Pseudo-maple syrup urine disease due to maternal prenatal ingestion of fenugreek


Correspondence: DrSKorman, Department of Clinical Biochemistry, Hadassah University Hospital, PO Box 12000, Jerusalem 91120, Israel. Fax: +972 2-6435778; email:


Abstract: Fenugreek, maple syrup and the urine of maple syrup urine disease (MSUD) patients all share a characteristic odour originating from a common component, sotolone. Ingestion of fenugreek by mothers during labour resulted in a maple syrup-like odour in their newborn infants, leading to a false suspicion of MSUD.