Sturge-Weber Syndrome
Sturge-Weber syndrome consists of a congenital, usually unilateral, cutaneous capillary angioma involving the upper face, leptomeningeal angiomatosis and, in many patients, choroidal angioma. It has no sex predilection and usually occurs sporadically. The cutaneous angioma sometimes has a more extensive distribution over the head and neck and is often quite disfiguring, especially if there is associated overgrowth of connective tissue. Focal or generalized seizures are the usual neurologic presentation and may commence at any age. There may be contralateral homonymous hemianopia, hemiparesis and hemisensory disturbance, ipsilateral glaucoma, and mental subnormality. Skull radiographs taken after the first 2 years of life usually reveal gyriform (“tramline”) intracranial calcification, especially in the parieto-occipital region, due to mineral deposition in the cortex beneath the intracranial angioma. Treatment is aimed at controlling seizures pharmacologically, but surgical treatment may be necessary. Ophthalmologic advice should be sought concerning the management of choroidal angioma and of increased intraocular pressure.