21. EMedicine - Sturge-Weber Syndrome : Article By Monte A Del Monte, MD sturgeweber syndrome. Background sturge-weber syndrome (SWS) belongs to a groupof disorders collectively known as the phakomatoses ( mother-spot diseases). http://www.emedicine.com/oph/topic348.htm

(advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Ophthalmology Phakomatoses Sturge-Weber Syndrome Last Updated: July 2, 2001 Rate this Article Email to a Colleague Synonyms and related keywords: encephalotrigeminal hemangiomatosis, SWS AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Clinical Differentials ... Bibliography Author: Monte A Del Monte, MD , Director of Pediatric Ophthalmology and Strabismus, Skillman Professor, Department of Pediatrics and Communicable Diseases, WK Kellogg Eye Center, University of Michigan Medical School Monte A Del Monte, MD, is a member of the following medical societies: Alpha Omega Alpha American Academy of Ophthalmology American Association for Pediatric Ophthalmology and Strabismus American Diabetes Association ... American Medical Association , and Association for Research in Vision and Ophthalmology Editor(s): Gerhard W Cibis, MD , Director of Pediatric Ophthalmology Service, Clinical Professor, Department of Ophthalmology, University of Kansas; Director, Clinical Professor, Department of Ophthalmology, Children's Mercy Hospital, University of Missouri at Kansas City;

22. EMedicine - Sturge-Weber Syndrome : Article By James J Riviello, Jr, MD sturgeweber syndrome. Last Updated October 12, 2001, Table 1. ClinicalManifestations of sturge-weber syndrome Risk of SWS with facial PWS, 8%. http://www.emedicine.com/neuro/topic356.htm

document.write(''); (advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Neurology Pediatric Neurology Sturge-Weber Syndrome Last Updated: October 12, 2001 Rate this Article Email to a Colleague Synonyms and related keywords: encephalotrigeminal angiomatosis, encephalofacial angiomatosis, Sturge-Weber-Dimitri syndrome AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Clinical Differentials ... Bibliography Author: James J Riviello, Jr, MD , Director of Epilepsy Program, Associate Professor, Department of Neurology, Division of Epilepsy and Clinical Neurophysiology, Children's Hospital, Harvard University Medical School James J Riviello, Jr, MD, is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics American Clinical Neurophysiology Society American Epilepsy Society , and Child Neurology Society Editor(s): Robert Baumann, MD

23. Epilepsy Action: Sturge-Weber Syndrome sturgeweber syndrome. The sturge-weber syndrome is usually a spasmodicoccurrence - in other words it does not run in families. Very http://www.epilepsy.org.uk/info/sturgfrm.html

HOMEPAGE EMAIL NEWSLETTER EPILEPSY INFORMATION EPILEPSY ACTION SERVICES ... ONLINE SHOPPING Epilepsy Action is the working name of British Epilepsy Association Sturge-Weber Syndrome The Sturge-Weber Syndrome (SWS) is a rare disease in which the presence of a birth mark (port wine stain), usually on one side of the face, is associated with an abnormality of the brain caused by abnormal blood vessels on the surface of the brain. This abnormality usually results in seizures or other problems. Sturge-Weber Syndrome is usually a spasmodic occurrence - in other words it does not run in families. Very rarely one or more family members may be affected. Symptoms The first abnormality noted in Sturge-Weber Syndrome is the birth mark which is present at birth. This is flat and deep red. It is due to abnormalities of the very small blood vessels in the skin. The most common site for the birth mark is the forehead on one side of the face, often coming down on to the cheek, nose or upper lip. Sometimes both sides of the face are involved and on occasions similar birth marks are present on other parts of the body. Seizures develop in nearly all patients with Sturge-Weber Syndrome. They may start at birth or later in the first year of life. They are usually partial motor seizures involving jerks of one side of the body only. The seizures may become generalised and evolve into other types such as drop attacks, myoclonic or infantile spasms. The seizures are often very frequent and prolonged seizures may occur.

