title: Intra-axonal polysaccharide deposits in the peripheral nerve seen in adult polysaccharide storage myopathy.
abstract: This is the first report of an adult polysaccharide storage myopathy demonstrating polysaccharide deposits within the axons of the peripheral nerve. Histochemical and electron-microscopic studies revealed that the intra-axonal deposits were basophilic, PAS-positive, diastase-resistant, and composed of filamentous and granular material. The structural similarity among the polysaccharide deposits in our case, amylopectin-like materials in type IV glycogenosis, Lafora bodies, corpora amylacea, and polyglucosan bodies is discussed.
mesh_terms: D000328:Adult; D001369:Axons / Q000378:metabolism; D002239:Carbohydrate Metabolism, Inborn Errors / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D008297:Male; D009132:Muscles / Q000473:pathology; D009135:Muscular Diseases / Q000378:metabolism*; D010525:Peripheral Nerves / Q000378:metabolism* / Q000473:pathology; D011134:Polysaccharides / Q000378:metabolism*
chemical_list: D011134:Polysaccharides