GABRG2 variants have been associated with a broad spectrum of autosomal dominant genetic epilepsy (GEs) syndromes, ranging from the relatively benign FS, childhood absence epilepsy (CAE), and genetic epilepsy with febrile seizures plus (GEFS+), to the more severe epileptic encephalopathies, such as Dravet syndrome, epilepsy with myoclonic-atonic, and Rolandic epilepsy [11,15,16,17,18,19,20,21,22,23,24,25]. Here, GABRG2 is linked to encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.