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Adult polyglucosan body disease: Clinical and nerve biopsy findings in two cases
Author(s) -
Vos A. J. M.,
Joosten E. M. G.,
GabreëlsFesten A. A. W. M.
Publication year - 1983
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410130411
Subject(s) - medicine , sural nerve , atrophy , pathology , nerve biopsy , disease , axon , anatomy , peripheral neuropathy , endocrinology , diabetes mellitus
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.