Interestingly, a loss-of-function variant of the S4–S5 Arg in human KCNQ2 (R213W) associates with both benign familial neonatal convulsions (Sadewa et al., 2008; Miceli et al., 2013) and the much more severe epileptic encephalopathy (Sadewa et al., 2008; Miceli et al., 2013; Milh et al., 2015). Here, KCNQ2 is linked to Epileptic encephalopathy.