Although there was no direct evidence suggesting the metabolic dysfunction in genetic epilepsies, some researchers have suggested that metabolism and mitochondrial defects contributing to seizure susceptibility or progression in Dravet syndrome (DS) caused by SCN1A mutations and the ketogenic diet (KD) seemed to be an effective treatment for the patients.37, 38. Here, SCN1A is linked to encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.