24. MEDLINEplus Medical Encyclopedia: Sturge-Weber Syndrome sturgeweber syndrome. Alternative names Return to top Encephalotrigeminalangiomatosis; Sturge-Weber-Dimitri syndrome. Definition http://www.nlm.nih.gov/medlineplus/ency/article/001426.htm

Skip navigation Medical Encyclopedia Other encyclopedia topics: A-Ag Ah-Ap Aq-Az B-Bk ... Z Sturge-Weber syndrome Contents of this page: Illustrations Alternative names Definition Causes, incidence, and risk factors ... Prevention Illustrations Sturge-Weber syndrome - legs Sturge-Weber syndrome - soles of feet Circulatory system Alternative names Return to top Encephalotrigeminal angiomatosis; Sturge-Weber-Dimitri syndrome Definition Return to top A rare and sporadically occurring disease without a recognized hereditary component. It consists of multiple vascular lesions (angiomas) involving the skin of the face, mucus membranes, and covering of the brain (meninges). Causes, incidence, and risk factors Return to top The cause of Sturge-Weber is unknown. Because of its spotty appearance in the population, specific risk factors are not yet recognized. Symptoms Return to top port-wine stain more commonly on the face than the body convulsions seizure disorder visual problems (hemianopsia) paralysis (hemiparesis-paralysis on one side) learning disabilities glaucoma Signs and tests Return to top A skull X-ray shows damage to the inner layer of the skull caused by the vascular lesion and intracranial calcifications . Increased pressure in the eyeball or glaucoma is found in up to 60% of patients. MRI or CT scans of the skull may show benign tumors of the lining of the brain (dural angiomas) or of the choroid plexus. Treatment Return to top Treatment is based on the findings: Seizures are treated with antiseizure medications such as phenobarbital, Dilantin, or carbamazepine. Occasionally

25. MEDLINEplus Medical Encyclopedia: Sturge-Weber Syndrome - Legs Medical Encyclopedia. sturgeweber syndrome - legs. Occasionally seizuresor learning disorders are also associated with sturge-weber syndrome. http://www.nlm.nih.gov/medlineplus/ency/imagepages/2536.htm

Skip navigation Medical Encyclopedia Sturge-Weber syndrome - legs Sturge-Weber syndrome is a disease that affects the skin and nervous system (neurocutaneous) and is associated with Port Wine Stain, red vascular markings on the face and other parts of the body (shown here on the legs). This is an unusual case, due to the large size of the lesion (extensive involvement). Occasionally seizures or learning disorders are also associated with Sturge-Weber syndrome. Health Topics Drug Information Encyclopedia Dictionary ... National Institutes of Health Page last updated: 10 January 2003

26. Sturge-Weber Syndrome - Online Neuropathology Atlas Sturge Weber - Dimitri Disease (Encephalotrigeminalangiomatosis) Angiomatosis encephalotrigeminalis. http://www.neuropat.dote.hu/stuge.htm

Sturge -Weber - Dimitri Disease (Encephalotrigeminal angiomatosis) Angiomatosis encephalotrigeminalis Naveus flammeus Microscopic appearance Microscopic appearance (Click on any image for larger view) Related Internet links Sturge-Weber Syndrome_Discussion Sturge-Weber syndrome - PedBase var site="sm5hegeduskdote" Last modified: February 17, 2001 If you have any comments, please, contact Neuroanatomy Structures Online Neuropathology Atlas Internet Handbook of Neurology

27. Virtual Children's Hospital: Paediapaedia: Sturge-Weber Syndrome Paediapaedia Neurological Diseases sturgeweber syndrome. MichaelP. D'Alessandro, MD Peer Review Status Internally Peer Reviewed http://www.vh.org/pediatric/provider/radiology/PAP/NeuroDiseases/SturgeWeber.htm

Paediapaedia: Neurological Diseases Sturge-Weber Syndrome Michael P. D'Alessandro, M.D. Peer Review Status: Internally Peer Reviewed Clinical Presentation: A cutaneous vascular nevus is seen usually along the distribution of the trigeminal nerve. Symptoms consist of seizures, contralateral hemiparesis, hemiatrophy, glaucoma, mental retardation, and abnormal behavior. Etiology/Pathophysiology: Small, thin walled angiomatous vessels in the pia of the cerebral convexities cause pericapillary calcification of the underlying cortex and the ipsilateral cutaneous vascular nevus (port wine flame nevus). Pathology: Small thin walled angiomatous vessels in the pia of the cerebral convexities are associated with pericapillary calcification in the underlying cerebral cortex. Imaging Findings: Calcification is usually unilateral, asymmetric, and curvilinear and seen most frequently in the posterior parietal and occipital regions. Brain atrophy on the side of the angioma is seen with compensatory calvarial thickening, a small hemicranium, ventriculomegaly and wide subarachnoid spaces. DDX: Gyral Calcification Treated CNS leukemia Post meningoencephalitis Oligodendroglioma References: See References Chapter.

