Sturge-Weber syndrome


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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-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.

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.

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.
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Sturge-Weber syndrome

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 syndrome
Sturge-Kalischer-Weber syndrome - Synonym(s): Sturge-Weber syndrome
Sturge-Weber disease - Synonym(s): Sturge-Weber syndrome
Sturge-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 Klippel
Rendu-Osler-Weber syndrome - see under Rendu
Sturge-Kalischer-Weber syndrome - Synonym(s): Sturge-Weber syndrome
Sturge-Weber disease - Synonym(s): Sturge-Weber syndrome
Sturge-Weber syndrome - see under Sturge
Weber-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 panniculitis
Weber-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.
References in periodicals archive ?
3],[4],[5],[6] Sturge-Weber syndrome (SWS), also called encephalotrigeminal angiomatosis, is a neurocutaneous disorder with angiomas that involve the leptomeninges and the skin of the face, typically in the ophthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve.
MR-imaging findings in children with sturge-weber syndrome.
The typical features of Sturge-Weber syndrome include port-wine facial nevus, associated with leptomeningeal angioma intracranial tram track calcification, and cerebral atrophy.
We report asymmetry of bispectral index (BIS) values during maintenance of general anaesthesia in a post-hemispherotomy patient with Sturge-Weber syndrome (SWS), and propose bilateral BIS monitoring for safe maintenance of general anaesthesia in patients with SWS.
Mum-of-two Sara Peachey, 32, from Treherbert, has been a regular user of the Royal Glamorgan with seven-year-old son Harrison, who suffers from Sturge-Weber syndrome - a rare genetic condition which can cause him to have seizures.
The most commonly recognised conditions include neurofibromatosis, tuberous sclerosis and Sturge-Weber syndrome.
6 Differential diagnoses of intraparenchymal-calcified lesions include metabolic disorders (hyperparathyroidism, lead poisoning), infectious disorders (cytomegalovirus infection, toxoplasmosis, tuberculomas), neoplasms (ependymoma, oligodendroglioma), and vascular malformation, including Sturge-Weber syndrome and pial arteriovenous malformation.
He was born with Sturge-Weber syndrome, a rare congenital neurological and skin disorder often associated with port-wine stains on the face, glaucoma and seizures.
2 This anomaly has also been reported to be associated with the Rubinstein-Taybi syndrome, Mohr syndrome, Sturge-Weber syndrome and with anomalies such as odontome, dens invaginatus, double tooth, supernu-merary tooth and impaction.
Due to a condition called Sturge-Weber syndrome, she kept having a series of lifethreatening fits which were attacking her brain.
Disconnection of the entire affected hemisphere is known as hemispherotomy and is usually indicated in syndromes such as hemimegencephaly, Rasmussen's disease and Sturge-Weber syndrome.