Recessive and rare dominant D2HGDH variants in the chiral configuration enzyme, D-2-hydroxyglutarate dehydrogenase induce D-2-hydroglutaric acid to 2-ketoglutarate, and patients present with epilepsy, hypotonia, and psychomotor retardation[357]. The gene discussed is D2HGDH; the disease is epilepsy.