A group of inherited and sporadic disorders which share progressive ataxia in combination with atrophy of the cerebellum; pons; and inferior olivary nuclei. Additional clinical features may include muscle rigidity; nystagmus, pathologic; retinal degeneration; muscle spasticity; dementia; urinary incontinence; and ophthalmoplegia. The familial form has an earlier onset (second decade) and may feature spinal cord atrophy. The sporadic form tends to present in the fifth or sixth decade, and is considered a clinical subtype of multiple system atrophy. (From Adams et al., Principles of neurology, 6th ed, p1085)

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