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Published on: January 22, 2017
L-carnitine supplementation in childhood epilepsy: current perspectives
D C De Vivo1, T P Bohan, D L Coulter
1Neurological Institute, Columbia-Presbyterian Medical Center, New York, New York 10032, USA.
Insights
L-carnitine supplementation is recommended for children with epilepsy, particularly those on valproate or ketogenic diets. It is crucial for treating valproate-induced liver damage and carnitine deficiency syndromes.
Area of Science:
- Neurology
- Metabolic Disorders
- Pediatric Medicine
Background:
- A 1996 panel updated 1989 guidelines on L-carnitine supplementation for childhood epilepsy.
- The panel comprised pediatric neurologists, neurologists, and metabolic experts.
Purpose of the Study:
- To provide updated consensus recommendations for L-carnitine supplementation in pediatric epilepsy.
- To address specific indications and dosages for L-carnitine in various pediatric epilepsy scenarios.
Main Methods:
- A consensus meeting of pediatric neurologists was convened in November 1996.
- The panel reviewed and updated existing guidelines based on expert consensus.
Main Results:
- Intravenous L-carnitine is indicated for valproate (VPA)-induced hepatotoxicity, overdose, and acute metabolic crises.
- Oral L-carnitine is indicated for primary carnitine transporter defects and strongly suggested for secondary deficiencies, VPA-related issues, ketogenic diets, dialysis, and premature infants on TPN.
- Recommended oral dosage: 100 mg/kg/day (max 2 g/day); IV doses for emergencies may exceed this.
Conclusions:
- L-carnitine supplementation plays a vital role in managing specific conditions in pediatric epilepsy.
- Updated guidelines provide clear indications for both intravenous and oral L-carnitine, along with dosage recommendations.
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.
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