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Ann Neurol. 1983 Apr;13(4):440-4.

Adult polyglucosan body disease: clinical and nerve biopsy findings in two cases.

Abstract

Two women suffered from a similar, slowly progressive illness characterized by upper and lower motor neuron involvement, sensory disturbances, and neurogenic bladder. Radiographic examinations showed cerebral atrophy and, in one, changes on computerized tomography suggesting leukodystrophy. Sural nerve biopsies revealed axonal neuropathy associated with a large number of polyglucosan bodies accumulating predominantly but not exclusively in myelinated axons.

PMID:
6301360
DOI:
10.1002/ana.410130411
[Indexed for MEDLINE]

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