) in the case
of Elizabeth Brown (42), there were some slight perivascular cell
accumulations in the Allen case, with a few mononuclear cells suggestive
of lymphocytes. The cerebrum, however, failed to show plasmocytosis. It
was seriously diseased, showing a marked neuroglia proliferation about
the atrophic nerve cells.
Robert Allen was a printer coming from a long-lived race. The following
are the main facts:
Married in 1875 (two children, healthy); again married in 1893 (one
child, healthy). Compositor from 1890. In 1898 and 1899 girdle and
lancinating pains. Thereafter for several years gait was unsteady.
During 1904 and 1905 freedom from pains and improvement in gait but
gradually increasing irritability and nervousness. Stopped work on last
of March, 1905, owing to sudden increase of irritability, emotionality,
boastfulness, expansive schemes, and ataxia.
Habits: no tobacco, very little alcohol at long intervals. No drug
habits, no sexual irregularity known.
Committed to Danvers April 3, 1905, with slight muscular development,
poor nutrition, acne, irregular, poorly preserved teeth, gingivitis,
flat-foot, slight radial arteriosclerosis, slight arcus senilis, a few
hyaline casts, leucocytes, epithelial cells, and trace of albumin in the
urine, scar in sulcus, and enlarged inguinal lymph nodes.
Ataxic gait, Romberg’s sign, fibrillary twitching of chest, abdominal
and facial muscles when standing; right pupil slightly larger than left,
pupillary margins irregular, light reactions (electric bulb test) both
consensual and direct absent, slight pupillary reaction in
accommodation; biceps, triceps and wrist reflexes lively and equal;
abdominal, cremasteric and plantar reflexes normal, knee-jerks, Achilles
and front taps negative even on reinforcement.
The patient himself stated that his ataxia began in 1904, that he had
been under treatment for swelling of legs and feet and pain in limbs
since 1903, and that there had been some trouble with limbs since 1895.
He had been told that his disease was lead-poisoning. About three weeks
before commitment patient said he had had an attack of unconsciousness.
The patient’s speech showed considerable defect. Words were pronounced
slowly with slurring and tripping especially of the labials. Orientation
perfect. School knowledge well retained. The easier arithmetical
problems were accurately performed. Memory imperfect for minor recent
events. Estimations of space and time often very imperfect. Variability
of mood, sometimes euphoric, sometimes tearful and irritable. Occasional
expansive estimates of personal powers (“Can lift three five-hundred
pound weights with one finger”). Indistinct expansive financial ideas.
The patient continued oriented, euphoric, expansive, untidy, till
October, 1905, but on October 12 developed an infection at the site of a
callus on the sole of the foot and died with pyemic symptoms, October
17.
=Post Mortem Findings.= The =cause of death= was streptococcus
septicemia with acute ulcerative colitis, acute splenitis, bilateral
purulent pleuritis, multiple infarctions of lungs.
There were no signs of =chronic disease outside the nervous system=
except a moderate thickening of the mitral valves, and slight dural
adhesions.
The brain weighed 1450 grams. The vessels at the base showed a slight
degree of sclerosis. There was a slight opacity of the frontal,
parietal, and temporal pia overlying slightly atrophied convolutions,
whose surfaces showed in a few places slight cuppings. The ependyma over
the thalami and the floor of the fourth ventricle was finely roughened.
The spinal cord showed a typical TABES DORSALIS.
Although we probably cannot regard either Case 42 or Case 43 as a case
of paretic neurosyphilis, and although it must remain doubtful whether
they are cases of any form whatever of neurosyphilis (in the absence of
the modern tests), yet it seems clear that both these cases may very
well have been cases of neurosyphilis on account of the existence of a
definite tabetic process in each. The symptoms of these cases, like
those of Cases 38 to 41, suggest how difficult it must be _to make a
clinical diagnosis of general paresis safely without employing available
laboratory tests_. Yet how frequently in the past have neurologists
brought data concerning various phenomena in long series of so-called
paretics in which the error of diagnosis was certainly between 5 and 15%
and frequently still greater. The entire question of the symptomatology
of paretic and non-paretic neurosyphilis, therefore, needs re-opening
and revision.
=CEREBRAL GLIOSIS (probably non-syphilitic) producing the clinical
picture of paretic neurosyphilis (“general paresis”). Autopsy.=
=Case 44.= John Hughes was a hostler, and later assistant with a
wholesale drug company, with which he remained for 32 years. He had been
moderately but constantly alcoholic all his adult life up to 50 years of
age, and at 45 had had an attack of so-called nervous prostration, in
which his head had troubled him and he had been seclusive. At 49, he had
a serious attack of otitis media, associated with delirium, swelling of
the feet, and what was called rheumatism. After this attack of otitis
media, Hughes appears to have been not altogether right.
At 53, after a quarrel with his employer, Hughes quit work, began to
trade a little in hens and pigs, became forgetful, especially of recent
events, and did “a variety of peculiar things.” He was a married man but
he had no children. There had been miscarriages but of unknown origin;
venereal disease was denied. At 55, a week before admission, Hughes had
a spell of unconsciousness for several hours, after which his speech was
thick, and restlessness, insomnia, and a wandering tendency set in.
Visual hallucinations, fabulation, tremors, “excited-looking” eyes, are
described. He would sweep things from the dining-room table, pulled a
hot stove into the middle of the floor, attempted to sweep paint off the
floor, and cut up a carpet with a knife.
The patient on commitment November 5, 1904, was well developed and
nourished. The mucous membranes were rather pale. Bruises and
excoriations of limbs. Harsh breathing at the base of each lung.
Enlargement of heart; sounds irregular. Accentuation of aortic second
sound; tension fair, rate 80. Slight brachial arteriosclerosis. Abdomen
slightly distended. The urine contained a faint trace of albumin and
many hyaline casts.
Moderate tremor of extended hands. Slight tongue tremor. Romberg’s sign
absent (slight swaying). Considerable ataxia of extremities (inability
to stand with foot on opposite knee). Vision poor. Hearing could not be
tested accurately. Prompt pupil reactions with direct light. Slight
consensual reaction in left pupil, absent in right. Deep reflexes equal
and lively.
Quiet and orderly at first. Later restless and noisy. Questions were
answered at times relevantly, more often irrelevantly. Patient
irritable, intractable. Required repeated urging to take nourishment.
Consciousness clouded. Orientation imperfect. Attendants are possibly
“officers.” Date September, 1995. Slight errors in repeating alphabet.
