Summary
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.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Carpenter S, Karpati G, Andermann F, Jacob JC, Andermann E (1974) Lafora's disease: Peroxisomal storage in skeletal muscle. Neurology 24:531–538
Carpenter S, Karpati G, Robitaille Y, Melmed C (1978) Adult polyglucosan body axonopathy-A distinct chronic neurological disease. J Neuropathyl Exp Neurol 37:598 [Abstr]
Ferguson IT, Mahon M, Cumming WJK (1983) An adult case of Andersen's disease-Type IV glycogenosis: A clinical, histochemical, ultrastructural, and biochemical study. J Neurol Sci 60:337–351
Fukuhara N (1977) Intra-axonal corpora amylacea in the peripheral nerve seen in a healthy woman. J Neurol Sci 34:423–426
Holmes JM, Houghton CR, Woolf AL (1960) A myopathy presenting in adult life with features suggestive of glycogen storage disease. J Neurol Neurosurg Psychiatry 23:302–311
Karpati G, Carpenter S, Wolfe LS, Sherwin A (1969) A peculiar polysaccharide accumulation in muscle in a case of cardioskeletal myopathy. Neurology 19:553–564
McMaster KR, Powers JM, Hennigar GR, Jr, Wohltmann HJ, Farr GH, Jr (1979) Nervous system involvement in type IV glycogenosis. Arch Pathol Lab Med 103:105–111
Okamoto K, Llena JF, Hirano A (1982) A type of adult polyglucosan body disease. Acta Neuropathol (Berl) 58:73–77
Pellissier JF, De Barsy T, Bille J, Serratrice G, Toga M (1981) Polysaccharide (amylopectin-like) storage myopathy: Histochemical, ultrastructural, and biochemical studies. Acta Neuropathol [Suppl] (Berl) VII:292–296
Ramsey HJ (1965) Ultrastructure of corpora amylacea. J Neuropathol Exp Neurol 24:25–39
Robitaille Y, Carpenter S, Karpati G, DiMauro S (1980) A distinct form of adult polyglucosan body disease with massive involvement of central and peripheral neuronal processes and astrocytes: A report of four cases and a review of the occurrence of polyglucosan bodies in other conditions, such as Lafora's disease and normal ageing. Brain 103:315–336
Torvik A, Dietrichson P, Svaar H, Hudgson P (1974) Myopathy with tremor and dementia: A metabolic disorder? Case report with postmortem study. J Neurol Sci 21:181–190
Vos AJM, Joosten EMG, Gabreëls-Festen AAWM (1983) Adult polyglucosan body disease: Clinical and nerve biopsy findings in two cases. Ann Neurol 13:440–444
Yagishita S, Itoh Y, Nakano T (1977) Corpora amylacea in the peripheral nerve axons. Acta Neuropathol (Berl) 37:73–76
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
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
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00687012