ALDH7A1 and status epilepticus: For example, patient G003-1 with EPD presented a favorable response to pyridoxine supplementation, and the seizures stopped completely upon treatment as the causative gene ALDH7A1 encodes the alpha-aminoadipic semialdehyde dehydrogenase involved in the catabolic pathways of lysine; characteristic liver failure was observed in proband G060-1, which was a common cause of death besides the status epilepticus for patients with MTDPS4A before the age of 3 years (Table 1).