Cerebral Perfusion Abnormalities in Children With Sturge-Weber Syndrome Shown by Dynamic Contrast Bolus Magnetic Resonance Perfusion Imaging

Abstract

Sturge-Weber syndrome is characterized by leptomeningeal angiomatosis and a facial naevus that is usually unilateral. Magnetic resonance imaging is the cornerstone of confirming the disease and judging the extent of the abnormalities. It has been shown, however, that brain perfusion abnormalities on nuclear medicine imaging often are more extensive than the abnormal leptomeningeal enhancement on magnetic resonance. In this article, we assess the utility of magnetic resonance perfusion in demonstrating perfusion abnormalities in pediatric cases of Sturge-Weber syndrome. Magnetic resonance perfusion studies were performed on 7 consecutive children who presented to our department with clinically suspected Sturge-Weber syndrome. The extent of time to peak abnormality on dynamic gadolinium bolus magnetic resonance perfusion imaging was compared with the extent of leptomeningeal enhancement and the presence of venous abnormalities. Good magnetic resonance perfusion data were obtained in all 7 cases. Perfusion abnormalities were closely anatomically related to meningeal enhancement on postcontrast T1-weighted imaging. However, perfusion abnormalities were found consistently in the vicinity of developmental venous anomalies that were present in 4 of 7 cases. In 1 child, there was a perfusion deficit in the cerebellar lobe contralateral to the leptomeningeal angiomatosis, consistent with crossed cerebellar diaschisis. Magnetic resonance perfusion is a sensitive indicator of perfusion abnormalities in Sturge-Weber syndrome and can be performed easily at the same time as the diagnostic scan. Magnetic resonance perfusion imaging therefore is useful in the assessment of this disease. This approach has the extra advantage of correlating the perfusion abnormalities with the high-resolution imaging that is provided from magnetic resonance imaging.