Based on the similarities of brain metabolism perturbation shared by GLUT1 deficiency and HI (neuroglycopenia and lack of cerebral alternative fuels), we successfully utilized KD in a patient with severe drug-unresponsive HI with dominant mutation in glucokinase (GCK) gene (GCK-HI), presenting recurrent hypoglycemia, refractory epilepsy and mild intellectual disability [11]. Here, GCK is linked to Hypoglycemia.