"Polymyositis" with involvement of facial and distal musculature. One form of the fascioscapulohumeral syndrome?
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Biomedical subjects
Publications and source records attributed to P Hudgson.
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Three cases are described presenting with progressive contractures in whom the muscles felt firm and `woody' and a clinical diagnosis of myosclerosis was made in each case. A patient and his young sister were shown to be suffering from spinal muscular atrophy with superimposed, excessive proliferative activity of fibroblasts, and in these cases a beneficial effect of penicillamine was demonstrated. The third case with a clinical picture of myosclerosis was found on muscle biopsy to have extensive inflammatory infiltration of the connective tissue, and responded temporarily at least to high doses of corticosteroids. It is emphasized that several different disease entities can give rise to this clinical picture.
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The finding of a relative absence of degeneration and regeneration in a muscle biopsy taken at 2½ weeks of age from a boy who later showed the florid pathological changes of preclinical Duchenne muscular dystrophy prompted a review of muscle biopsies taken from boys in the preclinical and early clinical stages of this disease. Only one other biopsy obtained in the first year of life was available. In this, taken at 2 months, there were active changes present. These findings suggest that biopsies taken to detect preclinical cases should not be performed at less than 2 months of age, and raise the possibility of the existence of a stage in Duchenne muscular dystrophy before active degeneration when the muscle shows little or no change on light microscopy.
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Baclofendagger (Lioresal), a derivative of gamma-aminobutyric acid, was introduced in 1966 as a possible treatment for spasticity due to corticospinal tract lesions. Preliminary studies suggested that it may be more effective than other spasmolytic agents currently available, and a double-blind controlled trial in a group of 23 patients against placebo has shown it to be significantly more effective.
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