Electroencephalograms in a case of maple syrup urine disease: their relation to serum levels of branched-chain amino acids.

During dietary treatment of a case of maple syrup urine disease, it was found that abnormal EEGs were observed when serum levels of leucine were abnormally high while those of valine and isoleucine were normal, and also when serum levels of valine and isoleucine were abnormally high while serum leucine levels were normal.