The phenotypic spectrum of GABRG2 variants extends to the pharmacoresistant epilepsies, including Dravet syndrome and developmental epileptic encephalopathies (DEEs) (Harkin et al., 2002; Shen et al., 2017; Oyrer et al., 2018). Here, GABRG2 is linked to encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.