(Cho et al. 2017)  This small study of 32 patients with clinically-diagnosed Dravet syndrome sought to determine whether stiripentol’s efficacy differed among those with and without SCN1A mutations. 15 patients had definite SCN1A mutations, and 17 had variants of unknown significance or benign variants. The severity of DS did not differ significantly between the two groups. Stiripentol reduced seizure frequency by 72% in the group with SCN1A mutations and by 50% in the group without SCN1A mutations. Within the mutation group, stiripentol was more effective in those with missense mutations than nonsense mutations, but the conclusion should be interpreted with caution due to the extremely small number of patients in the group.

Cho MJ, Kwon SS, Ko A, Lee ST, Lee YM, Kim HD, Chung HJ, Kim SH, Lee JS, Kim DS, Kang HC. Efficacy of Stiripentol in Dravet Syndrome with or without SCN1A Mutations. J Clin Neurol. 2017 Oct 31. [Epub ahead of print] PubMed PMID: 29141279.