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SEP results showed clear differences between groups of FA, evidence of peripheral involvement was seen in all patients, with absence of the N9 potential or a major reduction of its amplitude. In patients in whom central responses could be recorded, conduction velocity was normal or near normal up to the brainstem but was reduced from brainstem to cerebral cortex. Four patients with PEOCA had SEP abnormalities similar to those seen in FA. In the five other patients, the amplitude and latency of N9 were normal but conduction velocity was reduced from brainstem to cerebral cortex. In FA, BAEP were abnormal in all patients with a disease duration of four years or more but were normal in four of the five PEOCA patients. Systematic evoked potential recording is useful in the investigation of hereditary ataxias.<\/jats:p>","DOI":"10.1017\/s0317167100027773","type":"journal-article","created":{"date-parts":[[2016,7,28]],"date-time":"2016-07-28T11:39:37Z","timestamp":1469705977000},"page":"292-298","source":"Crossref","is-referenced-by-count":26,"title":["Evoked Potential Studies in Friedreich's Ataxia and Progressive Early Onset Cerebellar 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