ERTUĞRUL BOLAYIR, HAYDAR ERDOĞAN
Medeniyet Medical Journal - 1999;14(4):246-247
Sturge-Weber Syndrome (SWS) has no clear pattern of inheritance prevalence and is unknown, but it is less common. Patients have a characteristic conjenital facial port-wine stain that is usually unilateral and involves the V1 of the trigeminal nerve. Other findings include iridic heterochromia, optic atrophy, partial seizures, hemiplegia and hemianopsia. Seizures occur in up to 83 % of patients. Partial motor seizures in the first weeks or months are the usual first symptoms of cerebral dysfunction. Mental subnormality occurs in approximately 60% of patients with SWS. Bufthalmus or glaucoma associated with a diffuse choroidal haemangioma are the characteristic eye abnormalities. MRI and CT shows the leptomeningeal angioma and hemispheric or lobar atrophy. The characteristic "railroad track sign" seen on skull films is due to mineralization of adjacent gyri. The lesion is a venous angioma, with tortuous and dilated vessels on the face, leptomenings and choroid. Treatment is directed toward seizure control with anticonvulsant medications.
