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

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 witho...

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Bibliographic Details
Published in:American journal of neuroradiology : AJNR 2003-10, Vol.24 (9), p.1912-1915
Main Authors: Lin, Doris D.M, Barker, Peter B, Kraut, Michael A, Comi, Anne
Format: Article
Language:English
Subjects:
Aspartic Acid - analogs & derivatives
Aspartic Acid - analysis
Biological and medical sciences
Brain - pathology
Brain Chemistry
Cerebrovascular Circulation
Choline - analysis
Epilepsies, Partial - complications
Humans
Infant
Magnetic Resonance Angiography
Magnetic Resonance Spectroscopy
Male
Medical sciences
Neurology
Pediatrics
Sturge-Weber Syndrome - diagnosis
Sturge-Weber Syndrome - metabolism
Sturge-Weber Syndrome - pathology
Vascular diseases and vascular malformations of the nervous system
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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.
ISSN:0195-6108
1936-959X