title: Electrophysiological studies in spinocerebellar ataxia type 6: a statistical approach.
abstract: In spinocerebellar ataxia type 6 (SCA6), the cerebellum is predominantly affected, but several electrophysiological studies have revealed subclinical disorders other than cerebellar lesions. We conducted statistical analyses by comparing SCA6 patients and age-matched normal controls to asses whether electrophysiological abnormalities are directly associated with SCA6 because late onset of SCA6 may involve senile changes. We performed brain stem auditory evoked potentials (BAEP), visual evoked potentials, somatosensory evoked potentials and nerve conduction studies in 10 SCA6 patients. The BAEP latencies of wave I was prolonged and compound muscle action potentials of peroneal nerve and sensory nerve action potentials of sural nerve reduced in SCA6 patients. Our results suggest an existence of peripheral impairment in the auditory pathway and axonal neuropathy in SCA6.
authors.affiliation: Third Department of Internal Medicine, Hiroshima University School of Medicine, Japan.
authors.forename: R
authors.initials: R
authors.lastname: Kumagai
authors.forename: Y
authors.initials: Y
authors.lastname: Kaseda
authors.forename: H
authors.initials: H
authors.lastname: Kawakami
authors.forename: S
authors.initials: S
authors.lastname: Nakamura
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D002531:Cerebellum / Q000473:pathology / Q000503:physiopathology; D004594:Electrophysiology; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005073:Evoked Potentials, Somatosensory / Q000502:physiology; D005074:Evoked Potentials, Visual / Q000502:physiology; D006801:Humans; D008875:Middle Aged; D012091:Repetitive Sequences, Nucleic Acid; D020754:Spinocerebellar Ataxias / Q000473:pathology* / Q000503:physiopathology*; D016008:Statistical Distributions