21. Dorlands Medical Dictionary encephalofacial angiomatosis, encephalotrigeminal angiomatosis, SturgeWebersyndrome. hepatic angiomatosis, peliosis hepatis. angiomatosis of retina http://www.merckmedicus.com/pp/us/hcp/thcp_dorlands_content.jsp?pg=/ppdocs/us/co

22. EMedicine - Sturge-Weber Syndrome : Article By Ali Nawaz Khan, MBBS, FRCP, FRCR meningofacial angiomatosis, encephalofacial angiomatosis, The choroidplexus may be enlarged ipsilateral to the angiomatosis. http://www.emedicine.com/radio/topic660.htm

(advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Patient Education Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Radiology Pediatrics Sturge-Weber Syndrome Last Updated: March 17, 2003 Rate this Article Email to a Colleague Synonyms and related keywords: SWS, Sturge-Weber-Dimitri syndrome, encephalotrigeminal angiomatosis, trigeminal angiomatosis, meningofacial angiomatosis, encephalofacial angiomatosis, Dimitri's hemoangiomatosis, Jahnke's syndrome (variant without glaucoma), Kulisher's syndrome, Krabbe's II syndrome, Lawford's syndrome (variant with glaucoma and without increased ocular pressure), meningocutaneous syndrome, neurooculocutaneous syndrome, Parkes Weber's phacomatosis, vascular encephalotrigeminal Weber-Dimitri syndrome AUTHOR INFORMATION Section 1 of 11 Author Information Introduction Differentials X-ray ... Bibliography Author: Ali Nawaz Khan, MBBS, FRCP, FRCR , Consultant Radiologist, Department of Diagnostic Radiology, North Manchester General Hospital Coauthor(s): Ian Turnbull, MD

23. EMedicine - Glaucoma, Secondary Congenital : Article By Inci Irak, MD SturgeWeber syndrome (encephalofacial angiomatosis) The characteristic presentationis a red facial lesion since birth. http://www.emedicine.com/oph/topic141.htm

(advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Ophthalmology Intraocular Pressure Glaucoma, Secondary Congenital Last Updated: February 24, 2005 Rate this Article Email to a Colleague Synonyms and related keywords: developmental glaucoma, open angle, closed angle, vision loss, visual deficit, ocular anomalies, systemic anomalies AUTHOR INFORMATION Section 1 of 11 Author Information Introduction Clinical Differentials ... Bibliography Author: Inci Irak, MD , Fellow, Department of Ophthalmology, Medical Center at University of Utah Inci Irak, MD, is a member of the following medical societies: American Academy of Ophthalmology Editor(s): Andrew I Rabinowitz, MD , Consulting Staff, Department of Ophthalmology, Barnett Delaney Parkins Eye Center; Donald S Fong, MD, MPH , Assistant Clinical Professor of Ophthalmology, Director, Clinical Trials Research, Department of Ophthalmology, Southern California Permanente Medical Group; Martin B Wax, MD

24. CNS Syndromes Associated With Vascular Malformations NeuroLearn SturgeWeber syndrome (encephalotrigeminal or encephalofacial angiomatosis)Extensive Von Hippel s disease is heriditary angiomatosis of the retina. http://moon.ouhsc.edu/kfung/JTY1/NeuroHelp/ZNG0DD02.htm

CNS Syndromes Associated with Vascular Malformations NeuroLearn NeuroHelp Malformations Syndromes that involve the brain ... Syndromes that involve the spinal cord. S yndromes that involve the brain Head Sturge-Weber syndrome (encephalotrigeminal or encephalofacial angiomatosis): Extensive capillary-venous malformation results in unilateral cerebral cortical atrophy associated with angioma on the face which frequently include distribution of the ophthalmic branch of the trigerminal nerve. Angiomas in other tissue including the meninges, mucous membrane, and choroid can also occur. Heriditary mechanism unknown. Von Hippel-Lindau disease This is a combination of of retinal angiomatosis and angioma that is histologically identical to hemangioblastoma, multiple hemangioblastomas (most frequently in the cerebellum), pheochromocytoma, pancreatic cysts and islet cell tumor, renal cysts and bilateral, often multiple renal cell carcinoma of the kidney, and in males, bilateral papillary cystadenoma of the epidydimis, hepatic cyst, and endolymphatic sac tumor in the petrous bone [Kemperm a nn G ... and Neumann HP, 1998

25. Sturge-Weber Syndrome NeuroLearn NeuroHelp Malformations Also known as encephalofacial angiomatosis or encephalotrigerminal angiomatosis.Characteristics congenital angiomatosis which involves the skin of the http://moon.ouhsc.edu/kfung/JTY1/NeuroHelp/ZNG0IE06.htm

