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

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 ?
Magnetic resonance imaging is one of the key imaging modalities that confirms the clinical suspicion of Sturge-Weber syndrome based on physical and neurological examinations.
In addition to Sturge-Weber syndrome, this category includes such rare entities as Klippel-Trenaunay syndrome (CM + VM +/- LM + limb overgrowth), Proteus syndrome (CM, VM and/or LM + asymmetric somatic overgrowth) and Maffucci syndrome (VM +/- spindle cell hemangioma + enchondroma).
Brain CT which was performed with the pre-diagnosis of Sturge-Weber syndrome revealed calcifications in the involved hemisphere (Figure 2b).
Pathophysiology of Sturge-Weber syndrome. J Child Neurol 2003;18:509-516.
Pathophysiology, diagnosis, and management of glaucoma associated with Sturge-Weber syndrome. Int Ophthalmol 2017;[Epub ahead of print].
Sturge-Weber syndrome (SWS), or encephalotrigeminal angiomatosis, is a rare, congenital neurocutaneous syndrome characterized by unilateral facial cutaneous vascular malformation (nevus flammeus or port-wine stain [PWS]) in association with ipsilateral leptomeningeal angiomatosis.
Sturge-Weber syndrome is a sporadic, congenital neurocutaneous syndrome, characterised by the association of ipsilateral facial angioma in the distribution of the trigeminal nerve with angiomatosis of the leptomeninges.
Annie, from Barkisland, has a rare neurological condition called Sturge-Weber Syndrome which causes her to suffer several epileptic seizures every day.
Yasmin suffers from Sturge-Weber syndrome, a rare condition affecting the skin, brain and eyes.
The eight-year-old suffers from rare Sturge-Weber syndrome, which affects the skin, eyes and brain.
Differential diagnosis to be considered in a patient with cerebral hemiatrophy include Sturge-Weber syndrome, Rasmussen encephalitis, Silver-Russel syndrome, and basal ganglia germinoma.
Heterochromia iridis and pertinent clinical findings in patients with glaucoma associated with sturge-weber syndrome. J Pediatr Ophthalmol Strabismus.