Shah, DivaNirula, ApoorvShah, Dwiti2026-05-222026-05-222013https://hdl.handle.net/20.500.14572/6220Diva Shah Assitant Professor, Department of Radiodiagnosis, Pramukhswami Medical College, Shrikrishna Hospital, Gokal Nagar, Karamsad, Anand, Gujrat Apoorv Nirula Resident, Department of Radiodiagnosis, Pramukhswami Medical College, Shrikrishna Hospital, Gokal Nagar, Karamsad, Anand, Gujrat Dwiti Shah Resident, Department of Radiodiagnosis, Pramukhswami Medical College, Shrikrishna Hospital, Gokal Nagar, Karamsad, Anand, GujratAbstract: The newborn presenting with neurological symptoms such as seizures or lethargy due to inborn error of metabolism is an important problem. Maple syrup urine disease (MSUD) is an inherited genetic disease, caused by a deficiency of the catalytic components of α-ketoaciddehydrogenase complex, which is responsible for the catabolism of branched-chain amino acids. The purpose of this case report is to show diffusion-weighted imaging (DWI) MRI findings of acute phase of classic form of MSUD in a newborn although this imaging findings are rare but very typical, known as “MSUD oedema”.en-USMaple syrup urine diseaseMSUD oedemaClassic formmetabolic encephalopathyArticle