l-Carnitine Supplementation in Childhood Epilepsy: Current Perspectives
Version of Record online: 3 AUG 2005
Volume 39, Issue 11, pages 1216–1225, November 1998
How to Cite
De Vivo, D. C., Bohan, T. P., Coulter, D. L., Dreifuss, F. E., Greenwood, R. S., Nordli, D. R., Shields, W. D., Stafstrom, C. E. and Tein, I. (1998), l-Carnitine Supplementation in Childhood Epilepsy: Current Perspectives. Epilepsia, 39: 1216–1225. doi: 10.1111/j.1528-1157.1998.tb01315.x
- Issue online: 3 AUG 2005
- Version of Record online: 3 AUG 2005
- Accepted June 1, 1998.
- Ketogenic diet;
Summary: In November 1996, a panel of pediatric neurologists met to update the consensus statement issued in 1989 by a panel of neurologists and metabolic experts on L-carnitine supplementation in childhood epilepsy. The panelists agreed that intravenous L-carnitine supplementation is clearly indicated for valproate (VPA)-induced hepatotoxicity, overdose, and other acute metabolic crises associated with carnitine deficiency. Oral supplementation is clearly indicated for the primary plasmalemmal carnitine transporter defect. The panelists concurred that oral L-carnitine supplementation is strongly suggested for the following groups as well: patients with certain secondary carnitine-deficiency syndromes, symptomatic VPA-associated hyperammonemia, multiple risk factors for VPA hepatotoxicity, or renal-associated syndromes; infants and young children taking VPA; patients with epilepsy using the ketogenic diet who have hypocarnitinemia; patients receiving dialysis; and premature infants who are receiving total parenteral nutrition. The panel recommended an oral L-carnitine dosage of 100 mg/kg/day, up to a maximum of 2 g/day. Intravenous supplementation for medical emergency situations usually exceeds this recommended dosage.