Polysaccharide storage myopathy.

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.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Thompson AJ,Swash M,Cox EL,Ingram DA,Gray A,Schwartz MS

doi

10.1002/mus.880110411

subject

Has Abstract

pub_date

1988-04-01 00:00:00

pages

349-55

issue

4

eissn

0148-639X

issn

1097-4598

journal_volume

11

pub_type

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