Part 31
=Case 121.= Arthur Bright, a printer, had acquired syphilis in his 49th year, some six months before examination. He had been treated during these six months by three injections of salvarsan, injections of mercury, and mercury by mouth. He had been apparently cured until about a month before admission. He had fallen without warning from his chair in a convulsion accompanied by unconsciousness, which lasted about two hours. The patient had since been feeling rather peculiar. For instance, time seemed to flow too rapidly. Sometimes the patient had had difficulty in talking.
=Physically=, nothing abnormal could be found either in general condition or =neurologically=. The patient was, however, incontinent. =Mentally=, he was apathetic and unalert, even paying no attention to his outside physician when he came to visit him.
The =diagnosis= of cerebrospinal syphilis already suggested by his history was confirmed by the laboratory tests, which showed a positive serum and spinal fluid W. R., paretic gold sol reaction, 41 cells per cmm., an excess of albumin, and a positive globulin test.
1. What is the prognosis in cerebrospinal syphilis in the early secondary stage? The prognosis appears very good provided that intensive treatment be given and provided that no vascular insult or other focal destructive lesion occurs before treatment has had time to do its work.
2. Why did not the “effective” (?) treatment for the syphilis, dating from the primary lesion, succeed in staving off the cerebrospinal syphilis? It remains a question whether the treatment by three injections of salvarsan was efficient in this particular case. Of course, it may prove true that no treatment whatever in the present stage of knowledge will stave off cerebrospinal symptoms in certain cases.
=Treatment=: Bright was given intravenous injections of diarsenol twice a week, with occasional injections of mercury salicylate. After two weeks, the patient seemed markedly improved, and continued to improve rapidly. He was symptomatically well at six weeks. The spinal fluid had then become negative, although the serum W. R. had remained positive.
After discharge from the hospital, Bright returned to his work, but continued to take the diarsenol treatment weekly, and two months later the serum W. R. became negative.
Small injections of diarsenol at intervals of a month were continued, and Bright remained perfectly well for four months, when a peculiar seizure developed and lasted for several hours. This seizure consisted in a sort of somnambulism in which Bright stood up at a table, making marks on paper, and could not be persuaded to desist. After this seizure, Bright re-entered the hospital, again showed no mental or physical symptoms and no abnormalities of blood or spinal fluid.
3. What is the explanation of this seizure? It is possibly due to a small vascular insult, for which potassium iodid may be suggested with precautions as to hygiene and continued observation. He has since remained entirely well.
=Another example where MILD MEASURES (though conceived to be “adequate”) SEEMED TO BE LEADING TO FAILURE; INTENSIVE THERAPY SUCCESSFUL.=
=Case 122.= Levi Morovitz, a waiter, 39 years of age, came to the hospital with evidences of an old left hemiplegia, including the left side of the face (there was a left-sided Babinski, Gordon, and Oppenheim, and all the reflexes were fairly active; sluggish pupil reactions, Rombergism, and speech defect). Morovitz was much depressed, very slow in thinking processes, had a marked memory disturbance in general and apparently much deterioration mentally.
A history was obtained to the effect that Morovitz had acquired syphilis at about 33, but that he had received practically continuous treatment ever since at a dispensary. He had, in fact, received four injections of salvarsan a year before coming to the hospital. Of late, Morovitz had become much more cheerful and talkative, imagining he could do great things if he had money. He had begun to eat very rapidly and to be very nervous. His feet had begun to drag; a distinct speech defect developed, but from this he had recovered. About six weeks before entrance, Morovitz had a shock, which left him with the left hemiplegia above mentioned and with considerable headache.
Even while the preliminary examination was being performed, Morovitz developed a minor seizure without loss of consciousness. First came severe pain over the frontal region, which grew in severity so that the patient held his head in his hands. A bit later, twitching movements began in the thumb and in the fingers of the left hand, and the small muscles of the extensor group of the thumb and third finger showed contractions. These contractions grew more general and the excursions of the fingers greater, until finally every finger of the left hand became involved, whereupon movements of the same sort, though of smaller amplitude, began in the other hand. Finally the left arm began to jerk with alternate contractions of the biceps and triceps. The whole seizure lasted more than five minutes. During the seizure there was dizziness and pain in the head, chiefly on the right side.
=Diagnosis=: The attention is at once arrested by the data of the seizures described. It appeared that we had to assume an irritation of the right side of the brain, possibly due to vascular disease, or to brain tumor, or perhaps to syphilis. The shock with residual hemiplegia would be consistent enough with any of these diagnoses. However, the history seemed somewhat long for brain tumor. Nor were there any definite symptoms of intracranial pressure. “Adequate” treatment unfortunately does not rule out syphilis. The comparatively early age (39) of the patient makes it difficult to explain the vascular disease except on the basis of syphilis. Add to the hemiplegia the euphoria and grandiose ideas of a year’s duration, and we arrive at a diagnosis of neurosyphilis, probably PARETIC NEUROSYPHILIS.
