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  • Title: Large-fiber sensory neuronopathy in autosomal dominant spinocerebellar degeneration.
    Author: Bennett RH, Ludvigson P, DeLeon G, Berry G.
    Journal: Arch Neurol; 1984 Feb; 41(2):175-8. PubMed ID: 6318705.
    Abstract:
    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.
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