Part 13
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.
=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.
=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.