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American Journal of Neuroradiology 25:1041-1045, June-July 2004
© 2004 American Society of Neuroradiology

Case Report
PEDIATRICS

Vacuolating Megalencephalic Leukoencephalopathy with Mild Clinical Course Validated by Diffusion Tensor Imaging and MR Spectroscopy

Yi-Fang Tua, Cheng-Yu Chenc, Chao-Ching Huangb and Chang-Shin Leec

a Department of Emergency Medicine, National Cheng Kung University Hospital, Tainan, Taiwan
b Department of Pediatrics, National Cheng Kung University Hospital, Tainan, Taiwan
c Department of Radiology, Tri-Service General Hospital, Taipei, Taiwan

Address correspondence to Chao-Ching Huang, Department of Pediatrics, National Cheng Kung University Hospital, 138 Sheng-Li Road, Tainan City, Taiwan 704

Summary: We report a case of presumed vacuolating megalencephalic leukoencephalopathy (VML) in a 5-year-old boy that was diagnosed when the patient was 34 months old. The patient presented clinically with megalencephaly and delayed onset of slowly progressive neurologic dysfunction. Serial MR imaging and biochemical analysis were performed to differentiate VML from other disease entities with megalencephaly and leukoencephalopathy of infantile onset. Information obtained with diffusion tensor MR imaging revealed nearly intact white matter anisotropy and vectors in parietal and posterior temporal lobes in which hyperintense abnormality was shown on conventional T2-weighted images, and proton MR spectra showed a significant decrease in -acetylaspartate-creatine + phosphocreatine ratio.






Copyright © 2010 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X