Large-Fiber Sensory Neuronopathy in Autosomal Dominant Spinocerebellar Degeneration

• 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, h...

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Published inArchives of neurology (Chicago) Vol. 41; no. 2; pp. 175 - 178
Main Authors Bennett, Richard H, Ludvigson, Peter, DeLeon, Guillermo, Berry, Gerard
Format Journal Article
LanguageEnglish
Published Chicago, IL American Medical Association 01.02.1984
Subjects
Adolescent
Adult
Ataxia - complications
Ataxia - metabolism
Ataxia - pathology
Biological and medical sciences
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Female
Friedreich Ataxia - complications
Friedreich Ataxia - pathology
Humans
Male
Medical sciences
Middle Aged
Neurology
Peripheral Nervous System Diseases - complications
Peripheral Nervous System Diseases - pathology
Sensation
Human
Nervous system diseases
Autosomal character
Adolescent
Young adult
Heredodegeneration
Sensory disorder
Atypical
Dominant character
Friedreich ataxia
Spinocerebellous heredodegeneration
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Summary:• 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.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
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ISSN:0003-9942
1538-3687
DOI:10.1001/archneur.1984.04050140073028