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Richard H. Bennett, MD; Peter Ludvigson, MD; Guillermo DeLeon, MD; Gerard Berry, MD

Arch Neurol. 1984;41(2):175-178.

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.

Author Affiliations
From the Department of Neurology, Albert Einstein Medical Center, Northern Division (Dr Bennett); Department of Neurology, Temple Health Science Center (Drs Bennett, Ludvigson, and DeLeon); and the Division of Biochemical Development in Molecular Diseases, Department of Pediatrics, Children's Hospital of Philadelphia (Dr Berry), Philadelphia.

Footnotes
Accepted for publication April 14, 1983.

Reprint requests to Department of Neurology, Albert Einstein Medical Center, Northern Division, York and Tabor roads, Philadelphia, PA 19141 (Dr Bennett).

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ABSTRACT