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Dietary essential fatty acids, vitamin E, and Charcot-Marie-Tooth disease

Departments of Pediatrics and Physiological Chemistry and the Children's Hospital Research Foundation, Ohio State University, Columbus, OH.

Twenty patients with type I Charcot-Marie-Tooth disease received dietary supplementation with the essential fatty acids (EFA), linoleic and gamma-linolenic acids, and vitamin E. A 3-month blinded trial of placebo (paraffin oil and vitamin E, 81.6 IU/d) was followed by 1 year of 3 grams daily of EFA and vitamin E. Serum fatty acid values doubled, but total esterified fatty acid proportions did not change. Arachidonic acid proportions correlated with the amount of prostaglandin-mediated lymphocyte suppression measured at the same times. Improvement demonstrated at the end of the placebo period by neuropsychological tests and neurologic examination was maintained during the 1 year of EFA supplementation. This effect may reflect a membrane stabilization benefit of vitamin E.

Address correspondence and reprint requests to Dr. Williams, 214 Ross Hall, Children's Hospital, Columbus, OH 43205.

Supported by grants from the Muscular Dystrophy Association and EFAMOL Inc.

Accepted for publication December 27, 1985.

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