Visual evoked potentials and brain stem auditory potentials in Friedreich's ataxia--a longitudinal study.
Six patients with Friedreich's ataxia, 4 males and 2 females, their ages ranging from 13 to 33 years, were studied. The early manifestations started between age 7 and 13 with an evolution time between 6 and 20 years. Serial visual and brain stem auditory evoked potential recordings were made. A progressive increase in latency, reduction in amplitude and in latency inter-ocular difference of P100 were observed. The pattern of the reversal checker-board visual evoked potential was preserved. A disorganized BAEP pattern, a well defined potential I, a very small potential V and a delay in the interpeak latency were constant findings. The assumption is made of a progressive involvement of both visual and central auditory pathways. Pathophysiological mechanisms are discussed.
D000293:Adolescent; D000328:Adult; D001306:Auditory Pathways / Q000503:physiopathology; D001921:Brain / Q000503:physiopathology*; D001933:Brain Stem / Q000503:physiopathology; D005071:Evoked Potentials; D005072:Evoked Potentials, Auditory; D005074:Evoked Potentials, Visual; D005260:Female; D005621:Friedreich Ataxia / Q000503:physiopathology*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D009431:Neural Conduction; D011930:Reaction Time / Q000502:physiology; D014795:Visual Pathways / Q000503:physiopathology