Open AccessUnusual cause of hyperammonemia in two cases with short-term and long-term valproate therapy successfully treated by single dose carglumic acid
Author(s) -
Çiğdem Seher Kasapkara,
Murat Kanğın,
Funda Feryal Taş,
Yasemin Topçu,
Remezan Demir,
Mehmet Nuri Özbek
Publication year - 2013
Publication title -
journal of pediatric neurosciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.247
H-Index - 18
eISSN - 1998-3948
pISSN - 1817-1745
DOI - 10.4103/1817-1745.123697
Subject(s) - hyperammonemia , valproic acid , medicine , urea cycle , encephalopathy , anticonvulsant , adverse effect , liver failure , epilepsy , pediatrics , gastroenterology , pharmacology , psychiatry , biochemistry , chemistry , amino acid , arginine
Valproic acid (VPA) is an antiepileptic drug which is used in the treatment of various seizure disorders including tonic-clonic, myoclonic, absence, partial seizures and psychiatric disorders. VPA is usually well tolerated, but severe adverse effects may occur. Hyperammonaemic encephalopathy (HE) is a rare and potentially fatal complication of VPA treatment. The mechanism by which valproate induces hyperammonemia remains incompletely understood but is likely to relate to the urea cycle. Herein we present two cases with valproate-induced hyperammonemia at therapeutic valproate levels without signs of liver failure and were successfully treated by a single dose of carglumic acid.