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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 24:1912-1915, October 2003
© 2003 American Society of Neuroradiology

Case Report
PEDIATRICS

Early Characteristics of Sturge-Weber Syndrome Shown by Perfusion MR Imaging and Proton MR Spectroscopic Imaging

Doris D.M. Lin^a, Peter B. Barker^a, Michael A. Kraut^a and Anne Comi^b

^a Department of Radiology and Radiologic Science Johns Hopkins University School of Medicine, Baltimore, MD
^b Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD

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Summary: We report the case of a 9-month-old boy with Sturge-Weber syndrome and new onset of seizure. Perfusion MR imaging showed early changes compatible with impaired venous drainage in the affected hemisphere, whereas proton MR spectroscopic imaging revealed a focal parietal area of elevated choline without significant alteration of N-acetylaspartate levels. The perfusion and subtle metabolic abnormalities are comparable with the extent of the overlying leptomeningeal enhancement, illustrating the early pathophysiological manifestation of this disease.