L-carnitine supplementation in childhood epilepsy: current perspectives.
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.
De Vivo
D C
DC
Neurological Institute, Columbia-Presbyterian Medical Center, New York, New York 10032, USA.
Bohan
T P
TP
Coulter
D L
DL
Dreifuss
F E
FE
Greenwood
R S
RS
Nordli
D R
DR
Shields
W D
WD
Stafstrom
C E
CE
Tein
I
I
D000367:Age Factors; D000641:Ammonia / Q000097:blood; D000927:Anticonvulsants / Q000009:adverse effects / Q000493:pharmacokinetics / Q000627:therapeutic use; D002331:Carnitine / Q000008:administration & dosage / Q000172:deficiency* / Q000627:therapeutic use*; D056486:Chemical and Drug Induced Liver Injury; D002648:Child; D002675:Child, Preschool; D004827:Epilepsy / Q000097:blood / Q000178:diet therapy / Q000188:drug therapy*; D005526:Food, Formulated; D006801:Humans; D007223:Infant; D014635:Valproic Acid / Q000009:adverse effects / Q000493:pharmacokinetics / Q000627:therapeutic use
D000927:Anticonvulsants; D014635:Valproic Acid; D000641:Ammonia; D002331:Carnitine