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.

Author: Dimi Grotaur
Country: Saint Kitts and Nevis
Language: English (Spanish)
Genre: Technology
Published (Last): 2 January 2004
Pages: 278
PDF File Size: 19.41 Mb
ePub File Size: 1.17 Mb
ISBN: 274-9-35567-624-4
Downloads: 30685
Price: Free* [*Free Regsitration Required]
Uploader: Vukree

Sturge-Weber is an embryonal developmental anomaly resulting from errors in mesodermal and ectodermal development.

Syndrome de Sturge-Weber – EM|consulte

Personal information regarding our website’s visitors, including their identity, is confidential. The birthmark can vary in color from 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.

Congenital neurocutaneous syndromes of childhood III. Congenital disorders of eye, ear, face and neck Congenital disorders of nervous system Genodermatoses Rare syndromes Syndromes affecting the skin Syndromes affecting the nervous system Syndromes with mental retardation. Although we did not see an angiomattose in the 10 children with repeated EEGs, this number may be too small to replicate the findings from Chao Chao et al, Brain surgery involving removing the portion of the brain that is affected by the disorder can be successful in controlling the seizures so that the patient has only a few seizures that are much less intense than pre-surgery.


See other webwr in PMC that cite the published article.

A case of partial epilepsy, apparently due to a lesion of one of the vaso-motor centers of the brain. SWS neuroscores were obtained in 27 children Kelley et al. Genetic counseling The disease is sporadic. Sturgr within the two age subgroups, an association between age and SWS seizure subscore did not reach significance, with a mean age of 6.

EEG Evolution in Sturge-Weber Syndrome

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. The prognosis depends on the severity of the epileptic crises which may lead to variable degrees of psychomotor regression and intellectual disability. Create a free personal account to download free article PDFs, sign up for alerts, customize your interests, and more.

We hypothesized that the EEG evolution over time first reported by Chao over 50 years ago was accurate and could be replicated in a larger series of children with SWS.

Log in Sign up. QuainBS, Joshua B. Doctors recommend early monitoring for glaucoma, and surgery may be performed on more serious cases. Parents ahgiomatose subjects consented to have their information included in a single database.

Contraindications include a history of CME, epiretinal membrane formation, vitreous loss during cataract surgery, history of macular edema associated with branch retinal vein occlusion, history of anterior uveitiswwber diabetes mellitus. Sturge-Weber syndrome is a rare syndrome, with an incidence estimated at 1 case in 20, persons Cephalic form of neurocutaneous hemangiomatosis. Normally, this network goes away in the ninth week of development. Sturge-Weber syndrome was first described by Sturge infollowed by Weber and Volland in who described the intracranial calcification.


Create a free personal strge to make a comment, download free article PDFs, sign up for alerts and more. Infobox medical condition new Commons category link from Wikidata.

EEG Evolution in Sturge-Weber Syndrome

Inthe mission was expanded to include individuals with capillary vascular birthmarks, Klippel Trenaunay KT and Port Wine Birthmarks. Electroencephalographic evaluation in Sturge- Weber syndrome.

This term is derived from the Greek root [unk], which means “mother spot” or “mole” and implies a congenital factor. Epidemiology Clinical presentation Pathology Radiographic features Treatment and prognosis History and etymology Differential diagnosis Related articles References Images: Neurological symptoms include seizures that begin in infancy and may worsen with age.


J Magn Reson Imaging. Outline Masquer le plan.

Sujansky E, Conradi S. EEG scores were assigned by an investigator blinded to age, neuroscores, and seizure activity of the subjects.

Educational therapy is often prescribed for those with mental retardation or developmental delays, but there is no complete treatment for the delays. The facial capillary malformation classically referred to as angioma is a port-wine stain PWS that is generally present at birth and located on the forehead or upper eyelid on one or both sides of the face.