Sturge-Weber syndrome


Sturge-Weber syndrome (disease)

 [sterj´web´er] a congenital syndrome of nevus flammeus of the face (commonly called stains" >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-Web·er syn·drome

(stŭrj vā'bĕr), [MIM*185300] in its complete form, a triad of unilateral occurrence of congenital capillary malformation (flame nevus) in the distribution of the trigeminal nerve; ipsilateral leptomeningeal vascular malformations with intracranial calcification and neurologic signs; and vascular malformation of the choroid plexus, often with secondary glaucoma. Inheritance is unclear with most cases sporadic.
See also: encephalotrigeminal vascular syndrome, Jahnke syndrome. Synonym(s): cephalotrigeminal angiomatosis, encephalotrigeminal angiomatosis, Sturge-Kalischer-Weber syndrome, Sturge-Weber disease

Sturge-Web·er syn·drome

(stŭrj vā'bĕr), [MIM*185300] in its complete form, a triad of unilateral occurrence of congenital capillary malformation (flame nevus) in the distribution of the trigeminal nerve; ipsilateral leptomeningeal vascular malformations with intracranial calcification and neurologic signs; and vascular malformation of the choroid plexus, often with secondary glaucoma. Inheritance is unclear with most cases sporadic.
See also: encephalotrigeminal vascular syndrome, Jahnke syndrome. Synonym(s): cephalotrigeminal angiomatosis, encephalotrigeminal angiomatosis, Sturge-Kalischer-Weber syndrome, Sturge-Weber disease

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.

Sturge-Weber syndrome

The association of a large purple HAEMANGIOMA on one side of the face with a similar malformation of blood vessels in the brain. There may be weakness on the opposite side of the body, epileptic seizures, GLAUCOMA and sometimes mental retardation. (William Allen Sturge, 1850–1919, English physician; and Frederick Parkes Weber, 1863–1962, English physician)

Sturge,

William Allen, English physician, 1850-1919. Sturge syndromeSturge-Kalischer-Weber syndrome - Synonym(s): Sturge-Weber syndromeSturge-Weber disease - Synonym(s): Sturge-Weber syndromeSturge-Weber syndrome - a triad of (1) congenital flame nevus in the distribution of the trigeminal nerve, usually unilateral; - (2) homolateral meningeal angioma with intracranial calcification and neurologic signs; and - (3) angioma of the choroid, often with secondary glaucoma. Synonym(s): cephalotrigeminal angiomatosis; encephalotrigeminal angiomatosis; Sturge-Kalischer-Weber syndrome; Sturge-Weber disease

Weber,

Frederick Parkes, English physician, 1863-1962. Klippel-Trenaunay-Weber syndrome - see under KlippelRendu-Osler-Weber syndrome - see under RenduSturge-Kalischer-Weber syndrome - Synonym(s): Sturge-Weber syndromeSturge-Weber disease - Synonym(s): Sturge-Weber syndromeSturge-Weber syndrome - see under SturgeWeber-Christian disease - a group of conditions with recurrent subcutaneous nodules, with or without fever or suppuration, followed by depression of the overlying skin. Synonym(s): Christian disease (2); nodular nonsuppurative panniculitisWeber-Cockayne syndrome - epidermolysis bullosa of the hands and feet.

Sturge-Web·er syn·drome

(stŭrj vā'bĕr sindrōm) [MIM*185300] In its complete form, triad of unilateral occurrence of congenital capillary malformation (flame nevus) in distribution of the trigeminal nerve; ipsilateral leptomeningeal vascular malformations with intracranial calcification and neurologic signs; and vascular malformation of the choroid plexus.