Age dependence of diffusion-weighted magnetic resonance imaging findings in maple syrup urine disease encephalopathy.

A patient with maple syrup urine disease (MSUD) who developed encephalopathy twice and underwent magnetic resonance examinations, including diffusion-weighted (DW) imaging, is presented. Areas of abnormal intensity on DW images dramatically differed between the initial and second attacks, apparently attributable to the difference in myelination. Our observation demonstrates the age dependence of DW imaging findings in MSUD encephalopathy attributable to progression of myelination and would help in the proper diagnosis of MSUD encephalopathy at any age.