Friedreich's ataxia: Early detection and progression of peripheral nerve abnormalities

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Abstract

The clinical, electrophysiological and sural nerve biopsy features have been studied in 14 patients with Friedreich's ataxia whose ages ranged from 3 to 25 years. The degree of disability and the density of myelinated fibres in the sural nerve is related to the age of the patient. Electrophysiological and pathological abnormalities are present as early as the age of 3 years. Nerve conduction studies and sural nerve biopsy are valuable aids to the early diagnosis of the disease.

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The work was supported by a grant from the Children's Medical Research Foundation.

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