Multiple-system atrophy is a progressive disorder with features of parkinsonism, cerebellar, autonomic, urinary, and corticospinal dysfunction. It is of unknown etiology. It most commonly affects middle-aged individuals, and both genders are affected equally. Disease course is variable and median survival from first symptoms is about 9 years.
The parkinsonian features are usually unresponsive to levodopa therapy. There may be gait and limb ataxia, orthostatic hypotension, erectile dysfunction, constipation, and decreased sweating. Whereas multiple-system atrophy is a distinct neuropathological entity, the consensus diagnostic criteria depend on specific clinical features. Pathologically, glial cytoplasmic inclusions and degeneration are found throughout the basal ganglia, substantia nigra, brainstem autonomic nuclei, and Purkinje cells of the cerebellum.
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