Sturge-Weber Syndrome NeuroLearn NeuroHelp Malformations Background ... Gross Pathology BACKGROUND AND CLINICAL INFORMATION Head Also known as encephalofacial angiomatosis or encephalotrigerminal angiomatosis. Characteristics: congenital angiomatosis which involves the skin of the face and cervical area, mucous membranes, meninges, and choroid of the retina. Usually unilateral.The skin lesions ("nevus flammeus" or “port wine stain") usually involves the territories of the sensory branches of the5th nerve, especially the ophthalmic branch. The gross and microscopic pathology depends on the age of the individuals. Genetics: Sporadiac. No known hereditary factor but familial cases have been reported. Clinical: manifestations of cortical damage include convulsions, mental defect, and hemiparesis or hemianopia on the side contra- lateral to the lesion. Symptoms usually onset within the first year of life. Angioma in the choroid may lead to buphthalmos (in 70% of the cases) in infancy or to glaucoma in childhood. Treatment: surgical resection should be considered within the first 6 months of life if the patient has intractable seizure.

26. Glossary Refering to the face and the brain, as in encephalofacial angiomatosis malformation of skin and brain vascular tissue (encephalofacial angiomatosis) http://nanonline.org/nandistance/mtbi/NeuroIll/glossary/glossary.html

Course Home Page Course Syllabus Course Modules Case Studies Resources Neuroanatomy Atlas Neurological Disorders Infectious Disease Cerebral Vascular Disease ... Chronic Pain Glossary Behavioral Neuropsych MRI Scans Assistive Technology Self-Testing ... References Communication WWW Links e-mail Links Message Board NAN NAN Home Page NAN distanCE info@nanonline.org National Academy of Neuropsychology. Neurological Disorders Glossary A B C D ... Z abdominal - Pertaining to the abdomen usually the stomach and gastro-intestinal system abscess - An encapsulated area of infection abscesses - An encapsulated area of infection acromegaly - Excessive secretion of pituitary growth hormone in adults; it causes an abnormal enlargement of the extremities of the skeleton. ACTH - Adrenocorticotropic hormone, which is secreted by the anterior pituitary gland and has a stimulating effect on the adrenal cortex. Acute - Having a sudden onset. acutely - Having a sudden onset. adenoma - A benign epithelial tumor. adiposity - Obesity. AIDS - An immune deficiency illness caused by a virus. akinesia - Absense or poverty of movement.

27. Incomplete Monosymptomatic Leptomeningeal Angiomatosis Kumar Sarita, Muranjan Ma encephalofacial angiomatosis sparing the occipital lobe and without facial nevuson the spectrum of SturgeWeber syndrome variants? J Child Neurol 2003; http://www.ijppediatricsindia.org/article.asp?issn=0019-5456;year=2004;volume=71

28. Definitions Of Genetic Disorders-E. encephalofacial angiomatosis sturge.htm Encephalopathy west.htm EncephalotrigeminalAngiomatosis sturge.htm Enchondromatosis with Multiple Cavernous http://www.icomm.ca/geneinfo/def-e.htm

29. Index E: Contact A Family - For Families With Disabled Children: Information On encephalofacial angiomatosis see SturgeWeber syndrome Enchondromatosis see Ollierdisease Endocardial Fibroelastosis see Metabolic diseases http://www.cafamily.org.uk/Idx/e.html

printer friendly home how we can help medical information ... how you can help Please use the index below to access the condition you require information on. It may take longer to find what you are looking for this way compared with our "search this site" facility in the navigator on the left but we try to point you in the most appropriate direction using this index and therefore the results should be better. For speed, this index has been split into separate alphabetical files: numbers 0-9 A B C ... Z Contact a Family also has information on many other specific conditions and rare disorders. If you cannot find the information you require in The Contact a Family Directory Online , you may wish to use our Contact a Family Helpline service. E.Coli 0157 infection see Haemolytic Uraemic syndrome EB Simplex see Epidermolysis Bullosa EDS see Ehlers-Danlos syndrome EMG syndrome see Beckwith-Wiedemann syndrome Eagle-Barrett syndrome see Prune Belly syndrome Early Infantile Epileptic Encephalopathy see Ohtahara syndrome Eating Disorders Ectodermal Dysplasia Eczema ... Ellis-Van Creveld syndrome Emery-Dreifuss disease see Muscular Dystrophy and neuromuscular disorders Emphysema see Lung diseases Encephalitis Encephalofacial Angiomatosis see Sturge-Weber syndrome Enchondromatosis see Ollier disease Endocardial Fibroelastosis see Metabolic diseases Enthesitis-related Arthritis see Arthritis (Juvenile Idiopathic) Eosinophilic granuloma see Histiocytosis Epidemic Neuromyasthenia see