Mistakes in Lord’s Prayer with rhyming tendency. Simple arithmetical
tests answered automatically with many mistakes. More complex
combinations incorrect. Handwriting tremulous (noted as “typical of
general paresis”). Auditory hallucinations (answering invisible
persons), “All right, I’m coming.” Amnesia and confabulation. Q. “Have
you had breakfast?” A. “No,” (later) “Yes, I had a very light
breakfast.” Q. “What did you have?” A. “Anything that came along. A few
green peas and beans that were left, bread and butter and pie. I had a
good breakfast. Guess feed is very high.” Q. “Give names of your sisters
and brothers.” A. “There are three or four I never see. I will have to
think them up.” (Later)—“Lillie, Abbie, Julia, George.” On repetition of
question, “Elizabeth, Julia, Annie and Lizzie.”
Delusions somewhat doubtful. At no time euphoria.
The patient remained only nine days in the hospital, developing diarrhea
a week after admission.
=Post Mortem Findings.= The =cause of death= was bilateral
bronchopneumonia of hypostatic distribution, accompanied by bronchitis
and acute splenitis. The intestinal tract was normal (despite the
diarrhea). No cultures. The heart showed acute myocarditis.
The vessels in general showed no sclerosis, except that the aorta showed
a few patches with calcification near bifurcation. There was a moderate
degree of mitral sclerosis. The kidneys showed a moderate degree of
chronic interstitial nephritis. The heart weighed 530 grams and there
was moderate dilatation of all the valves.
There were some evidences of chronic disease outside the nervous system,
namely, an obliterative pleuritis on the right side, chronic
perisplenitis, and chronic external adhesive pachymeningitis.
The =nervous system= showed a pia mater thin and transparent, with a
moderate congestion of larger and smaller vessels. No noteworthy change
of the brain substance or of the ventricles was found, except that the
cerebral substance was of unusual firmness (autopsy twelve hours after
death).
It is clear that the brain was not wholly normal, exhibiting a general
induration due in part to subpial gliosis and in part doubtless to
perivascular gliosis. =Microscopically= the tissues showed features of
great interest, especially multiple focal neuroglia cell proliferations
of a perivascular distribution, considerable subpial fibrillar gliosis
of an unusually focal type, and a rather general subpial cellular
=gliosis=. Histologically, it seemed that this chronic progressive
process had started, not so much in relation with dying nerve cells, as
in relation with blood vessels. The =perivascular= deposits of neuroglia
cells were confined almost exclusively to the infragranular cortex
layers. It seems plain that the diagnosis of general paresis was not
justified. It is probable that the diagnosis of neurosyphilis is not
justified. The explanation may be that now and then cases of cerebral
sclerosis may clinically imitate the neurosyphilitic process. It must be
borne in mind that the diagnosis in this case was made, like the other
cases at head of Part III, without the advantage of modern systematic
methods. Clinically speaking, of course, there was no definite
Argyll-Robertson pupil, although the consensual reaction, slight on the
left side, was absent in the right pupil. The general picture appeared
to be one of the so-called demented form of paretic neurosyphilis.
=Differential diagnosis between NEUROSYPHILIS and NEURASTHENIA.=
=Case 45.= Albert Robinson, a man of 28 years, was shipwrecked on one of
the Great Lakes. The ship was on the rocks for eight days, and Robinson
was under a great strain. Ever after the wreck, Robinson had felt severe
pain in the head, neck, and back, and a feeling of great weakness
whenever he exerted himself physically or mentally, and seven months
after the wreck, he had several attacks of fainting.
For a number of weeks he had worried a good deal about his inability to
make money, especially as money was badly needed on account of his
wife’s approaching confinement. A few days before entrance, Robinson had
become very forgetful, and was unable to recall, the night before
entrance, where he had been during the day. On the whole, however, on
mental examination no actual evidence of memory defect could be shown to
exist.
=Physically=, Robinson was entirely negative, except for some hard
glands in each groin. =Mentally=, there was little to show except
depression, worry over his financial condition, and his inability to
work. The serum W. R. proved negative.
=Diagnosis=: On the whole, the diagnosis of psychoneurosis (see case
Harrison (9)) due to the shock at the time of the shipwreck seemed to be
proper. To be sure, the patient gave a history of a chancre at 25,
treated for two years, after which he was declared cured.
However, following up the clue of admitted syphilis, rigorous
questioning elicited the fact that a few months before there had been
diplopia, lasting part of a day.
=Lumbar puncture= seemed desirable. The fluid was clear but contained
125 cells per cmm. with appropriately increased amounts of albumin and
globulin. The spinal fluid W. R. was positive. The diagnosis of
CEREBROSPINAL SYPHILIS seemed established.
The lesson of this case appears to be that perhaps we should never
exclude syphilis until we have made an examination of the cerebrospinal
fluid. The W. R. of the blood in meningovascular (non-paretic syphilis)
is negative in many cases (the figure is sometimes set as high as 40%).
=Treatment=: After a half dozen injections of salvarsan, all symptoms
disappeared and Robinson went back to work, claiming to be in a better
condition than for some time past.
1. How shall we explain such a symptom as the transient diplopia?
This diplopia is probably an example of a neurorecidive, but it
will be observed that it occurred without salvarsan therapy. See
discussion above under the case of Bennett (34), where the general
result of the neurorecidive inquiry launched by Ehrlich early in
the history of salvarsan therapy showed that precisely similar
phenomena had always occurred in neurosyphilis, whether under
treatment or not. The anatomical and histopathological explanation
of such phenomena is, of course, doubtful, but a review of the
findings in the case of Alice Morton (1) will show how many
apparently serious symptoms in neurosyphilitics are actually
irritative or at least due to lesions which are entirely
recoverable. We may suppose, first, a local proliferation of
spirochetes; second, a local over-formation of toxic substances,
directly or indirectly the product of spirochetosis; thirdly, a
local exudation; fourthly, a local proliferation; fifthly, a
combination of these phenomena, any or all of which may be
regarded as but transient. We have sometimes found at autopsy very
little exudate except in small areas; sometimes not more than a
few mm. or cm. in superficial extent. Note, for example, the small
areas of lymphocytosis demonstrable in but two foci in the case of
Alice Morton, the paradigm placed at the beginning of this book.
=NEUROSYPHILIS(?) in the SECONDARY STAGE of syphilis. HYSTERICAL
symptoms. Diagnosis?=
=Case 46.= Alice Caperson was a colored girl of 18 years. She had
acquired syphilis five months before admission to the hospital, and the
secondary symptoms of this syphilis had just disappeared before
admission.
Very shortly after acquiring syphilis, the young negress began to act
peculiarly. She describes herself as having a sort of nightmare, both
when asleep and also when awake. For instance, she saw her dead
grandmother. It appeared at first like a seraph; then it came nearer to
her and seemed to fill out; and then was dressed precisely as her
grandmother had been. This seraph appeared as though trying to tell her
something, but she could not make out what the something was. The vision
had appeared on two or three occasions.
Our examination detected little beyond instability and irritability of
mood with some depression. The patient readily fell to weeping. She soon
made friends in the wards, however, and got on well. =Physical
examination= was entirely negative but the W. R. of the blood serum was
positive. The W. R. of the spinal fluid was negative, as was the gold
sol reaction; there was an excess of albumin and a positive globulin
test; there were seven cells per cmm.
