Based on the reports of gene abnormalities including haploinsufficiency and heterozygous mutations, an essential role of MUNC-18-1 has been predicted in the etiology of early infantile epileptic encephalopathy with suppression-burst (EIEE; Ohtahara syndrome) and other neurodevelopmental disorders such as neonatal epileptic encephalopathy (NEE), intellectual disability (ID) and autism spectrum disorders (ASD) [8–16]. This evidence concerns the gene STXBP1 and early-infantile DEE.