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- Title
Polysaccharide storage myopathy.
- Authors
Thompson, A. J.; Swash, M.; Cox, E. L.; Ingram, D. A.; Gray, A.; Schwartz, M. S.
- 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.
- Publication
Muscle & Nerve, 1988, Vol 11, Issue 4, p349
- ISSN
0148-639X
- Publication type
Article
- DOI
10.1002/mus.880110411