Polysaccharide storage myopathy.
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.
|Title:||Polysaccharide storage myopathy.|
|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
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