Sturge-Web·er syndrome (stûrj w b r)n. A congenital syndrome characterized by a port-wine stain nevus in the distribution of the trigeminal nerve, homolateral meningeal angioma with intracranial calcification and neurologic signs, and angioma of the choroid, often with secondary glaucoma. Also called angiophacomatosis, encephalofacial angiomatosis, encephalotrigeminal angiomatosis, Sturge's disease, Sturge-Weber disease. |
Sturge-Weber syndrome
[sturj′ web′ər]
Etymology: 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.
Sturge-Weber syndrome (disease) [sterj´web´er]
a congenital syndrome of nevus flammeus of the face (commonly called port-wine stains); angiomas of the choroid and leptomeninges, leading to anoxia; late glaucoma; and often intracranial calcification, mental retardation, and epilepsy may also develop.
Sturge-Weber syndrome
Encephalotrigeminal angiomatosis Neurology A rare disorder characterized by mucocutaneous angiomatosis with port wine stains that also affects the meninges. See Neurocutaneous syndrome.
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