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Stiripentol in the Management of Epilepsy

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Abstract

Stiripentol is a structurally unique antiepileptic drug that has several possible mechanisms of action, including diverse effects on the gamma-aminobutyric acid (GABA)-A receptor and novel inhibition of lactate dehydrogenase. Because of its inhibition of several cytochrome P450 enzymes, it has extensive pharmacokinetic interactions, which often necessitates reduction in doses of certain co-therapies, particularly clobazam. Stiripentol also has a neuroprotective action, by reducing calcium-mediated neurotoxicity. Evidence of its efficacy is most robust for Dravet syndrome, where stiripentol added to clobazam and valproic acid reduces seizure frequency and severity in the majority of cases. Small case series have also suggested benefit for malignant migrating partial seizures in infancy, super-refractory status epilepticus, and intractable focal epilepsy, although larger prospective studies are needed in these disorders.

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  1. Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA

    Katherine C Nickels & Elaine C Wirrell

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  1. Katherine C Nickels
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  2. Elaine C Wirrell
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Correspondence to Elaine C Wirrell.

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Dr. Nickels and Dr. Wirrell have no conflicts of interest.

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Nickels, K.C., Wirrell, E.C. Stiripentol in the Management of Epilepsy. CNS Drugs 31, 405–416 (2017). https://doi.org/10.1007/s40263-017-0432-1

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