The psychiatric diagnosis of a case like that of Alice Caperson would
waver between hysteria and dementia praecox. However, as for dementia
praecox there are hardly any typical symptoms. There is insight into the
hallucinations, which are hypnagogic. There are, however, no hysterical
stigmata.
The spinal fluid reaction is typical of the secondary stage of syphilis.
It is commonly said that in every case of syphilis the nervous system is
involved at some period, if only to the degree shown in the present
case. However, such involvement tends to disappear both with and without
antisyphilitic treatment, just as do the secondary skin symptoms. So far
as syphilis is concerned, the prognosis under radical treatment is as
good as usual. We are inclined to regard the case as one of the
HYSTERICAL or PSYCHOPATHIC group and inasmuch as cases occurring in the
developmental stage of a patient’s life are of fairly good general
prognosis, we are inclined to regard the prognosis in this particular
case as good under proper therapy and hygiene.
1. What is the relation of neuroses to syphilis? Neurasthenia,
chorea, hysteria, and epilepsy are often grouped (for example, by
Nonne) as neuroses bearing at times important relations to
neurosyphilis. (For the relations of neurasthenia, chorea, and
epilepsy, see cases of Greeley Harrison (9), Margaret Green (72),
and David Borofski (49), respectively.) As for the hysteria shown
in Caperson, Charcot enumerated syphilis among _agents
provocateurs_ of hysteria along with alcohol, lead, arsenic, and
the like. Fournier has also considered the problem. It is clearly
necessary to show that before infection there were no hysterical
symptoms, and that the hysteria developed during the operation of
the syphilitic process, and it is probably necessary to show that
the symptoms will clear up under antisyphilitic treatment, if we
are to concede the existence of a syphilitic hysteria.
2. What are the evidences of neurosyphilis in the secondary and
primary stages of syphilis? As above stated, the findings in
Caperson are typical enough. Wile and Stokes at first stated that
60 to 70% of the secondary syphilitics show changes in the spinal
fluid; in a further article they maintain that probably every case
shows such changes and that clinical symptoms of neurosyphilis of
the secondary period can probably be determined. They claim that
it is probable also that the same holds for primary syphilis
itself. The importance of these claims lodges partly in the
relation of these early signs of neurosyphilis to the whole
question of latency and to the question of _paresis sine paresi_.
For a discussion of _paresis sine paresi_ see cases Lawlor (25),
Vogel (52).
=Differential diagnosis between NEUROSYPHILIS and MANIC-DEPRESSIVE
PSYCHOSIS.[9]=
=Case 47.= As in other instances (compare Martha Bartlett (21) and Annie
Monks (85)) so also in the case of Ethel Hunter, a woman 61 years of
age, there was no initial suspicion of neurosyphilis. Mrs. Hunter was
brought to the hospital stuporous as a result of an overdose of
paraldehyd. The paraldehyd had been administered by a physician to
combat insomnia and agitation. As soon as Mrs. H. had recovered from the
drug stupor, this agitation appeared once more, and it was clear that
she was suffering from marked depression. There was tremendous worry
over the sickness of a woman with whom the patient lived. The patient
was very self-accusatory, blaming herself for many things that had
happened in the household. Besides her agitation, depression,
self-accusations, and insomnia, the patient showed a good deal of the
symptom frequently termed “retardation”—a kind of lagging of all mental
processes found, according to Kraepelin, in manic-depressive psychosis.
Accordingly, the diagnosis of manic-depressive psychosis might well have
been rendered. The fact that the psychosis so far as known began in the
involution period was not against the diagnosis since the so-called
involution-melancholia of this period is at least in a certain fraction
of cases nothing more or less than a form of manic-depressive psychosis.
However, the =physical examination= made the diagnosis of
manic-depressive psychosis a little doubtful. There was a superficial
thickening of the arteries (blood pressure: systolic, 170; diastolic,
104), which thickening would not in itself be against the diagnosis of
manic-depressive psychosis. (In point of fact, arteriosclerosis is
rather common late in this disease and previous attacks could not be
excluded on the basis of available history.) The contracted pupils were
irregular and both reacted sluggishly to light, although better to
accommodation; the right pupil was larger than the left. The arm
reflexes were pretty active. The left knee-jerk could not be obtained,
nor was the right knee-jerk more than very sluggish. The Achilles
reflexes could not be obtained. Although there was not a positive
Romberg sign, there was a considerable swaying in Romberg position.
There was no speech defect. The other reflexes showed nothing abnormal.
On the whole, we had to conclude that, although Mrs. Hunter might be an
instance of manic-depressive psychosis, still there was much of
neurological interest in the case.
This conclusion was emphasized when the W. R. of the blood serum was
found to be positive. The spinal fluid W. R. was also positive, and the
gold sol index was of the “paretic” type. There were 74 cells to the
cmm. Globulin stood at ++++, and albumin at ++++.
This case, therefore, again illustrates, as well the protean nature of
GENERAL PARESIS (the diagnosis rendered), as the doubtful value of
making a psychiatric diagnosis without due consideration of the physical
examination and laboratory findings. How easy might it have been, at
least some years ago, to consider that this patient of 61 years had
suffered a slight shock at some previous time (left knee-jerk absent),
but was as a matter of fact a case of manic-depressive psychosis with a
vascular complication!
Note: We must again duly insist that the merely sluggish light reactions
of the pupils in such a case as this do not especially point to general
paresis. The literature seems to establish that sluggishness of light
reaction precedes the classical Argyll-Robertson pupil. Yet it does not
do to say that, if the Argyll-Robertson pupil pretty conclusively points
to neurosyphilis (for exceptions see cases Falvey (55), Murphy (60)),
then a sluggish pupillary reaction to light looks in the same direction.
Sluggishness may precede stiffness in many, or perhaps all, cases, but
sluggishness of pupils is a frequent phenomenon outside the syphilitic
group of cases.
1. What part is played by emotional shock and psychic causes in the
starting up of general paresis? The answer to this question cannot
be definite. That a paretic process can be started up after trauma
is admitted on all sides; but we here suppose actual physical or
chemical brain disturbance permitting increased spirochetosis or
inflammatory reaction. In the case of psychic shock, or what might
be called _psychogenic general paresis_, our best resort will be
to the indirect effects of hormone action, or of vasomotor and
other autonomic disturbances produced directly or indirectly by
emotion. We are clearly here dealing with material too speculative
to be of practical service at this time.
2. Was the depressive drug therapy in the case of Hunter
justifiable? The paraldehyd had been administered by a physician
apparently on purely symptomatic grounds to combat the insomnia
and agitation of this woman of 61 years. With all due
acknowledgment of the difficulties of private practice, we must
insist that when ordinary measures in the relief of insomnia and
agitation are insufficient to curb these conditions, then a
positive danger ensues with the larger doses. As a rule, with
these larger doses and with the withdrawal of sensory stimulation,
the patients relapse into a stupor of grave moment. We need only
recall the situation in delirium tremens where adequately
depressive drugs often tend to kill the patient.
