Part 12
As for the pains in the legs, they had been first noticed some three or four years ago and considered to be mild rheumatic effects. Now, however, they have grown progressively worse and have been the effective cause of giving up business. The pains are sharp, darting, pinching, and burning, and last, say, about a second with an interval of about the same length. The attack will continue sometimes for many hours.
There is a strabismus of the left eye, ascribed by the patient to an accident with an umbrella (there had been operation without relief). The pupils showed the Argyll-Robertson effect and were markedly irregular. Despite the divergent strabismus with diplopia, the eye movements were well performed although not in parallel axes. Ankle-jerks could not be obtained even on reinforcement, but the knee-jerks were lively, and the other deep and skin reflexes proved normal. The blood and spinal fluid tests were characteristic of tabes dorsalis.
It appears that the syphilis was acquired by this patient 15 years before; that is, 5 years before neurological symptoms began. Three courses of treatment had been taken at a well-known watering-place, and mercury pills had been taken for two years by mouth. The patient is married; has no children; there have been no pregnancies.
1. What causes may be assigned for the absence of children in the family of a tabetic? There may be lesions of the genital apparatus (orchitis, or more specialized toxic lesions). But impotence such as characterized the present case must also be taken into account.
2. What is the therapy for tabetic pains? Pyramidon is nowadays much in favor; morphine may be used; some authors recommend that the patients be instructed to chloroform or etherize themselves slightly for relief of the pain. Surgery of the nerve roots may be resorted to in extreme cases. Intraspinous therapy, suggested by various authors, seems to exert beneficial effect in many cases.
3. Is the lack of control of the vesical sphincter an unusual initial symptom? On the contrary, the more careful the clinical observation, according to some observers, the more likely is the examiner to find that vesical symptoms were the earliest or among the earliest complaints of the patient. Baldwin Lucke found sphincter disturbances to be initial in 8¼% of his long Blockley series. He found sphincter disturbance to occur in some stage of the disease in 67.6%, being exceeded in frequency only by staggering gait (87.2%) and lancinating pain (71.6%). According to Lucke, the most frequent _initial_ symptom is lancinating pain in the lower extremity, which, it will be noticed, occurred also in our case of Stephen Green as an initial symptom along with vesical disturbance. Lucke’s figures show that paresthesia of the lower extremities (17.6%) and weakness of the extremities (16.4%) are the next initial symptoms in frequency.
4. Could the early treatment in the case of Stephen Green be considered as adequate? No better answer can be given to this question than by quoting from Dr. Joseph Collins,[8] who probably has done more than any other one man in this country in insisting on the need of proper treatment of syphilis. As to the adequate treatment of syphilis he says:
“It consists in the proper use of salvarsan and mercury begun at the earliest possible moment after infection and kept up till all biochemical evidence of the disease has ceased, while the metabolism of the individual is maintained as nearly normal as possible. But the physician does not do his whole duty when he has accomplished this. He must solicitously watch the individual to see that no evidence reappears for months and even years after the apparent cure. As an index of such reappearance the Wassermann test of the blood serum and of the cerebrospinal fluid is the safest guide.
“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.