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NEUROLOGY 1980;30:588
© 1980 American Academy of Neurology

Seizure management in acute hepatic porphyria

Risks of valproate and clonazepam

Herbert L. Bonkowsky, M.D., Peter R. Sinclair, Ph.D., Scott Emery, M.D. and Jacqueline F. Sinclair, Ph.D.

Departments of Medicine (Drs. Bonkowsky and Emery) and Biochemistry (Drs. Peter R. and Jacqueline F. Sinclair), VA Medical and Regional office Center, White River Junction, VT, and Dartmouth Medical School, Hanover, NH.

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.

Address correspondence and reprint requests to Dr. Bonkowsky, VA Medical and Regional Oflice Center, White River Junction, VT 05001.

This work was supported by research funds from the US. Veterans Administration and by grant No. CA 25012 from NCI, NM.

Accepted for publication September 25, 1979




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H. Thadani, A. Deacon, and T. Peters
Regular review: Diagnosis and management of porphyria
BMJ, June 17, 2000; 320(7250): 1647 - 1651.
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