Large-fiber sensory neuronopathy in autosomal dominant spinocerebellar degeneration
R. H. Bennett, P. Ludvigson, G. DeLeon and G. Berry
Autosomal dominant hereditary ataxias are heterogeneous groups of disorders
in which cerebellar ataxia and pyramidal, extrapyramidal, and extraocular
signs predominate. We studied a family with this type of disorder with
evidence supporting a large-fiber sensory neuronopathy. Electrophysiologic,
histologic, radiologic, and biochemical features were studied. Neuropathic
features of some forms of autosomal dominant spinocerebellar degeneration
are, therefore, believed to be due to a ganglioneuropathy similar to that
described in Friedreich's ataxia.