Letter: Serum creatine phosphokinase and malignant hyperpyrexia.
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Biomedical subjects
Publications and source records attributed to S Currie.
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Evidence is presented that serum creatinine phosphokinase (CPK) activity is of no direct value in screening patients for susceptibility to malignant hyperpyrexia and does not correlate with halothane-induced muscle contracture or the presence of myopathy. Widely differing CPK values were found at different times in the same people. In most "malignant hyperpyrexia" families the susceptible patients had either normal or inconsistently raised CPK values.
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Three cases are reported of concurrence of malignant gliomas (astrocytomas and glioblastomas) with multiple sclerosis (MS). This situation may lead to considerable diagnostic difficulties, yet an accurate appraisal is of prognostic, if not therapeutic, value. There is suggestive but inconclusive evidence that the two conditions may be causally related, malignant astrocytic tumours arising from plaques of MS. The small number of reported cases makes statistical analysis impossible, but the scanty literature may not reflect the true incidence.
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By means of an electrophoretic method lymphocytes from patients with the Guillain-Barré syndrome (`acute idiopathic polyneuritis') have been shown to be sensitized to both encephalitogenic factor (EF) and a similar basic protein prepared from human sciatic nerve (SNBP). Sensitization was more marked in the acute stage of the disorder during which there also appeared to be a degree of sensitization to muscle. The results are consistent with the view that lymphocytic infiltration of peripheral nerves in the condition is of pathogenetic significance.
Lymphocytes of patients suffering from Duchenne and other types of muscular dystrophy and the Kugelberg-Welander syndrome showed marked sensitization to muscle and also to central and peripheral nervous antigens even in preclinical disease. These findings are discussed in relation to the pathogenesis of the disease.
Immunosuppressive drugs were given to seven patients with polymyositis. The in-vitro activity of peripheral blood lymphocytes had previously been studied in five of these patients with findings suggestive of disturbed immunological processes. Some improvement occurred in five cases, but only in two was the improvement marked and sustained. In this small series of cases, the response to treatment was best in a patient with polymyositis who showed no evidence of involvement of tissues or organs other than muscle and in a second case with subacute polymyositis occurring in association with an unidentified connective tissue disorder. The response was less satisfactory in two patients with dermatomyositis, in two with polymyositis associated with systemic sclerosis, and in one in whom the muscle disorder complicated rheumatoid arthritis. At present such treatment is usually given only in cases which are resistant to, or intolerant of, steroids. The relative values of steroid and immunosuppressive therapy are discussed; a combination of the two in moderate doses may eventually prove to be the best initial treatment for the disorder.
An electrophysiological study has been made of extensor digitorum brevis muscles in 19 boys with Duchenne dystrophy. The isometric twitches of the dystrophic muscles developed less tension and were usually slower than those in controls. Impulse conduction velocity appeared to be reduced in distal regions of nerve axons but was normal proximally. The most interesting finding was a reduction in the number of functioning units in all but one of the patients. The sizes of the surviving units suggested that the results could be explained more easily in terms of a neurogenic process than a primary myopathic one.
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