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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Komure, O., Ichikawa, K., Tsutsumi, A. et al. Intra-axonal polysaccharide deposits in the peripheral nerve seen in adult polysaccharide storage myopathy. Acta Neuropathol 65, 300–304 (1985). https://doi.org/10.1007/BF00687012
- Polysaccharide storage
- Axonal degeneration
- Peripheral nerve
- Glycogen metabolism