EMG and Somatosensory, Visual and Auditory Brainstem Evoked Responses were investigated in 14 male patients with Friedreich’s Ataxia.

Needle EMG, motor and sensory conduction was investigated and the most pathology was found in sensory nerve conduction. The most prominent pathology was reduction in amplitude of sensory potentials. In addition to peripheral involvement, investigation of somatosensory pathway revealed markedly delayed central conduction time. Auditory Brainstem Evoked Responses showed signs of suggested slowed conduction in peripheral and central pathways. Visual Evoked Responses were found normal. Cerebral CT and MRI were investigated in 4 patients and found mild cerebellar atrophy in only one patient.

In the cases of Friedreich’s Ataxia, abnormalities of peripheral and central nerves system was found frequently by neurophysiological investigations.