Maple syrup urine disease (MSUD) is an inherited autosomal recessive brain dysmyelinating disorders due to enzymatic deficiency of branched-chain alpha-ketoacid dehydrogenase enzyme complex leading to errors in the metabolism of the branched-chains amino acids (leucine, isoleucine and valine) with their accumulation in the urine (hence the name: Maple syrup urine related to the sweetish odor of the urine) and blood.  Branched-chains amino acids (BCAA) are cytotoxic to the brain cells leading to vacculated myelinopathy and cytotoxic brain edema.  

Specific imaging is related to Maple syrup urine disease (MSUD) edema involving the white matter. MR spectroscopy typically shows branched-chain amino-acids and alpha-keto acids with their resonances at 0.9-1.0 ppm.