Seizure management in acute hepatic porphyria: risks of valproate and clonazepam.
Seizures may occur in acute intermittent porphyria or other hepatic porphyrias. Management is difficult, because barbiturates and hydantoins exacerbate the porphyric state. We studied one patient with major motor seizures and acute intermittent porphyria. The seizure disorder was exacerbated by phenytoin and did not respond to a high-carbohydrate diet or to intravenous hematin. Clonazepam was ineffective in treating the seizures and, in high doses, seemed to exacerbate the porphyria. Both clonazepam and valproate were porphyrinogenic in experimental test systems. Because both drugs may exacerbate the acute hepatic porphyrias, bromide remains the drug of choice to treat these seizures.
D000624:5-Aminolevulinate Synthetase / Q000378:metabolism*; D000328:Adult; D000622:Aminolevulinic Acid / Q000378:metabolism; D000818:Animals; D001570:Benzodiazepinones / Q000627:therapeutic use*; D002642:Chick Embryo; D002998:Clonazepam / Q000627:therapeutic use*; D003577:Cytochrome P-450 Enzyme System / Q000378:metabolism; D006427:Hemin / Q000494:pharmacology; D006801:Humans; D008107:Liver Diseases / Q000188:drug therapy*; D008297:Male; D011162:Porphobilinogen / Q000378:metabolism; D011164:Porphyrias / Q000188:drug therapy*; D051381:Rats; D012640:Seizures / Q000188:drug therapy*; D014635:Valproic Acid / Q000627:therapeutic use*
D001570:Benzodiazepinones; D002998:Clonazepam; D014635:Valproic Acid; D006427:Hemin; D011162:Porphobilinogen; D000622:Aminolevulinic Acid; D003577:Cytochrome P-450 Enzyme System; D000624:5-Aminolevulinate Synthetase