Using the advantage of next-generation sequencing (NGS) technologies, we discovered nine novel de novo variants in GABRA1, GABRB2 and GABRG2 that were associated with Dravet syndrome and code for subunits that form the most common GABAA receptor (the α1β2γ2 receptor). This evidence concerns the gene GABRA1 and encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.