We found that defects in receptor gating were the common deficiency of GABRA1 and GABRB2 Dravet syndrome variants, while mainly trafficking defects were found with the GABRG2 (c.269C>G, p. T90R) variant. The gene discussed is GABRA1; the disease is encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.