<i>Scn1b</i> deletion in adult mice results in seizures and SUDEP

Abstract

Pathogenic loss-of-function variants in <i>SCN1B</i> are linked to Dravet syndrome (DS). Previous work suggested that neuronal pathfinding defects underlie epileptogenesis and SUDEP in the <i>Scn1b</i> null mouse model of DS. We tested this hypothesis by inducing <i>Scn1b</i> deletion in adult mice that had developed normally. Epilepsy and SUDEP, which occur by postnatal day 21 in <i>Scn1b</i> null animals, were observed within 20 days of induced <i>Scn1b</i> deletion in adult mice, suggesting that epileptogenesis in <i>SCN1B</i>-DS does not result from defective brain development. Thus, the developmental brain defects observed previously in <i>Scn1b</i> null mice may model other co-morbidities of DS.