The anticonvulsant activity of SB2193 was first assayed in the Scn1a+/− mouse model which recapitulates the characteristic symptoms of Dravet syndrome, including the development of spontaneous seizures and premature mortality (Miller et al., 2014). The gene discussed is SCN1A; the disease is encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.