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Peripheral neuropathy associated with mitochondrial myopathy
Author(s) -
Yiannikas C.,
McLeod J. G.,
Pollard J. D.,
Baverstock J.
Publication year - 1986
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.410200211
Subject(s) - axonal degeneration , sural nerve , peripheral neuropathy , pathology , medicine , nerve conduction velocity , myopathy , peripheral , electrophysiology , nerve biopsy , anatomy , endocrinology , diabetes mellitus
Twenty patients with mitochondrial myopathy were investigated for the presence of peripheral neuropathy. There were clinical features of a mild sensorimotor neuropathy in 5 patients (25%) and nerve conduction studies were abnormal in 10 patients (50%). Electrophysiological studies of the whole group showed significant impairment of motor and sensory conduction, compared with controls. Sural nerve biopsy and morphometric studies were performed on 4 patients with clinical neuropathy. There was a reduction in density of myelinated fibers and electron microscope features of axonal degeneration affecting myelinated and unmyelinated fibers. Abnormal mitochondria containing paracrystalline inclusions were seen in the Schwann Cell cytoplasm of two nerves.