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Polysaccharide storage myopathy.

A J Thompson·M Swash·E L Cox·D A Ingram·A Gray·M S Schwartz
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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.

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ElectromyographyFemaleGlycogen Storage DiseaseHumansMicroscopy, ElectronMiddle AgedMusclesMuscular DiseasesPolysaccharidesTomography, X-Ray ComputedCase ReportsJournal Article

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A J Thompson, M Swash, E L Cox, D A Ingram, A Gray, M S Schwartz. 1988. Polysaccharide storage myopathy.. https://doi.org/10.1002/mus.880110411

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