The same amino acid change that we observed in GABRA2 (Pro280Leu) was reported at the equivalent location in GABRG2 in an individual with Dravet syndrome (NM_198903.2(GABRG2):p.Pro302Leu) (Hernandez et al., 2017), and a different amino acid change was observed in an individual with autosomal dominant hyperekplexia (NM_001146040.1(GLRA1):p.Pro278Thr) (Saul et al., 1999). This evidence concerns the gene GABRA2 and encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.