Electrophysiological studies in spinocerebellar ataxia type 6: a statistical approach

In spinocerebellar ataxia type 6 (SCA6), the cerebellum is predominantly af fected. 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 assess whethe r electrophysiological abnormalities are directly associated with SCA6 beca use 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 BAE P latencies of wave I was prolonged and compound muscle action potentials o f peroneal nerve and sensory nerve action potentials of sural nerve reduced in SCA6 patients. Our results suggest an existence of peripheral impairmen t in the auditory pathway and axonal neuropathy in SCA6. NeuroReport 11:969 -972 (C) 2000 Lippincott Williams & Wilkins.