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Long-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency neuropathy: Response to cod liver oil

From the Departments of Pediatrics (Drs. Tein and Vajsar, and L. MacMillan) and Laboratory Medicine and Pathobiology (Dr. Tein), University of Toronto, Ontario, Canada; and the Center for Genetics, Nutrition and Health (Dr. Sherwood), Washington, DC.

Address correspondence and reprint requests to Dr. Ingrid Tein, Division of Neurology, The Hospital for Sick Children, 555 University Ave., Toronto, Ontario, Canada M5G 1X8; e-mail: tein{at}sickkids.on.ca

Docosahexaenoic acid (DHA) deficiency has recently been documented in several children with long-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency (LCHADD). We studied a 13-year-old boy with LCHADD who had limb girdle myopathy, recurrent myoglobinuria, and progressive sensorimotor axonopathy with demyelination. At 11 years of age, he was started on cod liver oil extract, high in DHA content. Over 12 months, he demonstrated a marked clinical recovery. Nerve conduction studies (NCS) demonstrated reappearance of previously absent posterior tibial and peroneal nerve responses and the amplitudes on motor ulnar and median NCS markedly increased from 7- to 14-fold, respectively.