Sturge-Weber syndrome

Sturge-Weber syndrome /sturj″ web″ər/ [William A. Sturge, English physician, 1850–1919; Frederick P. Weber, English physician, 1863–1962] , a congenital neurocutaneous disease marked by a port-wine–colored capillary hemangioma over a sensory dermatome of a branch of the trigeminal nerve of the face. Radiographic examination of the skull reveals intracranial calcification. The cerebral cortex may atrophy, and generalized or focal seizures, angioma of the choroid, secondary glaucoma, optic atrophy, and new cutaneous hemangiomas may develop. There is no known cure. Treatment is supportive and includes anticonvulsive medication. Also called encephalotrigeminal angiomatosis.

Individual with Sturge-Weber syndrome (Albert, Miller, and Jakobiec, 2008)