The dietary management consists of a diet low in branched chain amino acids (i.e. low in native protein) with addition of amino acids supplements free from Leucine, Isoleucine and Valine.
Disorders in Branched Chain Amino Acid Metabolism (e.g. MSUD)
Maple Syrup Urine Disease (MSUD) is an inherited rare disorder in metabolism of the branched chain amino acids Leucine, Isoleucine and Valine, leading to an accumulation of these amino acids and their corresponding oxo acids in all body fluids. If not treated, this condition results in severe irreversible neurological damages and finally death.