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February 01, 1999; 52 (3) Brief Communications

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

Ingrid Tein, Jiri Vajsar, Lynn MacMillan, W. Geoffrey Sherwood
First published February 1, 1999, DOI: https://doi.org/10.1212/WNL.52.3.640
Ingrid Tein
MD
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Jiri Vajsar
MD
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Lynn MacMillan
RN
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W. Geoffrey Sherwood
MD
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Citation
Long-chain l-3-hydroxyacyl-coenzyme A dehydrogenase deficiency neuropathy: Response to cod liver oil
Ingrid Tein, Jiri Vajsar, Lynn MacMillan, W. Geoffrey Sherwood
Neurology Feb 1999, 52 (3) 640; DOI: 10.1212/WNL.52.3.640

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Abstract

Article abstract 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.

  • Received May 26, 1998.
  • Accepted October 10, 1998.
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