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 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.
Lin
Doris D M
DD
Department of Radiology and Radiologic Science Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Barker
Peter B
PB
Kraut
Michael A
MA
Comi
Anne
A
D001224:Aspartic Acid / Q000031:analogs & derivatives* / Q000032:analysis; D001921:Brain / Q000473:pathology*; D001923:Brain Chemistry; D002560:Cerebrovascular Circulation; D002794:Choline / Q000032:analysis; D004828:Epilepsies, Partial / Q000150:complications; D006801:Humans; D007223:Infant; D018810:Magnetic Resonance Angiography; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology
D001224:Aspartic Acid; C000179:N-acetylaspartate; D002794:Choline