The laboratory tests showed the W. R. of the serum and spinal fluid positive, 80 cells per cmm. in the fluid, large amounts of globulin and albumin, and a “paretic” type of gold sol reaction.
To be sure the Jacksonian seizure is not especially characteristic of paretic neurosyphilis, and even suggests a local irritation in the motor area, such as a localized meningitis, possibly of a diffuse gummatous nature.
This patient was put on intensive antisyphilitic treatment, namely, salvarsan twice a week and injections of mercury. He recovered rapidly. After a few months he left the hospital, and after treatment had continued for a year, he resumed his work by which time both blood and spinal fluid had become negative.
It must be recalled that this patient had from the time of his infection what has been considered good antisyphilitic therapy, in spite of which he developed after a period of years, the symptoms and signs of neurosyphilis in its most dangerous form. The conclusion must be drawn that however good such treatment is for the majority of cases, it was insufficient for Morovitz. That the early failure to cure was not due to any “drug fastness” of the spirochete or to any peculiarity of strain is proved by the result of more vigorous antisyphilitic treatment which caused an apparent if not a real cure. With our modern methods of treatment checked by Wassermann reactions and spinal fluid examinations, treatment is given according to the _needs of the individual patient_ rather than according to general preconceptions. We have reason to believe that under these conditions there will be fewer cases developing late symptoms on account of insufficient treatment given even to patients who are willing to co-operate to the last degree.
The fact that Morovitz had no apparent symptoms for several years led to rather desultory treatment chiefly in the form of mercury by mouth. Previous to the time when the W. R. and lumbar puncture were available, the physician had no exact means of determining cure except the non-appearance of symptoms. But a period of years of quiescence before the outbreak of symptoms referable to the involvement of the nervous system is characteristic of syphilis. With this knowledge in mind it is evident that today the care of a syphilitic patient must be guided, in part at least, by examinations of the spinal fluid and W. R.
=Salvarsan treatment may even occasionally be of value in simple FEEBLEMINDEDNESS due to congenital syphilis.=
=Case 123.= The somewhat unattractive Robert Matthews was brought, at 5 years of age, to the hospital for backwardness of mind. It appears that the patient was born at term, with instruments, that he began to talk at a year, and to walk at 13 months, but that in point of fact, he had not talked intelligibly to date. Robert had never played with other children and is regarded by his parents as backward. In fact, Robert’s sister—a year his junior—is much brighter. Robert had had scarlet fever but without sequelae.
Examination by the Binet scale showed that, although he is actually 5½ years, he graded by the Binet scale at 4 and was regarded as feebleminded.
The =physical examination= showed a general adenopathy and prominent frontal bosses. In the study of the family history in the search for an etiology for the evident feeblemindedness, little or none could be found. There were no miscarriages or stillbirths; the parents were living and well. There was only the one sister above-mentioned, who is brighter than Robert.
The advantage of a routine W. R. is here well shown, for the W. R. in the serum was positive.
1. What is the prognosis of cases of syphilitic feeblemindedness? It would appear that every case is an individual problem.
2. What is the effect of treatment? Robert Matthews was given mercury protoiodid ⅛ gr., three times a day, by mouth, for three months. The protoiodid was followed by ten injections of salvarsan, average: 0.15 gram, during six months. At the end of this period, the W. R. in the blood had become negative. A re-examination by the Binet scale, when Robert was 6–5⁄12 years of age, showed him to grade at 5⅖, so that one might conclude that Robert had shown more mental progress in a year than he had previously.
Note: The patient’s sister, 4 years of age, is attractive and bright, measuring beyond her actual age according to the intelligence tests. However, the girl was found to have a positive W. R. It may be that Robert and his sister illustrate the hypothesis of Mott: that the syphilitic virus becomes less potent as the years go on, and that the younger children in the family are less affected than the older. However, in our series, there are a number of instances in which this hypothesis is not substantiated.
3. What is the share of syphilis in the production of feeblemindedness? The percentage of syphilitic cases found in institutions is not high. A variety of cases have been proved to be congenitally syphilitic in the absence of a positive serum W. R.
Fernald[19] has charted a comparison of cases diagnosticated “moron” (that is, feeblemindedness proper, in the narrower English sense) and “imbecile.” Fernald says that the morons have, as a group, many more bad family histories than have the imbeciles, to quote—“Only 70% of the [imbecile] group have bad family histories. This at first seems surprising, but when we consider that more of our syphilitic, traumatic, and sporadic cases tend toward the lower end of the feebleminded group, and when we remember that with such cases there is often a seemingly normal family tree, the drop in the curve appears logical.”
