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

Docosahexaenoic acid (DHA) deficiency has recently been documented in sever al children with long-chain L-3-hydroxyacyl-coenzyme A dehydrogenase defici ency (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 ex tract, high in DHA content. Over 12 months, he demonstrated a marked clinic al recovery. Nerve conduction studies (NCS) demonstrated reappearance of pr eviously absent posterior tibial and peroneal nerve responses and the ampli tudes on motor ulnar and median NCS markedly increased from 7- to 14-fold, respectively.