=Case for diagnosis. Errors in the diagnosis of NEUROSYPHILIS are
possible even when abundant clinical and laboratory data are
available.=
=Case 48.= The first error chosen for demonstration is that in the case
of the machinist, Milton Safsky.
Safsky, about 8 months before his entrance to the hospital in the 42d
year of his life, had begun to lose strength, to grow thin and pale, and
to suffer from an extreme and continuous thirst. He was said to have
drunk as much as 6½ gal. in a day, and passed appropriately large
quantities of urine. After a time, his management at a general hospital
became difficult, as Safsky became confused, cried “hysterically,” and
was at times very noisy. He sustained a marked memory loss, seemed to
show visual hallucinations, and complained of headache, both frontal and
occipital, and of pain about the eyes. Sometimes the patient was very
euphoric and expressed what seemed to be delusions of grandeur, saying
he was wealthy and owned many machine shops.
Some symptoms, e.g., polydipsia and polyuria amounting to a diabetes
insipidus, associated with headache and arrested attention, suggested
possibly a new growth in the pituitary region. The mental symptoms might
naturally be supposed to be due to some infiltration or pressure effect
of intracranial growth. After admission to the Psychopathic Hospital,
the patient was found difficult to arouse, although he could eventually
be aroused. His orientation proved to be as poor as his memory. From
time to time, the patient became a bit more intelligent and able to
execute requests.
The =physical examination= was in general almost entirely negative.
=Neurologically=, the pupils were markedly contracted and reacted slowly
to light, though they were otherwise normal. The deep reflexes were all
somewhat lively, though equal. The umbilical and cremasteric reflexes in
particular were present. Systematic examination revealed no other reflex
disorder, nor any disturbance of sensation. There was a coarse tremor of
the extended hands. There were no phenomena of importance in the visual
fields.
As against the diagnosis of growth, pituitary or extrapituitary
(diabetes insipidus and headache), a hypothesis of neurosyphilis had to
be considered. Not only were the contracted, slowly-reacting pupils and
the active deep reflexes suggestive, but the euphoria with grandiose
ideas looked entirely consistent. As for the polyuria, one had to think
of the so-called syphilitic polyuria of the textbooks, which is regarded
as a more or less characteristic result of syphilitic involvement of the
_basis cerebri_. Moreover, the W. R. in the spinal fluid proved to be
slightly positive; 146 cells per cmm. were found therein; there was a
large quantity of globulin, and a very marked increase in albumin. These
observations seemed to be exceedingly suggestive of a cerebral syphilis.
However, as the case progressed, the diagnostic situation changed. The
W. R. upon a second puncture fluid proved negative. After some weeks,
characteristic symptoms of intracranial pressure developed; the
diagnosis of BRAIN TUMOR had to be taken as established, and there is no
doubt of its correctness.
1. What is the explanation of the weakly positive W. R. in Safsky’s
spinal fluid? An explanation is not easy to find. Possibly we may
regard the reaction as an example of error in technique. It is
even possible that it may have been produced by exudative products
in the spinal fluid.
2. What precautions may be taken against an error in diagnosis such
as was first made through the positive spinal fluid Wassermann in
the case of Safsky? First, repetition of the W. R.; secondly, it
is very unusual to find a weakly positive W. R. in a case with
such marked excess of albumin and such very marked increase of
globulin as was shown by this case.
3. How can we explain the inflammatory products in the puncture
fluid? Superficial brain tumors are frequently associated with a
so-called _meningitis sympathica_. The products of such meningitis
are exhibited: _viz._, globulin, albumin, and pleocytosis, exactly
as shown in Safsky.
=Can PARETIC NEUROSYPHILIS (“general paresis”) appear clinically
EARLY (e.g., two years) after the initial syphilitic infection?=
=Case 49.= David Borofski, a street car conductor, 27 years of age,
suddenly had a convulsion while at work in his car. For four months
Borofski continued to have rather numerous convulsions, was finally
compelled to discontinue work, and resorted to the Psychopathic
Hospital. It appears from his own story that, about two years before, he
had had a chancre, for which he had been treated at a general hospital
syphilis clinic, and of which he was told he was cured. With a
progressive loss of memory and with convulsions, Borofski became much
concerned about himself, and was finally persuaded by his fellow-workers
to come to the Psychopathic Hospital.
The convulsions were described as follows: The patient gives a short
cry, has convulsive movements for about ten minutes, remains unconscious
for perhaps half an hour, and wakes with headache, dizziness, and a
feverish appearance. Sometimes the attacks were more severe, with
frothing at the mouth, biting of lips, and loss of sphincter control.
There were also slight attacks, occurring almost every day, without loss
of consciousness; these latter attacks consisted of dizziness, inability
to speak for a few seconds, and some arm twitching.
=Physically=, Borofski was well developed and nourished, with a blood
pressure of 160. The only abnormal phenomena =neurologically= were
absent knee-jerks and ankle-jerks, sluggish pupillary reactions, and
slight tremor of the hands.
=Mentally=, despite suggestive complaint of amnesia, the memory was
found to be fairly good but knowledge of current events and school
knowledge was poor. The simplest problems in arithmetic Borofski gave
up.
The first diagnosis in such a case would naturally be epilepsy. However,
when an epileptic or epileptiform attack occurs for the first time in
adult life, the chances are probably against an idiopathic epilepsy.
(This is not a universal rule but will serve.) Borofski himself,
moreover, gave a history of syphilis. And the very nature of the
attacks, with arm twitching and without loss of consciousness, would not
readily fit into the frame of the idiopathic group. The absence of
certain reflexes and the sluggish pupils are naturally also suggestive
of syphilis, although not convincing.
The W. R. of the serum proved positive, as did that of the spinal fluid.
The gold sol reaction was characteristically “paretic”; there was an
excess of albumin and a positive globulin, and there were 15 cells per
cmm. There could be little or no doubt of the diagnosis of some form of
neurosyphilis. The laboratory picture was consistent either with general
paresis or with cerebrospinal syphilis. So far as we are aware in the
present stage of knowledge, the two conditions can hardly be
differentiated unless we choose to rely on therapeutics. However, it is
exceedingly rare for general paresis to occur only two years after the
original infection. If we can trust this statistical fact, we shall
perhaps be wiser to term the case of Borofski one of DIFFUSE
CEREBROSPINAL SYPHILIS, and not one of paresis.
=Treatment=: Borofski was put on antisyphilitic treatment consisting of
0.6 gram of salvarsan twice a week and potassium iodid, together with
intramuscular injections of mercury salicylate. The convulsions then
ceased. After four months Borofski returned to work, and he has remained
at work for a year. He has never regained his former health.
