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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.
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