MONDO_0017054 (thiamine-responsive maple syrup urine disease) (thiamine-responsive MSUD) is a less severe variant of MSUD that manifests with a phenotype similar to intermediate MSUD but that responds positively to treatment with thiamine. Also known as: thiamine-responsive BCKD deficiency, thiamine-responsive MSUD, thiamine-responsive branched-chain 2-ketoacid dehydrogenase deficiency, thiamine-responsive branched-chain alpha-ketoacid dehydrogenase deficiency.