ALDH7A1 and hyperinsulinemic hypoglycemia, familial, 4: On the other hand, the lack of response to standard treatment for these conditions can also not be used as a criteria to rule out the diagnosis: patients with GLUT1 deficiency can still show recurrence of seizures despite adequate ketosis (20), and in fact up to one third of patients respond poorly to a ketogenic diet (21), while patients with PDE might not show an instant and obvious response to pyridoxine administration (5).