VI TWO CASES OF MULTIPLE SCLEROSIS WITH OBSCURE NEUROLOGICAL AND MENTAL SYMPTOMS (FORMES FRUSTES) (PLATES XVII-XXVI) BY SOLOMON C. FULLER, M.D., HENRY I. KLOPP, M.D., The cases reported in this paper seem to us of interest from at least two points of view: first the obscure neurological and mental symptoms which rendered a clinical diagnosis, if not impossible, certainly difficult; and, second, the rather prevalent opinion that multiple sclerosis is a comparatively rare disease in this country. The neurological symptoms in each of the cases were chiefly of the spinal type; but Case II, toward the end, presented symptoms of Cranial nerve, bulbar and cerebral involvement. Mentally one of them exhibited delusions of a paranoid trend and certain hysterical traits. In the other case, the almost constant clouding of consciousness, motor restlessness and temperature elevation suggested the picture of an exhaustion-infection psychosis, especially when the immediate antecedent history was taken into consideration. 3 With reference to the frequency of multiple sclerosis, wellknown neurologists, such as Prof. Dana' of New York and Dr. Spiller of Philadelphia, incline to the view of a comparative rarity of the disease in America. But Taylor, among those in this country who have given serious attention to multiple sclerosis, assumes a skeptical attitude as to its infrequency. He believes that many cases escape recognition, and more, that it "is a common, organic disease." Aid in clinical diagnosis had been sought in each of the cases here reported, by consultation with neurologists and psychiatrists. of wide reputation, who from time to time had visited the hospital. The correct diagnosis, however, had never been suggested: it was established only after autopsy. Among the possible conditions which had been considered, were hysterical astasia abasia, and possibly a luetic or tubercular meningo-myelitis, for the first case; and for the second, meningitis, meningism, meningo-encephalitis, meningo-myelitis, exhaustion-infection psychosis and “quasi systemic" spinal disease (Putnam-Dana type). The writers had favored the last mentioned, for reason of the associated secondary anæmia, spinal symptoms of motor and sensory character, extensive areas of bronze-like pigmentation of the skin, general muscular wasting-particularly in one of the cases-and the preterminal paraplegia in both of them. In Case I, the "insular scleroses" were most numerous and most extensive in the spinal cord. In the medulla and pons these areas were not only rarely encountered, but were also very small, barely perceptible macroscopically. In the cerebrum of this case, none of the characteristic lesions were found. In addition to small blocks of brain tissue, sections were made on a microtome for whole brain sections, and stained for the display of fiber tracts; but the entire cerebrum was not sectioned serially, only the area limited by the anterior and posterior extremities of the corpus callosum. Moreover, the blocks were extremely brittle and complete sections were difficult to procure, so that an unbroken series, even for this area, was not obtained. In Case II, extensive and numerous lesions were shown in the spinal cord, medulla, pons and cerebrum, in the last mentioned region, some rather large lesions; and particularly were they present in the neighborhood of the ventricles. All of these disseminated sclerotic areas conform, in the main, to the classical descriptions of multiple sclerosis; and yet, as has been noted, the clinical symptoms, to say the least, were misleading. In the light of the completed histories, certain clinical data, perhaps, had not been given their due consideration. It is far from our intention to present these two cases as evidence of the frequent non-recognition of multiple sclerosis, but rather as two cases with clinical symptoms that were certainly baffling, and which would have remained "innocent" of a correct diagnosis without autopsy. Incidentally, they also serve to call attention to the somewhat protean character which the disease may assume clinically, as exemplified in Case II. The history of these cases is as follows: CASE I.-No. 7368, an unmarried women of forty-six years was admitted to Westborough State Hospital, April 12, 1907. Family History-Father died at the age of seventy-six from a cardiac affection; mother living, aged 82, but suffers from some form of heart disease which is not definitely described. Two brothers died in infancy of cholera infantum; one sister has pulmonary tuberculosis; four sisters and two brothers are living and enjoy fair health. A grandfather died at the age of 70, cause unknown; a grandmother at the age of 46 from "cancer of the stomach". One aunt was insane. All of the family are reported as more or less "nervous". Father and mother are first cousins. Previous History. The patient, seventh in a family of ten children, states that, as far back as her memory goes, she has always been a nervous person; that she had diphtheria as a child, when a young girl a sunstroke and once fell from a hay loft; that many years ago she had some "head trouble" and an ear affection; and that throughout her life she had been subject to frequent at tacks of tonsilitis. Menstrual function was established at the age of fifteen and a half years, but soon after ceased for about two months. At this time there was something the