Fifteen months after beginning of treatment the laboratory tests were
again made (there had been more than 60 injections of salvarsan), and
the cell count and gold sol reactions were found to be negative.
Globulin and albumin were also in smaller amounts than in the original
examination. However, the W. R. of the serum and the spinal fluid
remained positive.
Head and Fearnsides state that cases of cerebrospinal syphilis should
return negative spinal fluid tests after six months of treatment. Upon
this criterion of Head and Fearnsides, Borofski would not be a case of
cerebrospinal syphilis; but it is probably impossible to separate
various forms of neurosyphilis into categories on any such grounds.
1. Shall case David Borofski be regarded as one of paretic
neurosyphilis (“general paresis”)? He has returned to work and has
remained at work, though without regaining his former health. In
any event, however, he does not offer the typical picture of
inevitable decline and death presented by the typical case of
Pietro Martiro (15) presented in our discussion of systematic
diagnosis. However, we could not upon laboratory grounds, or even
upon the ground of clinical observation, distinguish Borofski from
Martiro; Borofski has greatly improved; Martiro is dead. Borofski
developed his obvious neurosyphilis only two years after the
original infection. The conservative syphilographer might,
accordingly, reply that David Borofski is not a typical case of
paretic neurosyphilis (“general paresis”) either in the length of
the incubation period for his neurosyphilitic symptoms, or in his
outcome.
2. What is the cause of such convulsions as those developed by David
Borofski? Evidence from clear cases of general paresis with
convulsions leads to the hypothesis that such convulsions as those
developed by Borofski are not necessarily based upon frank
destructive lesions such as would be produced by the plugging of
terminal arteries. They may well be produced through the
activities of minor lesions, only demonstrable by microscopic
methods, either through properly disposed cell losses or by the
pressure of exudate, or even by endotoxins or other substances
derived from the bodies of dead or living spirochetes.
3. Aside from the well-known syphilitic epilepsy due to meningitis,
is there a non-meningitic epilepsy (such a disease as Fournier
formerly described under the term parasyphilitic epilepsy)? We
dismiss from discussion the so-called symptomatic epilepsies which
are the result of a gross organic disease of the brain substance
or its membranes, and which do not differ so far as we are aware
from organic epilepsy produced by other gross lesions of an
identical size and structure. These symptomatic epilepsies may be
partial, or even may present the appearance of generalized
epilepsy. We may also leave out of account those epileptic
pictures which are produced in general paresis itself, and which
may be viewed as nothing but partial phenomena of general paresis.
The kind of so-called “parasyphilitic” epilepsy that Fournier
described is a kind of epilepsy that cannot be distinguished from
genuine epilepsy, in which the sole disease-phenomenon throughout
a long period of time consists of epileptic convulsions. It
appears that these “parasyphilitic” imitations of genuine epilepsy
occur in individuals with a very long post-infective “incubation
period,” but that there are some cases in which the epilepsy
appears, on the contrary, in the very earliest stages of syphilis.
The attacks are a little less common than those of idiopathic
epilepsy; they have the same apparently causeless beginning; are
associated with complete amnesia; and are followed by
characteristic dazed states. The patient’s intelligence, however,
suffers little. Now and then a case reacts well to antisyphilitic
treatment energetically pushed. (Spontaneous long remissions in
non-syphilitic epilepsy must be remembered.) Petit mal attacks
occur sometimes between the more severe attacks. In short, it
would appear that there is a group of syphilitic epilepsies in
which the brain shows no gross structural lesions, which
accordingly do not exhibit any Jacksonian appearances, and which
last a comparatively long time without changing their character,
and often without being especially altered for the better by any
form of antisyphilitic treatment. This condition is sometimes
known as a post-syphilitic epileptic neurosis. Nonne had been able
to collect up to 1902 some 12 cases from his own service.
4. Would it be proper to call Borofski a case of taboparesis? Absent
knee-jerks in a victim of paretic neurosyphilis should not be used
to suggest a diagnosis of taboparesis. This question of
terminology has been discussed above, under Sullivan (16).
5. What is the mechanism by which the amnesia of a case like
Borofski is produced? The answer runs in the same terms as the
answer to the questions concerning the cause of convulsions. The
amnesia in general paresis has surprising functionality. A study
of autopsied cases of general paresis has shown that amnesia is
practically as common in cases without marked destruction of brain
tissue as in cases with atrophy of classical extent and depth. The
clinical recovery in this case was practically complete in respect
to memory. We must regard the amnesia as not due to the
destruction of storage cells bearing the so-called neurograms
(Morton Prince).
6. What is the explanation of the persistently positive W. R.’s of
the serum and spinal fluid associated with diminished globulin and
albumin tests, a negative gold sol reaction, and normal cell
count? See discussion under Case Martha Bartlett (21).
7. How atypical is the early development of paretic symptoms in
David Borofski? C. B. Craig has collected, in 100 cases of brain
syphilis (a list including both paretic and non-paretic cases),
some data on this point. The shortest period reported by Craig was
in a case in which the neurosyphilitic symptoms appeared one month
after infection. Craig found three cases where symptoms appeared
in six months, and six cases within a year. The longest
post-infective period of Craig’s list was thirty years. Our case
of Chatterton (73) developed symptoms 33 years after infection and
Washington (66), forty years after infection. Nonne casts some
doubt on statements to the effect that tabetic symptoms may occur
three to four months after infection. It seems to be admitted that
pupillary anomalies and reflex changes may occur in the early
secondaries and may recover under antisyphilitic treatment.
Nonne’s case of longest post-infective interval, like that of
Craig, was one of 30 years.
Myerson has reported a 20–year old patient who acquired chancre
April 1, 1911 (spirochetes demonstrated); salvarsan was
administered April 20th. There were no secondary symptoms, but in
May, headache, visual disturbance, vertigo, and other symptoms
developed (neurorecidive). Upon June 20th, that is, 11 weeks after
development of the chancre, aphasia and astasia developed, with
numbness of the left side. At this time, the pupils were slightly
irregular and unequal but reacted normally. The signs in the fluid
were positive. Upon this question see our cases of Bright (121)
and Bennett (34).
=Hemitremor following hemiplegia in PARETIC NEUROSYPHILIS (“general
paresis”). Autopsy.=
=Case 50.= Achilles Akropovlos, 39 years, had symptoms six months before
commitment to Danvers Hospital. There were attacks of confusion,
difficulty in walking, and speech defect, resulting in an entire
incapacity to work and eventual commitment. Rather unusual and striking
was a very marked tremor, apparently limited to the right side of the
body. =Physically=, Akropovlos was normal, but =neurologically= he
showed, in addition to the marked right-sided tremor, a marked speech
defect, and a degree of ataxia. The tendon reflexes were very active,
but there were no abnormal reflexes, and the pupils reacted normally.
