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Sturge–Weber syndrome or Sturge–Weber–Krabbe disease, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and. Sturge-Weber syndrome, or encephalotrigeminal angiomatosis, is a phakomatosis characterised by facial port wine stains and pial angiomas. It is part of a wide. Combined Sturge-Weber-Dimitri and Klippel-Trénaunay-Weber .. Liaras, H.: Un cas de syndrome de Klippel-Trénaunay avec angiomatose osseuse localisée.

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This type is only diagnosed via brain scan.

Sturge-Weber syndrome | Radiology Reference Article |

It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucomaseizuresmental retardationand ipsilateral leptomeningeal angioma cerebral malformations and tumors. Support Radiopaedia and see fewer ads. Genetic counseling The disease is sporadic. CT and MRI are most often used to identify intracranial abnormalities.

This reduces the amount of oxygen and blood flowing to the brain, which can affect brain tissue development. Register for email alerts with links to free full-text articles Access PDFs of free articles Manage your interests Save searches and receive search alerts. Diagnosis is confirmed by imaging techniques radiography, CT scan or MRI with contrast showing, as the disease progresses, ipsilateral cerebral hemiatrophy, cortical calcifications delineating the cerebral gyri, and leptomeningeal angiomatosis.

Srurge, there has angkomatose a paucity of dermatologic writing on the Sturge-Weber syndrome. Sturge Weber Syndrome can be classified into three different types.

The Hemispherectomy Foundation was formed in to assist families with children who have Sturge—Weber syndrome and other conditions that require hemispherectomy.

Privacy Policy Terms of Use. Glaucoma can be expressed as leukocoriawhich should include also further evaluation for retinoblastoma. Cathy Bachur, Kennedy Krieger Institute.


Type 1 includes facial and leptomeningeal angiomas as well as the possibility of glaucoma or choroidal lesions. J Magn Reson Imaging.


Quantitative analysis of cerebral cortical atrophy and correlation with clinical severity in unilateral Sturge-Weber syndrome.

This sturte is the most common.

Prior to the availability of MRI, EEG was also able to help demonstrate asymmetry, with the area of focal slowing corresponding to the leptomeningeal angioma. Although it sturgf possible for the birthmark and atrophy in the cerebral cortex to be present without symptoms, most infants will develop convulsive seizures ed their first year of life. Case 11 Case Focal resection or hemispherectomy should be investigated when medical management fails to control the seizures.

Sturge–Weber syndrome

References Andriola M, Stolfi J. Top of the page – Article Outline. The birthmark can vary in color angiomatlse light pink to deep purple and is caused by an overabundance of capillaries around the ophthalmic branch of the trigeminal nervejust under the surface of the face.

QuainBS, Joshua B. Type I Type II. Archived from the original on March 29, Contributor Information Eric H.

The charity exists to support those affected by Sturge Weber syndrome, promote research into the condition and raise awareness of the condition amongst both public and professionals.

Latanoprost Xalatana prostaglandinmay significantly reduce IOP intraocular pressure in patients with glaucoma associated with Sturge—Weber webe. Create a free personal account to make a comment, download free article PDFs, sign up for alerts and more.

Sujansky E, Conradi S. There is no evidence of brain involvement. Eye involvement can occur at anytime but is generally observed during infancy and young adulthood.

Journal page Archives Contents list. Sturge-Weber syndrome was first described by Wsber infollowed by Weber and Volland in who described sturte intracranial calcification.

Two children were excluded due to lack of consent for research analysis. In some ways this is similar to what has been reported in the EEG of children with Rett syndrome over time Hagne et al, This may represent the somewhat variable nature of this particular outcome measure. Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account.


Prognosis The prognosis depends on the severity of the epileptic crises which may lead to variable degrees of psychomotor regression and intellectual disability. Author information Copyright and License information Disclaimer.

Sturge–Weber syndrome – Wikipedia

Create a free personal account to access your subscriptions, sign up for alerts, and more. Butterworth 3 reported the case of an epileptic idiot, a woman aged 20, with angiomatosis of the brain and a vascular nevus of the trigeminal area of the skin. Access to the text HTML. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.

Quain, Eastern Virginia Medical School. The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties. Health care resources for this disease Expert centres Diagnostic tests 10 Patient organisations 65 Orphan drug s 0.

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A note on the association of extensive hemangiomatous naevus of the skin with cerebral meningeal hemangioma, especially cases of facial vascular naevus with contralateral hemiplegia.

Encephalotrigeminal angiomatosis Sturge-Weber disease: It supports affected individuals and their families with education, advocacy, and research to promote effective management and awareness. Infobox medical condition new Commons category angiomarose from Wikidata.