Intermediate maple syrup urine disease (intermediate MSUD) is a milder form of MSUD characterized by persistently raised branched-chain amino acids (BCAAs) and ketoacids, but fewer or no acute episodes of decompensation. The disease is intermediate maple syrup urine disease (Monarch Disease Ontology identifier MONDO_0017052). Also known as: Intermediate BCKD deficiency, Intermediate MSUD, Intermediate branched-chain 2-ketoacid dehydrogenase deficiency, Intermediate branched-chain alpha-ketoacid dehydrogenase deficiency.