matter with her head and there was also a discharge from one of the ears. The character of the head affection was not definitely described; the discharge from the ear was evidently, from her descriptions, the result of middle ear disease; neither was associated with the sunstroke or fall noted above. Her knees, she says, have always been weak, and ever since her twelfth year she has experienced, off and on, "creeping sensations" in the lower extremities. Of late, these parasthesias have increased and she has had difficulty in walking. She has been treated at several sanatoria for these conditions but without any favorable result. In 1890 she had a severe attack of influenza, and in 1894 typhoid fever and pneumonia. In 1899 she suffered what is described by the patient and her friends as a "shock", but there was never any loss of consciousness. This "shock", from the descriptions given, appears to have been a sudden culmination in an inability to walk. For some little time prior to this affair the parasthesias described above had been more severe; there had been also considerable numbness of the legs and feet, causing her to stumble frequently. Finally, one day while out for a walk she fell and could not get up unaided; when assisted to her feet she claimed an inability to walk without support, but this latter her friends had doubted. Ever since this occasion she has been afraid that she would fall and injure herself whenever she attempted to walk, and since then she has walked but little; in fact, soon after she ceased walking. The mental make-up of the patient, her friends say, has always been vascillating, moody and reserved. Since 1891, a marked change in her disposition has been noted: she has been faultfinding, deceptive to her relatives, would not comply with reasonable requests, communicated her fancied grievances to strangers, wrote complaining letters to city authorities and to the officials of sanatoria at which she had been formerly a patient. On one occasion, before she ceased walking, she had attempted to run away from home and recently has wanted to jump from a window. She explained these actions as only attempts to get medicine for her illness which relatives did not provide, since none of them believed she was ill and all of them were persecuting her. She had been very bitter, especially against a brother. Lately she has become very slovenly in habits and untidy in her person. She has developed the idea that, in addition to the trouble with her legs, she has other serious diseases. Here. On admission, a poorly nourished, middle-aged woman, 5 ft. 41⁄2 inches tall and weighing 7534 lbs., presents a very striking bronze-like pigmentation of the skin of face, arms and anterior surface of the trunk and thighs. On the dorsa of the hands are leucodermic areas, some of these sharply defined by the pigmented areas, others less so. The rather elongated face, high cheek bones and the peculiar bronzing of the skin of the face suggest the features of an American Indian. She appears unable to walk without support on each side, even then the gait is ataxic. There is slight dorsal scoliosis, right. The heart's action is weak but regular and a faint systolic murmur is heard; pulse 80; respirations 24 per minute, prolonged expiration. The tongue is heavily coated, except for its borders, where it is very red; bowels loose, but no incontinence of feces or of urine. A blood examination reveals erythrocytes 3,000,000 per cmm. leucocytes 6,000, Hb. 45. The pupils are equal, reacting to light and accommodation, although the left pupil reacts rather sluggishly to light. No sensory or motor disturbance of cranial nerves detected. Knee jerks elicited, somewhat exaggerated; double ankle clonus and a questionable double Babinsky; wrist, pectoral, jaw and abdominal reflexes active. The pharyngeal reflex is absent. There seems to be a rather general diminished pain and tactile perceptibility, particularly on the chest, over the mammæ, and the upper three-fourths of the back. Coordination tests are poorly executed. She complains of numerous paræsthesia of the trunk, abnorminal viscera and extremities, and also of almost constant pains in the legs and arms which she calls "neuralgia". She says she is chilly all the time, and that is the reason for being clad in three suits of heavy underwear, although it is mid April and the temperature is fairly warm. The speech is a little indistinct, something of a lisp, but in good tempo and without stumbling over test phrases. Mentally she was without true insight into her condition; expressed delusions of persecution, chiefly against her relatives; and related various subjective symptoms which to her mind indicated clearly certain diseases-cancer of the stomach, large abdominal tumor, etc. Her general manner was somewhat affected and in many little ways her conduct suggested a bid for sympathy. When her stories of ill treatment did not arouse in the examiner a partisan feeling for her, and when certain special inadvisable favors were denied her, she became quite irritated and was just a bit abusive. During the six weeks following her admission, she was constantly demanding of the nurses some attention, making no effort to aid herself when they were near but managing quite well when alone. Any failure to immediately grant her requests, whether reasonable or not, was at once spoken of as abuse from the hospital |