Chapter XIII: Introduction (5)

“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.

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.

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.