| ... also known as encephalotrigeminal angiomatosis ... rare ... disorder ... arteriovenous malformation on one side of the brain ... present at birth ... port-wine stains of the face, glaucoma, seizures, mental retardation, and an ipsilateral leptomeningeal angioma ... rarely affects other organs in the body ... port-wine stain is the most striking skin characteristic ... deeply dilated capillaries ... produce reddish purplish discoloration ... found around the ophthalmic branch of the trigeminal nerve ... seen over the eye and forehead on the same side as the arteriovenous malformation ... start at birth and progressively get worse ... convulsions usually happen on the side opposite the lesion ... severity varies ... developmental delays ... can vary ... glaucoma at a very young age ... is common ... bulging of the eye ... Laser treatment can be beneficial ... |
Sturge - Weber - Dimitri syndrome
Charles E. Kahn, Jr., MD, September 1, 2006
 "... intended to provide quick reference information to medical professionals and students ... last updated in September of 2006."
Encephalotrigeminal angiomatosis … leptomeningeal capillary-venous angiomatosis … ipsilateral port-wind stains (venous angioma) of face … ipsilateral congenital glaucoma … contralateral focal sz (90%) + hemiparesis (33-66%) … subnormal mentation (50%).
Sturge - Weber Disease
Prof. Katalin Hegedüs,
February 17, 2001
"... for professionals. "
Encephalotrigeminal angiomatosis - illustrations of face and microscopic ...
Interessengemeinschaft Stureg-Weber-Syndrom (Germany)
[Support Group] "Excellent resource. "
The clinical features of the SWS are prot-wine nevus, leptomeningeal angioma, seizures, hemiparesis and mental retardation. Ocular complications include glaucoma, buphthalmos, choroidal angiomas and optic atrophy. The malformations of the meningeal vessels often cause seizures, which can difficultly be controlled (epilepsy) accompanied by hemiparesis relatively well compensated by the children.
The Sturge - Weber Foundation
2007
[Support Group] "Excellent resource. "
Glaucoma can be managed … About Epilepsy … Become educated about seizures … About Klippel Trenaunay Syndrome … KT is often misunderstood.
Sturge - Weber Syndrome Support Group of New Zealand
April 9, 2002
[Support Group] "Good support group resource. "
National Institue of Neurological Disorders and Stroke - Sturge Weber
February 14, 2007
"... basic information ... helpful list of organizations ... "
Sturge-Weber Syndrome Community
2007
[Support Group]"Excellent support group website."
A worldwide community for individuals and families living with Sturge-Weber Syndrome.
Sturge-Weber Syndrome
U.S. National Library of Medicine, November 21, 2005
"... information on symptoms and treatment along with illustrations. "
Sturge-Weber Syndrome
Medically reviewed by Dr Rob Hicks December 2005
"... helpful article from the BBC ... "
Sturge-Weber syndrome was named after two doctors, William Allen Sturge and Frederick Parkes Weber, who discovered this rare disease over 100 years ago...
Sturge-Weber Foundation UK
"British support group. "
Sturge Weber syndrome is a rare neurological disorder of unknown incidence and origin. It is NOT thought to be hereditary. Sturge Weber is usually indicated by a birthmark(port wine stain) somewhere on the face, usually involving the eye and forehead, also similar blood vessels on the brain, called an angioma. Those who suffer from SW often suffer from epilepsy, hemiplegia (a weakness or stiffness affecting one side of the body, similar in appearance to that of a stroke), glaucoma and learning difficulties. Symptoms usually occur in the child`s first year of life.
Hunter Nelson Sturge-Weber Center
Kennedy Krieger Institute, 2007
"... treatment of Sturge-Weber Syndrome."
The Hunter Nelson Sturge-Weber Center at Kennedy Krieger Institute is dedicated to caring for patients with Sturge-Weber syndrome (SWS)...
Sturge-Weber Syndrome
Children's Hospital & Regional Medical Center Seattle, Washington, 2007
"... treatment of Sturge-Weber Syndrome."
The most apparent sign of SWS is a birthmark or port wine stain (PWS) on the face. The PWS is due to an overabundance of capillaries just beneath the surface of the involved areas.
Sturge - Weber Syndrome
Johns Hopkins University, February 6, 2004
[for Professionals mainly]
This condition, sometimes called the fourth phacomatosis, is characterized by nevus flammeus of the face and angioma of the meninges ... Unlike the other phacomatoses--tuberous sclerosis, neurofibromatosis, and von Hippel-Lindau disease--no clear evidence of heredity has been discovered … Sujansky and Conradi (1995) reviewed the outcome in 52 adults … The distribution of port-wine stains (cranial 98%, extracranial 52%) and the prevalence of glaucoma (60%), seizures (83%), neurologic deficit (65%), and other complications were established. The age of onset of glaucoma varied from birth to 41 years and the age of onset of seizures from birth to 23 years. In those with and without seizures, the prevalence of developmental delay (43% vs 0%), emotional and behavioral problems (85% vs 58%), special education requirements (71% vs 0%), and employability (46% vs 48%) was determined.
Neurologie: Les dossiers classés en "Sturge Weber"
[French]"... for professionals. "
Brain: Sturge Weber, Brain, CT, Brain, Calcification.
Sturge - Weber syndrom (Denmark)
[Support Group][Danish]"Good support group link. "
Sturge-Weber syndrom er en medfødt sygdom, som er ret sjælden I Danmark. Formodentlig er der færre end 20-30 personer med syndromet i Danmark.
Norsk Forening for Sturge - Weber syndrom (Norway)
[Support Group][Norwegian]
De Nederlandse Sturge - Weber - Vereniging (Holland)
[Support Group][Dutch]
________________________________________________________________________________________________
Last Updated: 2007/10/10
________________________________________________________________________________________________
| 
