clobazam + stiripentol + valproate

This is a combination therapy consisting of three antiepileptic drugs—clobazam, stiripentol, and valproate—used primarily in the management of Dravet syndrome, a severe form of childhood epilepsy. Clobazam is a benzodiazepine that enhances GABAergic neurotransmission by acting as a positive allosteric modulator at the GABA-A receptor. Stiripentol is an aromatic alcohol anticonvulsant that also acts as a positive allosteric modulator at the GABA-A receptor and inhibits several cytochrome P450 enzymes, thereby increasing plasma concentrations of clobazam (and its active metabolite norclobazam) and valproate. Valproate increases brain levels of gamma-aminobutyric acid (GABA) by inhibiting its degradation and has additional effects on sodium channels. The triple-drug regimen has demonstrated significant efficacy in reducing seizure frequency and duration in patients with Dravet syndrome who are refractory to other treatments[1][2][3][5][6]. This combination is considered standard second-line or adjunctive therapy for Dravet syndrome when monotherapy or dual therapy fails to provide adequate seizure control.

Other names

CLB + STP + VPAtriple-drug therapy