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 ?
Sturge-Weber Foundation's (The SWF) international mission is to improve the quality of life and care for people with Sturge-Weber syndrome and associated Port Wine Birthmark conditions through collaborative education, advocacy, research, and friendly support.
A STURGE-WEBER Syndrome is a condition where a port wine stain or a birthmark on the face co-exists with epilepsy.
13) One of the typical imaging findings in Sturge-Weber syndrome is calcification occurring adjacent to a pial angioma, originating in the subcortical white matter and then extending to the cortex (Figure 8).
Texas Children's experts are pursuing another clinical trial to determine if everolimus can reduce seizures in children with Sturge-Weber syndrome, a rare congenital neurological and skin disorder.
Angelina was diagnosed with Sturge-Weber syndrome at just 18 weeks.
The term encephalotrigeminal angiomatosis accurately describes the manifestations of Sturge-Weber syndrome that are the expression of a malformation of meningeal and facial vascular structures during weeks 4 to 8 of gestation.
Mary Haslob lived with Sturge-Weber syndrome for three years before she knew what it was.
May 8, 2013 /PRNewswire-USNewswire/ -- In new findings published today in the New England Journal of Medicine (Epub ahead of print), researchers from the Kennedy Krieger Institute reveal the discovery of the cause - a genetic mutation that occurs before birth - of Sturge-Weber syndrome (SWS) and port-wine stain birthmarks.
Sturge-Weber syndrome (encephalotrigeminal angiomatosis) is a sporadic phakomatosis of unknown etiology that may be characterized by (1):
Thanks to many generous donations, the 2005-2006 Sturge-Weber Foundation raised almost $100,000 in research funding which will be applied to research grant awards benefiting the thousands of children born annually with Sturge-Weber syndrome (SWS) -- a progressive neurological disorder indicated at birth by a port-wine stain birthmark on the forehead and upper eyelid, glaucoma, and seizures, and/or Klippel-Trenaunay -- a birthmark on the limbs causing limb differential and blood flow impairment.
See also: Hemangioma, Ichthyosis, Klippel-Trenaunay Syndrome, Sturge-Weber Syndrome, Vascular Malformations
As national "Save Your Vision" Month gets underway, the importance of preventive eye care is especially meaningful for individuals with Sturge-Weber syndrome, a condition that impacts nearly fifteen thousand Americans.