Management of and prophylaxis against status epilepticus in children with severe myoclonic epilepsy in infancy (SMEI; Dravet syndrome) – A nationwide questionnaire survey in Japan

Efficacy of antiepileptic drugs for the treatment of Dravet syndrome with different genotypes

“…PB, and especially CBZ, fails to decrease seizures in all SCN1A-positive Inoue et al [12] performed a study to survey the effects of conventional AEDs on Dravet syndrome in Japan, and observed that Br was the most effective drug, followed by CLB, VPA, and ZNS. In another study [13], the efficacy of daily-used drugs against SE was investigated in 99 patients with DS, and it was observed that high efficacy against SE evolution was obtained with the following treatments: KBr (41.7%), ZNS (13.5%), CLB (10.0%), VPA (8.0%), PB (6.7%), and PHT (2.6%). DS patients usually have multiple seizure types and the spectrum of phenotype varies significantly, thus only the effects on GTCS were analyzed in this study.…”

Synergistic GABA-Enhancing Therapy against Seizures in a Mouse Model of Dravet Syndrome

“…A wide range of seizure medications has been used (Chiron and Dulac, 2011) but evidence for best single drug or combination therapy from clinical trials is lacking. Recent case series suggest that bromides (Ernst et al, 1988;Tanabe et al, 2008), topiramate (Nieto-Barrera et al, 2000;Coppola et al, 2002), and levetiracetam (Striano et al, 2007) may be helpful but provide incomplete seizure protection and are associated with significant adverse effects. Monotherapy with any medication tested to date is insufficient to provide adequate seizure control, indicating that combination drug therapy is required.…”

“…Only a single placebo-controlled trial has been performed to date (Chiron et al, 2000). Improved but incomplete seizure control has been reported with bromides (Tanabe et al, 2008), valproate (Rantala et al, 1997), topiramate (Nieto-Barrera et al, 2000;Coppola et al, 2002), stiripentol (Perez et al, 1999;Chiron et al, 2000;Inoue et al, 2009), and clobazam (Chiron et al, 2000;Thanh et al, 2002;Chiron and Dulac, 2011) in various combinations, whereas the sodium channel blockers lamotrigine and carbamazepine exacerbate seizures (Guerrini et al, 1998;Thanh et al, 2002) in a majority of patients.…”

Crisis con fiebre en el primer año de vida: ¿epilepsia del espectro Dravet?

“…Por otra parte, hay fármacos, como el estiripentol, que han demostrado una eficacia en el control de las crisis clónicas en niños con Dravet y que deben ser considerados como tratamiento en estos niños, a pesar de ser fármacos poco empleados en otros tipos de epilepsias 24 . También se conoce que los estatus pueden responder bien al midazolam endovenoso 25 . En un estudio retrospectivo de pacientes adultos, observaron que la evolución cognitiva variaba, pero la mayoría de los pacientes tenía un déficit cognitivo entre moderado y severo 26 .…”