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Peripheral neuropathy in spinocerebellar degenerations
Author(s) -
McLeod J. G.,
Evans W. A.
Publication year - 1981
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880040110
Subject(s) - peripheral neuropathy , medicine , peripheral , physical medicine and rehabilitation , neuroscience , psychology , diabetes mellitus , endocrinology
Nerve conduction studies have been performed in 19 subjects with hereditary spinocerebellar degenerations other than Friedreich ataxia. Clinically, they may be classified as olivopontocerebellar atrophy or cerebello‐olivary degeneration. In 9 patients, sensory conduction was abnormal, and in the whole group there was a significant impariment of sensory conduction and mild slowing of motor conduction in the lateral popliteal nerve. Sural nerve biopsies were performed on 5 patients. In 3 cases there was a mild to moderate reduction of myelinated fibers of all diameters; unmyelinated fibers were normal. In 1 patient from a kindred with a spinocerebellar degeneration in which the inheritance was autosomal dominant, neuropathological findings at autopsy confirmed the clinical diagnosis of the Menzel type of olivopontocerebellar atrophy; thee was degeneration of dorsal root ganglion and anterior horn cells as well as of myelinated fibers of all diameters in the sural nerve.