28. Sturge-Weber Syndrome angiomatosis). SturgeWeber-Dimitri syndrome; sturge-weber syndrome;sturge-weber syndrome; sturge-weber syndrome Internet Mailing List; http://www.bdid.com/sturge.htm

HOME Sturge-Weber(-Dimitri) Syndrome (Encephalo-facial angiomatosis) Sturge-Weber-Dimitri syndrome Sturge-Weber Syndrome Sturge-Weber Syndrome Sturge-Weber Syndrome Internet Mailing List ... HOME

29. Sturge-Weber Syndrome What is sturgeweber syndrome? Information from the British Epilepsy Associaion. Outcomeof sturge-weber syndrome in 52 adults (abstract). Link Lists. http://www.nhbvi.com/internet/Eye/sturge-weber.html

Search: What is Sturge-Weber Syndrome? Information from the British Epilepsy Associaion Information from Contact a Family Information from the Medical College of Wisconsin Information from the National Institute of Neurological Disorders and Stroke ... Information from SpecialChild.com Discussion, Publications, and Support Sturge-Weber Foundation Sturge-Weber Foundation (UK) Sturge-Weber Support Group of New Zealand Sturge-Weber_Syndrome Listserv at Yahoo! Groups ... SWFYA Listserv at Yahoo! Groups Research and Treatment "Outcome of Sturge-Weber syndrome in 52 adults" (abstract) Link Lists Family Village Links from Stephie's Corner Yahoo! Personal Web Pages Breezy's Page Jordon's Story Nicole's Page Our Daily Lives with SWS ... Stephie's Corner

30. NINDS Sturge-Weber Syndrome Information Page More about NINDS sturgeweber syndrome Information Page. Content for thispage. NINDS sturge-weber syndrome Information Page. Synonym http://accessible.ninds.nih.gov/health_and_medical/disorders/sturge_doc.htm

Disorders - National Institute of Neurological Disorders and Stroke (NINDS) Skip menus Main sections of the NINDS web site Home About NINDS Disorders-you are in this section ... Find People Disorders section pages and search Image Description Science For the Brain The nation's leading supporter of biomedical research on disorders of the brain and nervous system. Select Topic Disorder Quick Links Alzheimer's Autism Cerebral Palsy Chronic Pain Epilepsy Headache Multiple Sclerosis Parkinson's Stroke Traumatic Brain Injury More about NINDS Sturge-Weber Syndrome Information Page Studies with patients Research literature Press release NINDS Search (search help) Contact us My privacy NINDS is part of the National Institutes of Health Contact us Content for this page NINDS Sturge-Weber Syndrome Information Page Synonym(s): Encephalotrigeminal Angiomatosis Reviewed 07-01-2001 Get Web page suited for printing Email this to a friend or colleague Table of Contents (click to jump to sections) What is Sturge-Weber Syndrome? Is there any treatment? What is the prognosis? What research is being done? ... Organizations What is Sturge-Weber Syndrome? Is there any treatment?

31. Sturge Weber Syndrome - Nicole Nicole Danielle. This site is dedicated to Nicole Danielle. She was diagnosedin October '98 with a rare brain disease called sturgeweber syndrome. http://www.nbtexas.com/nicole/

Nicole Danielle This site is dedicated to Nicole Danielle. She was diagnosed in October '98 with a rare brain disease called Sturge-Weber Syndrome. Our hope with this site is to share her story and ours with those who may have recently been faced with this diagnosis. Please be patient I want to do a journal on this site to share with others what our life with Sturge-Weber Syndrome has been like since October 6, 1998. This site comes from two parents who only want to help others deal with this disease the best way possible. ABOUT STURGE-WEBER SYNDROME Sturge-Weber Syndrome (encephelotrigeminal angiomatosis) is a congenital, non-familial disorder of unknown incidence and cause. It is characterized by a congenital facial birthmark and neurological abnormalities. Other symptoms associated with Sturge-Weber can include eye and internal organ irregularities. Each case of Sturge-Weber Syndrome is unique and exhibits the characterizing findings to varying degrees. Nicole's Diary (I have much to add since the last entry so please be patient. Nicole had her VNS implant done and I have much to share.)