According to the history, the difficulty had followed a slight attack of
apoplexy. =Mentally=, there was a marked confusion. The blood serum and
the spinal fluid were both positive to the W. R.; globulin was present,
and albumin was increased; there were 43 cells per cmm. There was hardly
any diagnosis to make except general paresis.
Death followed 18 months later, or two years after onset of symptoms.
Increasing weakness, emaciation, and dementia preceded death. Autopsy
confirmed the diagnosis of PARETIC NEUROSYPHILIS.
1. What is the usual cause of death in general paresis? Intercurrent
disease very frequently occurs in general paresis, and such
intercurrent disease is then given as the cause of death. As a
matter of fact, however, one feels that in many of these cases the
intercurrent pneumonia or infection—frequently of the
bladder,—bedsores, sepsis, and the like, are merely accidental
incidents in a condition that is leading to death, and which has
caused a lowered resistance to infection. In certain instances
where nursing is exceptionally good and where no such infection
occurs, the patient continues to grow weaker and weaker, paralyses
of all the muscles follow and finally paralysis of deglutition or
respiration may lead to death. The emaciation and paralyses may be
of such a grade that the patient is entirely devoid of fat and
unable to move at all. Not infrequently vascular crises occur, and
one of these may be responsible for death.
2. What was the cause of the hemitremor? The hemitremor suggested an
irritative or destructive lesion in the motor path. Delving into
the history it was learned that the patient had had a shock
followed by a right hemiparesis. This had cleared up leaving the
tremor as a residuum. The autopsy disclosed a reddish-brown
pigmentation and fibrous thickening of the pia over the left motor
area, confirming the idea of a previous hemorrhage. As a rule the
shock phenomena occurring in paresis clear up more completely and
no gross lesion is visible post mortem. However, cerebral
hemorrhage must be expected in any person suffering from syphilis,
and is no rarity in paretic neurosyphilis.
=PARETIC NEUROSYPHILIS (“general paresis”) with NORMALLY REACTING
PUPILS. History of trauma. Autopsy.=
=Case 51.= Daniel Wheelwright, a barber of English extraction, 57 years
of age, had had a sunstroke at 15. At 42, there had been pneumonia,
after which an attack of rheumatism was said to have kept the patient
from work for a year. There was trauma of head (falling wrench) at 44.
This blow on the head was the assigned cause of the mental disease,
symptoms of which, however, did not develop until about the first of
September, 1905, about three months before entrance, January 9, 1906,
and about six months before death, March 20, 1906.
It seems that the patient had begun to change in manner; he had become
despondent and apathetic, silent, and somnolent. Two weeks later, he
stopped working, began to read the papers once more, and became somewhat
more cheerful.
About Thanksgiving, Wheelwright got up at midnight, and remained up,
lighting all the fires and talking continuously. During the next two
weeks, he talked much to himself, laughing out at times. About two weeks
before Christmas he went out and started to make a sidewalk of old
boards, working in his shirtsleeves, without a hat. He would work until
midnight making screens for windows. During the day, he would go out and
give money to passing children; would offer to pay the grocer twice as
much as articles were worth.
On the day before Christmas, he put out all the fires and lights in the
house, sent all the family to bed, and opened all the doors. Christmas
morning, he rose early and got the washtubs ready. He helped his
compliant wife to do the washing, then put out all the fires and opened
the windows. After Christmas, he began to tell how rich he was going to
be through starting a garden and by making butter. He bought six or
seven quarts of milk daily, and procured carrots and oranges, grinding
them up to color the milk. January 9th he was committed to Danvers
Hospital.
=Physically=, there were few symptoms. =Neurologically=, there was a
tremor of tongue, fingers, and face. The knee-jerks were lively. The
pupils reacted normally; the patient was restless, pacing up and down.
There was a speech defect demonstrable with test phrases. Orientation
was imperfect for time and for place. Handwriting was poor, memory
impairment was marked, but the patient was given to fabrication as to
past events. A characteristic sample of statements:
“Do you know that this is an insane hospital?” “Yes; there are two or
three men here out of their heads. I could cure them with my hands but
they won’t let me. I could get all the sick men on their feet just by
rubbing them. I can do anything with my hands. I can build a house by
just sitting down and thinking about it. I can whip all the men in this
place. I have better sense now than I ever had in my life.”
Again, “How long have you been here?” “Over three months; they have put
me in heaven three times since I have been here. They killed me, crushed
my heart, and turned my blood to water. I am all right now. I let the
sun shine on my heart and it brought it together. I can whip every man
in here as fast as they come up.”
Again, “I will make a million dollars on my garden when I get it. I can
make a million dollars on half an acre. I can do anything. I can move
this house by just thinking of it.”
During a special examination, the patient told how he had fastened wings
on his hands and feet, and how he had gone to heaven; he told how he had
soared high above the earth, and how differently the stars look when up
near heaven than they do from the earth. He spoke of seeing angels and
of the beauties of heaven.
The diagnosis of PARETIC NEUROSYPHILIS was confirmed at autopsy.
1. What is the significance of the normally reacting pupils? While
it is usual to find pupillary anomalies in neurosyphilis, these
changes are not an essential part and it is not rare to find
normal pupils in all forms of neurosyphilis. It is less frequent
to find a normal pupil in tabetic than in diffuse or paretic
neurosyphilis. In paretic neurosyphilis it is the rule to find
pupillary changes during some stage of the disease, but not
necessarily early. At times the pupillary sign may be one of the
earliest signs of neurosyphilis—again it may occur only as a late
symptom, if at all. One of the most important of the pupillary
signs is irregularity of contour. While this does not always mean
neurosyphilis it is highly suggestive and certainly indicates
careful examination even though the W. R. in the blood be
negative.
2. What was the relation of trauma to the development of the
neurosyphilitic symptoms? It is, of course, the rule in all forms
of mental disease to have some factor offered by the patient or
relatives as the cause of the psychosis. Often these assigned
causes are minor events thought of only after the later appearance
of symptoms. In this case it was not thought that the trauma had
any causal effect. For a discussion of trauma and neurosyphilis
see cases Joseph O’Hearn (90), Levi Sussman (91), and Joseph
Larkin (92).
=NEUROSYPHILIS, probably PARETIC, with symptoms highly suggestive of
MANIC-DEPRESSIVE PSYCHOSIS.=
=Case 52.= Bessie Vogel[10] was admitted to the Psychopathic hospital
New Year’s day, 1915, in a very much excited condition. The family
history is very meagre, and all that is of significance is that mother
has always been very “nervous.” The records in part:
=Past History.= Very healthy as a child, and except for occasional
throat trouble and headache had no physical ailments until eight years
ago, when she had an operation for appendicitis, and two and one-half
years ago was operated upon for hernia and adhesions. Following this she
began to show a lack of energy, neglected her housework, was much
depressed, wept frequently, complained constantly of pain in various
places, and was ill-tempered. In about five months she improved, and
then after a couple of weeks at the shore seemed entirely well.
