Sturge-Weber syndrome revisited. Evaluation of encephalic morphological changes with computerized tomography and magnetic resonance.

Pedro Ferro Vilela

Abstract


Sturge-Weber Syndrome (SWS) is characterized by facial flammeus nevus, leptomeningeal angiomatosis and coroidal hemangioma and MRI and CT scans are used to disclose the angiomatosis and secondary brain lesions. We review the CT and/or MRI scan of 26 patients with SWS. In 75% of cases the SWS was unilateral and in 25% bilateral, being the angiomatosis more frequent on occipital lobe (93%) than on the parietal (83%), frontal (53%) and temporal (53%) lobe. Diencephalon was involved in 13%, midbrain in 6% and cerebellum in 6% of cases. Other imaging features were: calcifications (88%), brain atrophy (85%), coroidal plexuses hypertrophy (72%), medullar veins enlargement (61%), ocular coroidal enhancement (20%). MRI was superior in depicting all morphological abnormalities of SWS, but calcifications. MRI is the best imaging method to evaluate morphologically the SWS, being useful to confirm the diagnosis and establish the extension of the disease.

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