February 1984

Large-Fiber Sensory Neuronopathy in Autosomal Dominant Spinocerebellar Degeneration

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.

Arch Neurol. 1984;41(2):175-178. doi:10.1001/archneur.1984.04050140073028

• 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.