There are various epileptic encephalopathies related to vitamin B6 metabolism (Table 6), and pyridoxine-dependent epilepsy (PDE) is the prototype, resulting from a loss of the biologically active pyridoxal-5′-phosphate (PLP) due to a dysfunction of the protein antiquitin (ALDH7A1) [65]. Here, ALDH7A1 is linked to Epileptic encephalopathy.