Polysaccharide storage myopathy

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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 mucoprtein. No abnormality of glygogen‐pathway enzymes was detected. This suggested that this polysccharide accumulation occurred because the polysaccharide was laid down in a non 7hyphen;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.