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International Birth Defects Information Systems
Sturge - Weber - Dimitri

International Birth Defects Information Systems



Sturge - Weber - Dimitri


Topics: | Encephalotrigeminal Angiomatosis | Fourth Phacomatosis | Leptomeningeal Capillary - Venous Angiomatosis | Sturge Weber Dimitri Syndrome |

Related Topics : | Asperger Syndrome | Disabilities | Birth Defects | Fetal Alcohol Syndrome |

Service Related: | Support Groups | Professional Associations | Key Information Sources |

Languages: | Danish | Dutch | English | French | Norwegian | Spanish |

Notes per Visitors  
... 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
Visitor Comments "... 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%).

Interessengemeinschaft Stureg-Weber-Syndrom (Germany)
Visitor Comments [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
Visitor Comments [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
Visitor Comments [Support Group] "Good support group resource. "

National Institue of Neurological Disorders and Stroke - Sturge Weber
February 14, 2007
Visitor Comments "... basic information ... helpful list of organizations ... "

Sturge-Weber Syndrome Community
2007
Visitor Comments [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
Visitor Comments "... information on symptoms and treatment along with illustrations. "

Sturge-Weber Syndrome
Medically reviewed by Dr Rob Hicks December 2005
Visitor Comments "... 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
Visitor Comments "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
Visitor Comments "... 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
Visitor Comments "... 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
Victor A. McKusick, OMIM, Johns Hopkins University, April 3, 2008
Visitor Comments [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"
Visitor Comments [French] "... for professionals. "
Brain: Sturge Weber, Brain, CT, Brain, Calcification.

Síndrome de Sturge Weber, Revisión de la Literatura y Presentación de un Caso con Maloclusión Severa
Rafael J. Prato G., Sociedad Venezolana de Ortodoncia, March 29, 2005
Visitor Comments [Spanish]
El síndrome de Sturge Weber es una rara alteración vascular con componentes neurocutaneos, en el que se presentan manifestaciones sistémicas y bucales. Entre sus características se incluyen; el nevo cutaneofacial, angioma venoso en las leptomeninges, calcificaciones intracraneales, hemangiomas intrabucales e hiperplasia gingival. El caso presentado se refiere a un paciente que acude a consulta para realizar tratamiento ortodóntico por presentar una maloclusión severa concomitante con asimetría facial. En este trabajo se realizó una extensa revisión de la literatura en relación al síndrome de Sturge Weber y se señalan las alteraciones intra y extraorales que presentó el paciente al realizarse el diagnóstico ortodóntico ... El síndrome de Sturge Weber es un raro desorden neurológico que por lo general exhibe manifestaciones sistémicas y bucales. El signo clínico más característico es la presencia de un nevo en alguna parte de la cara y que suele apreciarse siguiendo una o varias de las ramas del nervio trigémino. En pacientes con el síndrome de Sturge Weber es común observar alteraciones vasculares intracraneales y oculares, así como también la afectación de estructuras intrabucales. Se relata el caso de un paciente que acude a consulta para realizar tratamiento ortodóntico por presentar una maloclusión severa. En este trabajo se realizó una extensa revisión de la literatura en relación al síndrome de Sturge Weber y se señalan las alteraciones intra y extraorales que presentó el paciente al realizarse el diagnóstico ortodóntico ...

Síndrome de Sturge weber. Revisión
Dr. Otman Fernández et al., Rev Cubana Pediatr v.71 n.3 Ciudad de la Habana jul.-set. 1999
Visitor Comments [Spanish]
La angiomatosis encefalotrigeminal (síndrome de Sturge-Weber) es el más frecuente de los síndromes neurocutáneos con predominio de anomalías vasculares. Es una enfermedad congénita, que aparece de manera esporádica y en su forma completa consiste en la asociación de angiomas cerebral, cutáneo y ocular que se caracterizan clínicamente por una mancha color vino en la cara, epilepsia, retraso mental, déficits neurológicos (hemiparesia y hemianopsia) y glaucoma. Para el diagnóstico de la enfermedad resultan de mucha utilidad los estudios de tomografía axial computadorizada y resonancia magnética nuclear craneales, así como para estudiar la relación del angioma leptomeníngeo u atrofia subyacente, importantes para el pronóstico y la conducta médica que se debe seguir. El control de las crisis epilépticas constituye un elemento capital para una evolución favorable de los pacientes. El tratamiento quirúrgico debe ser reservado para enfermos de corta edad, con afectación unilateral exclusiva, con epilepsia refractaria y sin retraso mental profundo; mediante la resección del área afectada, lobectomía o hemisferectomía, siempre que el otro hemisferio esté intacto. Se profundiza en los aspectos clínicos, diagnósticos, patogénicos y terapéuticos de esta enfermedad ... Descriptores DeCS: SINDROME DE STURGE-WERBER/terap/diag; HEMANGLIOMA; EPILEPSIA/terap; MANIFESTACIONES NEUROLOGICAS ...

Sturge - Weber syndrom (Denmark)
Visitor Comments [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)
Visitor Comments [Support Group] [Norwegian]

De Nederlandse Sturge - Weber - Vereniging (Holland)
Visitor Comments [Support Group] [Dutch]

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Last Updated: 2008/6/11

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American Medical Association