30. Bilateral Focal Cerebral Angiomatosis Associated With Nervous Signs In A Cat -- encephalofacial angiomatosis (SturgeWeber syndrome) has been reported in humansand in a horse, and proliferative vasculopathy and http://www.vetpathology.org/cgi/content/full/38/3/350

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS This Article Abstract Full Text (PDF) Alert me when this article is cited ... Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager ... Cited by other online articles PubMed PubMed Citation Articles by Kipar, A. Vet Pathol American College of Veterinary Pathologists BRIEF COMMUNICATIONS AND CASE REPORTS Bilateral Focal Cerebral Angiomatosis Associated with Nervous Signs in a Cat A. Kipar U. Hetzel A. G. Armien and Abstract A case of cerebral angiomatosis in a cat was associated with neurologic signs characterized by clusters of severe generalized seizures. Bilaterally in the gray matter, most prominent in the cingulate gyrus, there was focal accumulation of garlandlike arrangements of blood vessels. Vessels exhibited activated, hypertrophic endothelial cells and thickening and progressive dystrophic mineralization of the basement membrane, with complete luminal obstruction of some affected vessels. Thickening of

31. Anne M. Comi, MD Comi, AM, Kossoff EH, and Fischer R. The spectrum of SturgeWeber Syndromevariants encephalofacial angiomatosis sparing the occipital lobe and without http://www.neuro.jhmi.edu/profiles/comi.html

02-Dec-2002 Anne M. Comi, M.D. Dr. Anne Comi has been a member of the Department of Neurology since July 1996. She is an Assistant Professor of Neurology with specialization in Pediatric Neurology. Particular areas of focus are the neurobiology of Sturge-Weber Syndrome (SWS). She is the Director of the Johns Hopkins - Kennedy Krieger Sturge-Weber Syndrome Center and has served on the research board of the National Sturge-Weber Foundation. Dr. Comi's clinical practice expertise includes the evaluation and management of Sturge-Weber Syndrome, epilepsy, migraine and pervasive developmental disorders. Current areas of research are the clinical, molecular, and neuropathologic study of patients with Sturge-Weber Syndrome. Dr. Comi sees patients by referral in the Neurological Outpatient Center on Mondays from 1:00 PM to 5:00 PM. Appointments may be scheduled by referral and by calling her office at 410-614-5807. CURRENT ADDRESS The Johns Hopkins Hospital Department of Neurology Jefferson Building, Room 123

32. Full Listing EMOTIONALLY DISTURBED, EMPHYSEMA, ENCEPHALITIS, encephalofacial angiomatosis,ENCEPHALOMYELITIS MYALGIA, ENCOPRESIS, ENDOCHRONDROMATOSIS, ENDOMETRIOSIS, http://www.doctor.gp/help/full_listing.htm

DIRECTORY OF BRITISH SELF HELP GROUPS AND SUPPORT ORGANISATIONS by Steve and Julie Garrill Home

33. VI. VASCULAR MALFORMATIONS There Are Mour Major Categories Of B. SturgeWeber Syndrome (encephalofacial angiomatosis) This syndrome is associatedwith intracranial venous malformation and hemangioma of the face in the http://kobiljak.msu.edu/CAI/Pathology/Cerebrovascular_F/cerebrovascular_6.html

VI. VASCULAR MALFORMATIONS There are mour major categories of vascular anomalies: arteriovenous malformations (most common), cavernous angiomas, capillary telangiectasias, and venous antiomas. These lesions are found in about 5% of patients at autopsy. They are generally asymptomatic. Clinical manifestations occur most often in young people. In fact, a vascular malformation would be the most likely cause of intracerebral hemorrhage in a child. The arteriovenous angioma is most often clinically significant. A. Arteriovenous Malformations (AVM) 1. Characteristics a. Tangles of abnormal vessels or channels of various sizes with thin walls; vessels are separated by gliotic neural parenchyma b. Developmental origin c. Bleeding most common in 10-30 year age group d. Most often located in cerebral hemispheres Clinical Signs a. Seizuresthe initial sign in 25-50% of cases b. Headachethe initial sign in 15% of patients c. Focal neurologic signsspecific signs related to location of lesion Consequences a. Hemorrhagesubarachnoid or intracerebral b.