32. Hopkins Sturge-Weber Syndrome Center The Johns Hopkins Kennedy Krieger sturge-weber syndrome Center Whatis sturge-weber syndrome? Dermatologic Features; Neurologic http://www.neuro.jhmi.edu/HopkinsSWSCenter/sitemap.htm

The Johns Hopkins - Kennedy Krieger Sturge-Weber Syndrome Center What is Sturge-Weber Syndrome? Dermatologic Features Neurologic Features Ophthalmologic Features ... Eric Kossoff, MD Doris Lin, MD, PhD Carlos Pardo, MD Camerson Parsa, MD Melissa Trovato, MD Marion Woefel Contact the Sturge-Weber Syndrome Center Glossary Related Links The Johns Hopkins University Dermatology Image Atlas ... Hopkins Medicine Website

33. Hopkins Sturge-Weber Syndrome Center sturgeweber syndrome sturge-weber syndrome (SWS) is a disorder involving the associationof a facial port-wine stain with glaucoma and abnormal blood vessels http://www.neuro.jhmi.edu/HopkinsSWSCenter/glossary.htm

Epilepsy Epilepsy refers to the occurrence of repeated seizures not triggered by a specific acute stimulus such as fever or head trauma. Seizures eventually develop in about 80% of patients with SWS, most commonly in the first year or two of life. Glaucoma Glaucoma is vision loss resulting usually from increased pressure in the eye. Glaucoma occurs in 30-70% of patients with SWS. Leptomeningeal angioma Leptomeningeal angioma is the presence of numerous abnormally dilated blood vessels on the surface of the brain. These abnormally formed blood vessels frequently result in abnormal blood flow (perfusion) to the involved side of the brain. Impaired brain perfusion, in turn, can result in brain injury, seizures, stroke-like episodes and developmental delays. Port-wine Stain Port-wine stains (PWS) are the most common kind of abnormality of blood vessel development and involves dilated capillaries in the skin. PWS may occur anywhere on the body, however the risk of brain or eye involvement is very low if the PWS is located anywhere other than the upper face. Sturge-Weber Syndrome Sturge-Weber Syndrome (SWS) is a disorder involving the association of a facial port-wine stain with glaucoma and abnormal blood vessels called a leptomeningeal angioma on the surface of the brain. The facial port-wine stain is present from birth and when it ocurrs on the forehead and/or upper eyelid (ophthalmic distribution) is associated with an increased risk (10-20%) of the full syndrome. SWS can involve one side of the head (unilateral) or both sides (bilateral). Infants with Sturge-Weber Syndrome are at increased risk of developing, epilepsy, developmental delays, mental retardation, weakness on a side of the body (hemiparesis), and vision loss.

34. Avera Health - Sturge-Weber Syndrome sturgeweber syndrome. Definition Alternative Names Encephalotrigeminal angiomatosis;Sturge-Weber-Dimitri syndrome. Causes, incidence, and risk factors http://www.avera.org/adam/ency/article/001426.htm

Disease Injury Nutrition Poison ... Circulatory system Sturge-Weber syndrome Definition: A rare and sporadically occurring disease without a recognized hereditary component. It consists of multiple vascular lesions (angiomas) involving the skin of the face, mucus membranes, and covering of the brain (meninges). Alternative Names: Encephalotrigeminal angiomatosis; Sturge-Weber-Dimitri syndrome Causes, incidence, and risk factors: The cause of Sturge-Weber is unknown. Because of its spotty appearance in the population, specific risk factors are not yet recognized. Symptoms: port-wine stain more commonly on the face than the body convulsions seizure disorder visual problems (hemianopsia) paralysis (hemiparesis-paralysis on one side) learning disabilities glaucoma Signs and tests: A skull X-ray shows damage to the inner layer of the skull caused by the vascular lesion and intracranial calcifications . Increased pressure in the eyeball or glaucoma is found in up to 60% of patients. MRI or CT scans of the skull may show benign tumors of the lining of the brain (dural angiomas) or of the choroid plexus. Treatment: Treatment is based on the findings: Seizures are treated with antiseizure medications such as phenobarbital, Dilantin, or carbamazepine. Occasionally

35. Avera Health - Sturge-Weber Syndrome - Legs sturgeweber syndrome is a disease that affects the skin and nervous system (neurocutaneous)and is associated with Port Wine Stain, red vascular markings on http://www.avera.org/adam/imagepages/2536.htm

Sturge-Weber syndrome is a disease that affects the skin and nervous system (neurocutaneous) and is associated with Port Wine Stain, red vascular markings on the face and other parts of the body (shown here on the legs). This is an unusual case, due to the large size of the lesion (extensive involvement). Occasionally seizures or learning disorders are also associated with Sturge-Weber syndrome. Acquired Date: 1/1/1997 12:00:00 AM Acquired From: Mosby Publishing Company