The situation with the idiots, of whom only 38 came into Fernald’s study, was similar; 12 out of 38, or 32%, of idiots, had good family histories. On these figures, how unfortunate it would be to dub feeblemindedness hereditary! It is true, however, that 68–70% of the idiots and imbeciles, judging by W. E. Fernald’s intensive study, do have bad family histories.
Goddard[20] states that of all the causes of feeblemindedness, there is perhaps none for which there is less evidence than syphilis. Goddard found syphilis in 27 of his intensively charted cases of feeblemindedness, that is, in 9% of all his charts. He finds the majority of the syphilis cases occurring in relatives of the feebleminded to be in the hereditary group; for example, of 164 charts in the hereditary group, 17, or 10%, showed syphilis. In 34 charts in a group termed “probably hereditary” 3, or 9%, showed syphilis. Of 37 charts in the group termed “neuropathic” 4, or 11%, showed syphilis, whereas in 57 “accident” and 8 “no cause” groups, there were but 2 (4%), and one, or 13%, showing syphilis. However, Goddard concedes that much more careful studies are necessary if we are to give an exact evaluation of syphilogenic feeblemindedness.
The first ten of the Waverley Anatomical Series are shortly to be described in a forthcoming publication.[21] Of these ten cases, four showed some slight evidence of chronic inflammatory changes, indicating the possibility of a syphilitic or similar infectious condition. These cases, be it remembered, were not cases of juvenile paresis, but cases of what, for the lack of a better name, may be called “ordinary” feeblemindedness.
If all or any of these processes are syphilitic, the syphilis is virtually extinct. The cases had not been treated for syphilis and were not regarded as syphilitic, though several of them showed a few stigmata somewhat suggestive of syphilis. The anatomical conclusion at this time is still doubtful.
As in the text case, the hypothesis of syphilis as a direct cause for simple feeblemindedness must be entertained for a few cases. In any event, it would not seem logical to let any institution for the feebleminded run without a Wassermann analysis of the population. In addition to the Wassermann data from the blood serum, osteological data from the X-ray have proved of occasional value for syphilis diagnosis in this as in other groups.
“Within the gates of Hell sat Sin and Death.”
Paradise Lost, Book X, Line 230.
VI. NEUROSYPHILIS AND THE WAR
Although the American toll of war syphilis has not yet begun and although the crop of neurosyphilis due to war infections may not arrive until the mid or late twenties of the century (witness German experience in the eighties of the last century), it seems proper here to give a number of abstracts _re_ neurosyphilis as it has developed in the war. Available reports from English, French, and German sources have been levied upon for the years 1914–16.
It is clear that all the armies have had their share of neurosyphilitics, some clearly diseased before enlistment, some developing symptoms as a result of training, stress, or shock, others hastened or made worse by war conditions.
There are important questions of pension, retirement, and compensation for neurosyphilitics. No previous war has had the benefit of the Wassermann reaction and other exact tests bearing upon the nature, progress, and curability of neurosyphilis.
That we shall have our fill of pension and other problems can already be seen from continental reports. Thibierge,[22] for example, states that syphilis has become a real epidemic among the French soldiers and mobilized munition workers.
Hecht[23] of Austria claims that no less than an equivalent of 60 army divisions have been temporarily withdrawn from fighting on the Teutonic side for venereal diseases. He commends Neisser’s idea that salvarsan and mercury should be given in the trenches. While hundreds or thousands of Austrians are sick with syphilis, sound and healthy men are being shot down in their stead. The diagnosis of syphilis, according to Hecht, ought to be a signal for sending the men to the front. He makes even the somewhat bizarre suggestion that special companies of syphilitics should be formed, for convenience of treatment, on the firing line.
Not only is the syphilis problem in the army of importance to the military authorities, but also to the civil population, and perhaps to them a greater problem. With the great increase of venereal disease that is the result of the conditions of army life in war time, there will be a considerable percentage of cases developing neurosyphilis a number of years after discharge from the army, but caused by the infection acquired during service. In addition many men will bring the disease back to America in an infectious stage and spread it. We would advocate that the names of all soldiers who had acquired syphilis and were not considered cured at time of discharge should be given to health organizations in their home states that they may be given further care.
These practical and several theoretical questions are raised by the following fourteen cases which we have condensed from their sources.
=A tabetic lieutenant “shell-shocked” into paresis? Case from Donath of Vienna.=
=Case A.=[24] An apparently competent German professor in an intermediate school, a lieutenant of infantry reserves, 33 years old, on the 17th August, 1914, was stunned for a while by the shock of a cannon-firing 25 feet away. Urination became difficult. Headaches and limb pains ensued, with paralysis of fingers, gastric troubles, forgetfulness especially for names, insomnia, and general scattering of mental faculties.
