A number of mutations have been identified in the γ2 subunit-encoding gene GABRG2, and these mutations are associated with a range of neurological phenotypes, from anxiety and childhood absence epilepsy on one end to Dravet syndrome on the other end [1,2,3,4,5,6,7,8,9,10,11]. This evidence concerns the gene GABRG2 and encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.