36. FAQ - Sturge Weber Syndrome New Zealand Are individuals with sturgeweber syndrome prone to strokes? Individuals Doindividuals with sturge-weber syndrome suffer from Migraines? Many http://www.geocities.com/HotSprings/Spa/1563/faq.htm

Frequently Asked Questions Courtesy of The Sturge-Weber Foundation Was it my fault? Was it something I did during my pregnancy to cause this to happen? No, it wasn't your fault. According to our most recent knowledge of the syndrome, no action, food, drug, or other external influence causes Sturge-Weber Syndrome to occur. The exact cause is unknown but may be related to an error that occurs in one cell during development of the fetus. As that cell then divides, all its "offspring" cells will have the same "mistake". If a cell gets the "mistake" very early in development of the fetus, then there will be a more severe effect (because more derived tissues are involved) than if the error occurs quite late in development. Yet, we don't know why the original mistake in one cell occurs. What are the numbers? How many SW people are there? At this time the total number of people with Sturge-Weber Syndrome is not known. Estimates range between 1 in 400,000 and 1 in 40,000. Since many individuals are not diagnosed with the syndrome, it is very difficult to obtain a good estimate. Similarly, few agencies or health care systems keep statistics on this condition, confounding efforts to create a meaningful guess of how common SWS is in the general population. At present, there are just over 1,000 families who are members of Foundation. What is the life expectancy for SWS?

37. Entrez-PubMed Click here to read Increased Fibronectin Expression in SturgeWeberSyndrome Fibroblasts and Brain Tissue. Comi AM, Hunt P, Vawter http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1

38. Sturge-Weber Syndrome sturgeweber syndrome. What is it? sturge-weber syndrome is also knownas encephaiofacial angiomatosis. It is a rare, progressive http://www.hmc.psu.edu/childrens/healthinfo/s/sturgeweber.htm

39. Sturge-Weber Syndrome And The Eye sturgeweber syndrome and the Eye. Chandru Krishnan, MD Mandar Joshi, MD. Approximately30% to 40% of patients with sturge-weber syndrome have glaucoma. http://www.ophthalmic.hyperguides.com/Tutorials/glaucoma/Sturge_Weber_Syndrome/t

You've spent minutes on Ophthalmic Hyperguide Sturge-Weber Syndrome and the Eye Chandru Krishnan, MD Mandar Joshi, MD Introduction Epidemiology Glaucoma Treatment ... References Introduction Sturge-Weber syndrome is one of several syndromes classified under the heading of phakomatosis because it involves pathology in the skin, eye, and brain. The first reported description of Sturge-Weber syndrome associated with eye involvement has been attributed to Schirmer in 1860. He reported a patient with facial nevus flammeus and ipsilateral buphthalmos. Sturge later reported on a patient who had these findings, along with neurologic findings, and proposed that there were vascular abnormalities in the brain and eye along with the skin. Weber verified neurologic involvement radiologically. Inheritance of disease is sporadic and eye involvement is almost exclusively unilateral. It is believed that men and women are equally affected. Histopathology is significant for hamartomatous malformation of cephalic blood vessels causing hemangiomas of the skin, meninges, and eye. A layer of endothelium lines these dilated blood vessels. Typically, the lesions remain constant throughout life. Calcification of brain tissues adjacent to hemangiomas causes seizures, mental retardation, hemiparesis, and hemianopia. The facial lesion (port-wine stain) occurs along a distribution similar to the branches of the trigeminal nerve. However, the hemangioma often traverses the midline ( Slide 1 Slide 1 Ocular manifestations include choroidal, episcleral, and conjunctival hemangiomas, glaucoma, and heterochromia of the iris

40. Sturge-Weber Syndrome And The Eye sturgeweber syndrome and the Eye. Chandru Krishnan, MD Mandar Joshi, MD. Slide1, Slide 1. Port-wine stain associated with sturge-weber syndrome. Slide 2A, http://www.ophthalmic.hyperguides.com/Tutorials/glaucoma/Sturge_Weber_Syndrome/s

You've spent minutes on Ophthalmic Hyperguide Sturge-Weber Syndrome and the Eye Chandru Krishnan, MD Mandar Joshi, MD Click on each slide to view a larger image. Slide Description Slide 1. Port-wine stain associated with Sturge-Weber syndrome. Slide 2A. Homonymous quadrantanopia demonstrated by visual field. Slide 2B. Homonymous quadrantanopia demonstrated by visual field. Slide 3. Dilated episcleral vessels in a patient with Sturge-Weber syndrome.

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