Coincidence of myoclonus and multiple sclerosis: dramatic response to clonazepam.
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Biomedical subjects
Publications and source records attributed to L Scheinberg.
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Multiple sclerosis (MS) is one of the most common diseases capable of producing severe disability in the young adult population. In fact, only rheumatic disorders and trauma exceed MS in this respect. Because the total population of patients in the United States is probably 250,000 to 500,000 and as a substantial number suffer severe disability, MS is a disease of considerable social and economic impact. The authors discuss the treatment of many of the most troubling symptoms of MS, including disorders of gait, bladder and bowel, upper extremities, speech, and deglutition. Such treatment entails careful assessment of the patient's neurologic and other symptoms; knowledge of his or her intellectual, emotional, and social skills and demands; and an understanding of the patient's support network.
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We performed a sural nerve biopsy in a patient with cerebrotendinous xanthomatosis (CTX) because of electrophysiologic evidence of peripheral neuropathy. The sections showed a striking loss of myelinated axons, the distribution of which suggested a compressive and/or ischemic process. Biochemical analysis disclosed large amounts of cholestanol, a cholesterol derivative that characteristically accumulates in CTX. However, the biochemical abnormality was not associated with any obvious structural alterations in the myelin lamellae or with abnormal storage material in Schwann's cells.
As part of a larger study of psychosocial aspects of chronic disease, the employment status of 79 male and 233 female multiple sclerosis patients was evaluated by interview to identify: (1) the relationship between degree of disability and employment status and (2) other determinants of employment status. Seventy-seven per cent of the patients were unemployed. Disability level, age, sex, and level of education accounted for 14% of the differences in employment status, with less disabled, older, more educated males being the most likely to be employed. Employment status was unrelated to marital status or type of occupation. Case histories are presented to illustrate psychosocial and environmental factors which might further account for the differences in employment status.