title: Peripheral neuropathy in spinocerebellar degenerations.
abstract: Nerve conduction studies have been performed in 19 subject 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 impairment 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; there was a degeneration of dorsal root ganglion and anterior horn cells as well as of myelinated fibers of all diameters in the sural nerve.
mesh_terms: D000200:Action Potentials; D000293:Adolescent; D000328:Adult; D000368:Aged; D001921:Brain / Q000648:ultrastructure; D002524:Cerebellar Ataxia / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D002648:Child; D005071:Evoked Potentials; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction; D009475:Neurons, Afferent / Q000502:physiology*; D014459:Ulnar Nerve / Q000502:physiology