AbstractNerve 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 nerv
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