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.
From the *Department of Radiology, Osaka National Hospital, Osaka, Japan, †Department of Radiology, Institute of Medical Science, University of Tokyo, Tokyo, Japan, ‡Department of Radiology, University Hospital, Mizonokuchi, Teikyo University, Kanagawa, Japan, §Department of Radiology, School of Medicine, Teikyo University, Tokyo, Japan, ∥Department of Pediatrics, University Hospital, Mizonokuchi, Teikyo University, Kanagawa, Japan, and ¶Department of Radiology, Osaka University Graduate School of Medicine, Faculty of Medicine, Osaka, Japan.
Received for publication March 3, 2005; accepted March 25, 2005.
Reprints: Mio Sakai, Department of Radiology, Osaka National Hospital, 2-1-14 Hoenzaka, Chu-o-ku, Osaka 540-0006, Japan (e-mail: email@example.com).