D-2-hydroxyglutaric aciduria (Monarch Disease Ontology id MONDO_0010924) (D-2-HGA) is a rare clinically variable neurological form of 2-hydroxyglutaric aciduria characterized biochemically by elevated D-2-hydroxyglutaric acid (D-2-HG) in the urine, plasma and cerebrospinal fluid. Also known as: D-2-HGA, D-2-hydroxyglutaric acidemia, D-2-hydroxyglutaric aciduria type 1.