Next-generation sequencing on 870 patients with Dravet syndrome identified nine variants in GABRA1, GABRB2 and GABRG2 genes, which encode the most common α1β2γ2 GABAA receptor in the CNS. The gene discussed is GABRG2; the disease is encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.