=Present Illness.= In November, 1914, that is, about seventeen months
after the recovery from the previous depression, she again began to show
practically the same symptoms. She was depressed, could not sleep, and
would get up in the night and sew; was self-centered and hypersensitive,
then became restless and nervous; wanted to go shopping and out for
dinner; went to New York and then to New Bedford. Symptoms became more
marked; she became very ill-tempered, threatened her husband when angry
over trifles, threatened suicide, then began to get active and spent
money extravagantly. At the end of two months, that is, Jan. 1, 1915,
she was admitted to the hospital.
=Physical Examination.= A small, thin woman, appearing to be about 45
years old (actual age 37). Aside from the absence of teeth and the
operation scars, the general examination is negative. =Neuromuscular
system=: The pupils are round, regular, equal, and react to light and
accommodation, but do not hold very well. Extraocular movements well
performed, no palsies of facial muscles, tongue protruded medially
without tremor. Uvula is raised symmetrically. Biceps and triceps and
supinator reflexes are present and brisk. Patellar and Achilles reflexes
are equal on the two sides and brisk. Abdominal skin reflexes not
obtained. Plantar reflex active and flexor in type. No Babinski, Gordon,
or Oppenheim. No tremors.
Wassermann reaction serum positive. Examination of spinal fluid: clear,
globulin ++++, albumin ++++; cells, 130 per cmm.; small lymphocytes,
79.9%; large lymphocytes, 14.1%; polymorphonuclear leucocytes, 4.6%;
plasma cells, 0.7%; endothelial cell, 0.7%. W. R. positive. Gold sol
reaction, 55555522 +-.
=Mental Examination.= On admission patient showed great psychomotor
activity, was very playful, marked flight of ideas, was expansive, very
emotional, very erotic. She slept very little, appetite was poor, and
she lost weight rapidly. Orientation and memory intact. No
hallucinations elicited. In about three weeks improvement began, and at
the end of eight weeks she appeared practically recovered. On April 9,
1915,—that is, 13 weeks after admission,—she was allowed home on visit.
On leaving, she appeared normal in every way. There was no evidence of
psychotic symptoms, she had good insight, and physically there was
absolutely nothing of a neurological nature that was abnormal.
This case, with the history of a previous depression and its clinical
picture during the acute stage, and its recovery, is certainly in every
respect typical of manic-depressive insanity, and only the positive
result of the six tests causes us to put it in the group of GENERAL
PARESIS. Only the further course will shed any light as to the correct
significance of these findings, and even then we shall not be too sure
that we had not been dealing with a manic-depressive psychosis in a
latent neurosyphilitic. We would strongly emphasize the point that at
the present time this patient presents no mental or physical signs of
cerebrospinal syphilis or general paresis; but the six tests are still
positive. This case differs from the ordinary general paresis remission
in that there is not a single physical sign of paresis present.
There are many transitional cases between this case which shows no
symptoms or signs of neurosyphilis except the laboratory tests, and the
typical case of general paresis. Thus we have cases with slight
character change and no physical signs except rare “seizures.” On the
other hand, in many cases the presence of abnormal neurological
phenomena without definite mental signs is first noted. Certain remitted
cases show only some slight pupillary or reflex abnormality. We believe
we have here added the last link in the chain between the primary and
quaternary symptoms.
This case is illustrative of several which we have published elsewhere
under the name of _paresis sine paresi_ or latent neurosyphilis to
illustrate how all the laboratory signs of neurosyphilis may be present
in a patient without any physical or mental symptoms that may be
correlated with these findings.
We summarize our discussion of this as follows:
1. There is a group of cases showing the laboratory signs characteristic
of central nervous system syphilis: (_a_) positive W. R. in the serum,
(_b_) positive W. R. in the spinal fluid, (_c_) pleocytosis, (_d_)
excess of albumin, and (_e_) of globulin in the spinal fluid, (_f_) gold
sol reaction of central nervous system syphilis, and which show no sign
or symptom of neurosyphilis.
2. We believe these cases represent a form of chronic cerebrospinal
syphilis, probably paretic in type.
3. They have the greatest theoretical and practical significance in the
consideration of the life history of neural syphilis, in the concept of
_Allergie_, in regard to results of treatment, and finally as to the
evaluation of the laboratory tests.
4. Here is perhaps offered the last link to form a complete chain
between the symptoms of the primary stage of syphilis and its final
termination of life as the result of the diseases cerebrospinal syphilis
or general paresis.
=SYPHILIS (?); EXOPHTHALMIC GOITRE; neurosyphilitic old lesion of
optic thalamus; unilateral induration and atrophy of left cerebral
cortex. Autopsy.=
=Case 53.= Carrie Pearson, a housewife 25 years of age, died at Danvers
Hospital less than a week after admission, and it was at first stated
that her symptoms had lasted but two weeks before admission. In point of
fact, a further investigation showed an important succession of
symptoms, lasting some four years.
Carrie had been considered a healthy child, going to school at the usual
age, and progressing well with her studies. She however, left school in
the ninth grammar grade, at the age of 15, and went to work in a
milltown. She married a worthless person at the age of 18, and lived
with her husband for three years. There was one child born a year after
marriage. Two years later, however, a tremendous goitre had developed
such that her neck was described as “out square with the face,” and at
the same time the patient’s eyes had become prominent.
About two weeks before admission, she had gone to a neighboring town to
take care of a sick woman, but during her endeavor to be a nurse, she
had broken out into a mania, tearing up furniture and bedding, and
talking irrelevantly for a period of four days. She also showed insomnia
and continually tore off her clothing from her body.
Upon =examination=, the marked enlargement of the thyroid gland together
with the prominent eyeballs, husky voice, and pulse rate of 150 per
minute, were entirely consistent with the diagnosis of exophthalmic
goitre. The patient described herself as “Carrie Nation.” Asked to write
her name, she took the pen and tried to spatter ink, wrote hurriedly and
carelessly her maiden name and several words without apparent meaning.
Asked to write, “God save the Commonwealth of Massachusetts,” she wrote:
“God save the common pal U S Spe Manor Gen, or til pat. Since Lord, or
no prime in Hear to the God Tel. Ho. n and or Mabel, or gal.” After
this, she took paper and wrote meaningless scrawls, saying that it was
Japanese writing. There was much motor restlessness with
distractibility, pointing and grimacing, mimicking the actions of those
about her.
Death occurred from exhaustion, and the case might not have been
regarded as unusual except for the autopsy, which showed a peculiar
brain lesion, described below. The point of greatest interest in the
case was the fact that syphilis is, although not proved to exist by
laboratory tests, beyond question a factor in the case. Although the
woman had given birth to a normal child, who is still alive, yet in the
period of a few years her breasts had atrophied, her hair had
disappeared from the axilla and from the pubes; varicose veins had
developed in both legs. Whereas there was little or no fat over the
chest or back, the omentum and mesentery were very plentifully supplied
with fat. It is probable, then, that we are dealing with a case of
exophthalmic goitre somehow of syphilitic origin. The brain lesion is
consistent with this hypothesis.
