GABRA1 and encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy: They are widely distributed in the CNS, especially on neocortical and hippocampal interneurons, and so individuals with any one of the Dravet syndrome associated GABRA1, GABRB2 or GABRG2 variants would have widespread impairment of α1β2γ2 receptors in the CNS despite having the variants in one of three different GABR genes.