Abstract
Nerve conduction studies were performed on the median, ulnar, and lateral popliteal nerves of 8 patients with Friedreich's ataxia. There was significant slowing of motor conduction, and impairment of sensory conduction. Sural nerve biopsies were performed on 5 of the patients. Histological examination demonstrated a reduction in the numbers of myelinated fibres, particularly those of large diameter. Teased single fibres showed no evidence of active degeneration, but a few fibres were seen to have regenerated after segmental demyelination. A striking feature of the myelinated fibres of two of the nerves was a significant reduction in internodal lengths. Quantitative electron-microscopic studies of the unmyelinated fibres demonstrated that they were present in normal numbers and that their axons were of normal diameter.
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