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Article |

Large-Fiber Sensory Neuronopathy in Autosomal Dominant Spinocerebellar Degeneration

Richard H. Bennett, MD; Peter Ludvigson, MD; Guillermo DeLeon, MD; Gerard Berry, MD
Arch Neurol. 1984;41(2):175-178. doi:10.1001/archneur.1984.04050140073028.
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• 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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