Stiripentol - characteristic of a new antiepileptic drug

Background: As 20 - 30% of patients are refractory to the presently available antiepileptic drugs, there is a strong need for the development of newer antiepileptics. Objective: A question arises whether stiripentol fulfills the criteria for a promising newer antiepileptic drug. Methods: A literature search, including experimental and clinical studies, with no time limit was performed. Results/conclusion: Stiripentol represents a group of drugs indirectly enhancing GABA-ergic neurotransmission and it exerts a activity in some experimental seizure models, including maximal shock (MES)-, pentylenetetrazol-, bicuculline-, strychnine-, and induced convulsions. The drug potently inhibits metabolism of antiepileptic drugs, significantly elevating their plasma and brain concentrations. This requires dosage adjustments of the concomitant drugs used in combination. In the form of an add-on therapy, stiripentol has proved to be effective in partial and atypical absence epilepsies. Considering its particular efficacy against severe myoclonic seizures in infancy (Dravet's syndrome), stiripentol has been awarded an orphan drug status for an adjunctive therapy of this difficult-to-treat condition.