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Polysaccharide storage myopathy.

Thompson, AJ; Swash, M; Cox, EL; Ingram, DA; Gray, A; Schwartz, MS; (1988) Polysaccharide storage myopathy. Muscle Nerve , 11 (4) pp. 349-355. 10.1002/mus.880110411.

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Abstract

In a woman with a slowly progressive adult onset proximal myopathy, muscle biopsy showed storage of PAS positive material in type 1 fibers. This material consisted of a branched chain polysaccharide associated with a mucoprotein. No abnormality of glycogen-pathway enzymes was detected. This suggested that this polysaccharide accumulation occurred because the polysaccharide was laid down in a non-bioavailable form. The clinical and histochemical features in this patient and in the few similar reported cases indicate that polysaccharide storage myopathy is a distinct entity that is allied to the glycogen storage myopathies.

Type: Article
Title: Polysaccharide storage myopathy.
Location: UNITED STATES
DOI: 10.1002/mus.880110411
Keywords: Electromyography, Female, Glycogen Storage Disease, Humans, Microscopy, Electron, Middle Aged, Muscles, Muscular Diseases, Polysaccharides, Tomography, X-Ray Computed
UCL classification: UCL > School of Life and Medical Sciences
UCL > School of Life and Medical Sciences > Faculty of Brain Sciences
URI: http://discovery.ucl.ac.uk/id/eprint/147301
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