=Autopsy=, March 3, 1907. Four hours post mortem.
Body length, 165 cm. Body of a well developed and well nourished
young woman. Lividity in dependent parts. Purplish discoloration of
left thigh to knees. Skin rough and scaly. Petechial eruption over
chest. Neck thick, protrudes anteriorly. Varicose veins over upper
parts of calves on both legs. Eyes protruding, not covered entirely
by lids. Pupils equal, dilated. Subcutaneous fat very deep over
lower part of body. Very little fat over chest and back. Breasts are
very small, apparently atrophied. Normal amount of hair on head,
slight amount over pubes. Axillary hair absent. Fat on section of a
light yellow color. Omentum extends to pubes, plentifully supplied
with fat. Large amount of mesenteric fat. Appendix normal.
Intestines smooth and glistening. Slightly injected. No fluid in
peritoneum. Uterus small, retroverted.
[Illustration:
Cortical hemiatrophy—A, relatively normal right precentral (“motor”)
cortex; B, atrophic left precentral.
Note in B:
1. Absence of giant pyramids of Betz (corticospinal, upper motor
neurones).
2. Superficial (subpial) condensation of tissues with sclerosis
(gliosis). The tissues in all areas examined _on the left side_
yielded this effect.
]
HEAD: HAIR in good quantity. SCALP normal. CALVARIUM shows diploë.
DURA MATER over left cerebral hemisphere inseparably adherent to
calvarium, over right hemisphere normal. Arachnoidal VILLI
moderately developed. PIA MATER shows injected veins, notably in the
sulci of the right hemisphere. Pia mater everywhere thin and clear.
VESSELS at base of normal appearance.
BRAIN weight 1180 grams. Spread on a board, the right hemisphere
tends to flatten so that it measures 1.5 cm. more from side to side
than its fellow. Besides more marked venous injection, the right
hemisphere shows also flatter and slightly more plastic
convolutions. The posterior poles of the hemispheres are a little
firmer than the parts anterior. The orbital and hippocampal gyri on
the right side are a little firmer than the surrounding parts. On
section the gray and white matter shows no lesions, excepting the
slight plasticity of the tissues at large on the right side and a
well marked induration, with retraction under the knife, of the
occipital and hippocampal white matter. The basal ganglia of the
left side are normal. On the right side a sagittal section
demonstrates a rounded area of induration, with ill-defined borders,
measuring perhaps 1.5 cm. from above downwards by 2 × 2 cm.,
situated largely in the lenticular nucleus and involving the greater
portion of the globus pallidus, a small segment of the putamen below
and behind and the regionary part of the anterior commissure with
surrounding tissues. The most striking feature of this lesion is the
occurrence in the middle of a cluster of vacuoles or cystic clefts,
with smooth pale interiors, ranging from pinhead to 0.25 cm. or even
0.5 cm. in greatest diameters. There are six to eight clefts to a
surface of section. The color of the lesion differs little from that
of the globus pallidus itself, but the tissue is a trifle
translucent. It is impossible to demarcate the lesion with the eye.
Induration is demonstrable several mm. beyond the visible part of
the lesion. The consistence of the lesion slightly surpasses the
usual consistence of the olivary bodies.
CEREBELLUM, PONS and BULB weight 165 grams. Cerebellar tissue a
trifle more plastic than usual. The right olive is not so prominent
as usual.
=Note.= THYROID: Weight 125 grams. Both lobes and isthmus enlarged.
One lobe more than the other; lobe on one side measuring 6 × 4 cm.
Anatomical Diagnoses
Enlargement of thyroid gland.
Exophthalmos with dilated pupils.
Fatty degeneration of thoracic muscles.
Slight aortic sclerosis.
Dilatation of right heart.
Hypertrophy of left ventricle.
Slight tricuspid endocarditis.
Bicuspid aortic valve.
Hypostatic pneumonia.
Acute and chronic splenitis.
Fatty liver (central necroses?).
Acute nephritis.
Chronic gastritis.
Small breasts.
Axillary hair absent.
Petechial eruption of chest.
Varicose veins.
Chronic external adhesive pachymeningitis of left side.
Moderate swelling of right hemisphere with venous injection.
Slight occipital gliosis of both sides.
Slight gliosis of orbital and hippocampal gyri of right side.
Sclerosis with atrophy of occipital and hippocampal white matter of
right side.
Gliotic lesion (1.5 × 2 × 2 cm. of right lenticular nucleus
involving anterior commissure).
1. Was the exophthalmic goitre in Carrie Pearson due to syphilis?
Unfortunately we have no clear proof that Carrie Pearson was
syphilitic. She was stated to have been syphilitic by the
physician who treated her before her commitment to Danvers
Hospital. There is, however, no proof of syphilis, inasmuch as the
patient died in the pre-Wassermann period.
2. Is the thalamic lesion probably syphilitic? No lymphocytosis or
plasmocytosis characterizes the lesion, which is the only lesion
of the sort in the Danvers collection. It would not do to call a
lesion syphilitic just because it is _sui generis_. In any event,
the clinical analysis of the case faced the claim of syphilis as
an actual factor in the patient’s life and as a possible factor in
the goitre.
=It is well known that the ARGYLL-ROBERTSON PUPIL is characteristic
of the so-called “PARA-SYPHILITIC DISEASES” (“general paresis” and
“tabes”); does this sign occur in other neurosyphilitic conditions?=
=Case 54.= Julius Kantor was a shoemaker of 35 years, who came to the
hospital for treatment because his family physician had found a positive
W. R. in Kantor’s blood serum. He had had a cough for a number of years,
and during the last year a little blood had been found in the sputum;
whereupon Kantor had been placed under active anti-tuberculosis
treatment. The enterprising family physician had found the positive W.
R. in the first days of his treatment for tuberculosis. There was, in
fact, a history of a chancre nine years before, which had not been
followed by any secondary or tertiary symptoms, and which had been but
scantily treated.
There were no mental symptoms.
Kantor was =physically= fairly well developed and nourished. There were
a few piping râles in the left upper chest, both in front and back, and
also a slight dulness with increased vocal and tactile fremitus. No
tubercle bacilli, however, could be found on repeated sputum
examination.
=Neurologically=, the pupils were myotic and both showed the
Argyll-Robertson reaction. There were no abnormal reflexes whatever, and
there was neither ataxia nor speech defect. Not only the blood but also
the spinal fluid W. R. proved to be positive; there was a marked
increase in the albumin and globulin; there was a gold sol reaction of
the syphilitic type, and there were but three cells per cmm.