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These are a group of diseases characterized by neuronal degeneration in the cerebellum, pontine nuclei, and inferior olive. They are also referred to as spinocerebellar atrophies or ataxias (SCA). Some also involve brain stem motor nuclei and/or cerebral cortex. All produce gait ataxia, and some also result in tremors, proprioceptive abnormalities, dysarthria, brain stem motor impairment, or dementia. Most are autosomal dominant in inderitance pattern. The primary cause of these hereditary ataxias also appears to be an unstable expansion of the repeat trinucleotide CAG, similar to Huntington's disease.
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