L-carnitine supplementation in childhood epilepsy: current perspectives

Epilepsia. 1998 Nov;39(11):1216-25. doi: 10.1111/j.1528-1157.1998.tb01315.x.

Abstract

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.

MeSH terms

  • Age Factors
  • Ammonia / blood
  • Anticonvulsants / adverse effects
  • Anticonvulsants / pharmacokinetics
  • Anticonvulsants / therapeutic use
  • Carnitine / administration & dosage
  • Carnitine / deficiency*
  • Carnitine / therapeutic use*
  • Chemical and Drug Induced Liver Injury
  • Child
  • Child, Preschool
  • Epilepsy / blood
  • Epilepsy / diet therapy
  • Epilepsy / drug therapy*
  • Food, Formulated
  • Humans
  • Infant
  • Valproic Acid / adverse effects
  • Valproic Acid / pharmacokinetics
  • Valproic Acid / therapeutic use

Substances

  • Anticonvulsants
  • Valproic Acid
  • Ammonia
  • Carnitine