=Neurologically=, the pupils were irregular, left larger than right; Argyll-Robertson reaction. Right knee-jerk livelier than left. Achilles reactions absent. Slow and dissociated pain reactions in feet, lower thighs and lower quarter of upper thighs, with hypalgesia or analgesia. Station good; gait steady. Mentally depressed, slow of thought. Speech poor and of indistinct construction (mild dementia). Calculation ability poor. No pleasure in work.
Wassermann reaction of serum weakly positive.
It seems that for a year the patient had been subject to spells of anger. He was irritated by his wife who had been nervous since an earthquake.
_On the occasion of the earthquake_, 1911, the patient himself had had a spell of _difficulty with urination_. The spell had lasted two or three months. The patient had had a chancre in 1902, “cured” in four or five weeks with xeroform. In 1908, when about to marry, he had had six mercurial inunctions.
1. Is this a case of traumatic paresis? From the somewhat meagre account it would appear that Donath’s lieutenant should rather be termed “shell-shock paresis,” in the sense of a paretic neurosyphilis liberated by shell-shock (using shell-shock in the sense of a shock _without_ direct brain injury).
2. What compensation is due such a man as Donath’s lieutenant? The ordinary principles applicable to traumatic paresis are not here in point, since no symptoms pointing to trauma of brain ever supervened. See discussion under Case G.
3. How frequent is paresis in armies? R. L. Richards in White and Jelliffe’s Treatment of Nervous and Mental Diseases writes as follows (of course concerning peace times):
“The French estimate that paresis cases are 7 per cent of all their military cases. The German estimate is 6.6 per cent. In our own army at the Government Hospital for the Insane, of 490 cases of mental diseases among officers and enlisted men, 37, or 7 per cent, were paresis. During the Russo-Japanese War, in the Russian Psychiatric Hospital at Harbin, the percentage of paresis was 5.6 per cent among the cases developing at the front.”
=A French soldier “shell-shocked” (also burial) into incipient tabes dorsalis? Case from Duco and Blum of Paris.=
=Case B.=[25] A French soldier was buried by effects of shell explosion September 8th, 1914. He sustained no wound or fracture.
Incontinence of urine developed. Anesthesia of penis and scrotum. Reflexes absent; pupils sluggish. Wassermann reactions suspicious.
The diagnosis =tabes dorsalis incipiens= was made (hematomyelia of conus terminalis eliminated).
The patient was estimated to be “40% incapacitated,” according to the French “_échelle de gravité_” of conditions. A full pension would not be justified in the opinion of the French authors.
1. Is there evidence of an increase or exacerbation of tabes dorsalis in the war? Birnbaum,[26] reviewing German war neurology, quotes Weygandt as believing that the war has probably had to do with the production of both tabes and paresis in many instances. Other cases, however, have merely been made worse by the war stress. Thirdly, there are cases in which the war stress has done no harm whatever. Westphal has seen both tabes and paresis develop in men who had never before shown any mental or physical symptoms whatever, and accordingly, Westphal must be counted among those who regard war stress as a liberating factor for these diseases. Redlich and Donath are cited in the same connection. (The case of Donath is the case presented above as Case A.)
A very interesting claim was made by Cimbal to the effect that he found many examples of paresis developing in the early period of the war, particularly in November and December, 1914. Later, according to Cimbal, cerebrospinal syphilis and tabes became more prevalent.
=Neurosyphilis in a German recruit, possibly AGGRAVATED ON military SERVICE. Pension not allowable. Case from Weygandt.=
=Case C.=[27] A German, long alcoholic and thought to be weakminded, volunteered, but shortly had to be released from service. He began to be forgetful and obstinate, cried, and even appeared to be subject to hallucinations. The pupils were unequal and sluggish. The uvula hung to the right. The left knee-jerk was lively, right weak. Fine tremors of hands. Hypalgesia of backs of hands. Stumbling speech. Attention poor.
It appeared that he had been infected with syphilis in 1881 and in 1903 had had an ulcer of the left leg.
The military commission denied that his service had brought about the disease. In the phrase of the Canadian Pension Board the German commission would probably have rendered a report “aggravated on service,” not “by service.” (See Canadian cases D, E, and F.)
1. Has paresis increased in the war? Both French and German figures controvert the claim. Marie, for example, found not a single paretic amongst the skull injury cases at the Salpétrière. Most authors are found demonstrating cases which they clearly regard as in some way produced or unfavorably influenced by the war. There seems, therefore, to be a little inconsistency between the general statement that paresis has not increased in the war and the somewhat frequent cases described as occurring in and modified by the war. However, Bonhoeffer, on the basis of nine months’ war experience, also holds it to be probable that paresis is no more frequent in the field than in the home population.