34. Turn Of The Century - British Association Of Dermatologists Rare diseases and syndromes which are rightly associated with his name include Sturge-Kalischer-Weber (encephalofacial angiomatosis) Weber-Christian http://www.bad.org.uk/public/history/turn/

About BAD BAD Constitution What is a Dermatologist Access to Care ... Frequently Asked Questions Some Remarkable Clinicians On the whole little interest was taken in promoting dermatological knowledge at this time, nevertheless a few outstanding individuals were to make lasting impressions. In Manchester, Louis Savatard, Brooke's pupil, was meticulously analysing all the various cutaneous malignancies. Louis Savatard (1874-1942) was a pioneer of radiotherapy but it must have been Brooke's interest in pathology that set him on his way; he was to become an acknowledged expert, spending endless hours at his microscope. From 1920 to 1940 he published many papers on superficial and pre-malignant skin changes and his interest in "mulespinner's cancer", led to his appointment to the special Home Office Committee. He virtually founded both the North of England and the Manchester Dermatological Societies and was President of the BAD in 1940. Savatard worked in isolation, but he made Manchester aware of the subject at what was surely a barren period. In London one or two others were now to make a name for themselves and for dermatology.

35. Miscellaneous Applications Of PET Scanning Down s syndrome; Huntington s chorea; Kinkyhair disease (Menkes syndrome);Sturge-Weber syndrome (encephalofacial angiomatosis) and the phakomatoses http://www.regence.com/trgmedpol/radiology/rad35.html

Policies TRG Medical Policy TRG Dental Policy TRG Behavioral Health Policy Radiology Section - Miscellaneous Applications of PET Scanning Topic: Miscellaneous Applications of PET Scanning Date of Origin: Section: Radiology Policy No: Approved Date: Effective Date: Next Review Date: IMPORTANT REMINDER This Medical Policy has been developed through consideration of medical necessity, generally accepted standards of medical practice, and review of medical literature and government approval status. Benefit determinations should be based in all cases on the applicable contract language. To the extent there are any conflicts between these guidelines and the contract language, the contract language will control. The purpose of medical policy is to provide a guide to coverage. Medical Policy is not intended to dictate to providers how to practice medicine. Providers are expected to exercise their medical judgment in providing the most appropriate care. Description Unlike standard radiologic techniques, such as computed tomography (CT) and magnetic resonance imaging (MRI), positron emission tomography (PET) is able to image biochemical reactions and physiological functions. This is accomplished by measuring concentrations of radioactive chemicals that are partially metabolized in the body region of interest.

36. AKAs [Web Review Of Ophthalmology] @ WebEyeMD.com Neurofibromatosis. Von Recklinghausen disease. Von HippelLindau disease.Retinal angiomatosis. Sturge-Weber syndrome. encephalofacial angiomatosis http://webeyemd.com/wro/wro_aka.htm

Back to Home Web Review of Ophthalmology AKAs Age-Related Macular Degeneration (AMD) Age-related maculopathy Senile macular degeneration Dystrophy – any disorder arising from defective or faulty nutrition Granular corneal dystrophy – stroma clear? Groenouw’s type I Macular corneal dystrophy – indistinct irregular borders, intervening cloudy stroma Groenouw’s type II Lattice corneal dystrophy Biber-Haab-Dimmer dystrophy Epithelial dystrophy MDF dystrophy; map-dot-fingerprint dystrophy Cogan’s microcystic dystrophy ABMD; anterior basment membrane dystrophy Grave's Eye Disease Thyroid ophthalmopathy Thyroid orbitopathy Thyroid-related immune orbitopathy (TRIO) Von Basedow eye disease (in European literature) Large cell lymphoma Histiocytic lymphoma (old literature) Reticulum cell sarcoma (old literature) Phacoantigenic endophthalmitis

37. Positron Emission Tomography (PET Scans), 358 Down’s syndrome; Huntington’s chorea; kinkyhair disease (Menkes’ syndrome);Sturge-Weber syndrome (encephalofacial angiomatosis) and the phakomatoses http://www.bcbsma.com/common/en_US/medical_policies/358.htm

Policy: 358, Reviewed: 3/05 PET Scans Positron Emission Tomography For FDG SPECT, see policy 330 When services are covered SEIZURES/ EPILEPSY: We cover PET scans for patients with complex partial seizures when the patient has failed medical therapy and is being evaluated for epilepsy surgery. LUNG CANCER: We cover PET scans to diagnose lung cancer, when all the following are met: The patient has a solitary pulmonary nodule Chest x-ray and CT scans cannot distinguish between benign and malignant disease FDG PET results will change patient management. We also cover PET scans to stage and re-stage lung cancer. HEAD AND NECK CANCER: We cover PET scans to diagnose the following head and neck cancer Unknown primary tumors suspected in the head and neck Initial staging of cervical lymph node metastases Detection of residual or recurrent head and neck cancer. THYROID CANCER: We cover Blue Care 65 members in accordance with CMS (Centers for Medicare and Medicaid Services) guidelines. (Effective October 1, 2003)

38. Scope Of Work Cutaneus angiomatosis with abnormalities of the central nervous system StugeWebersyndrome (meningo- or encephalofacial angiomatosis with cerebral http://www.cochraneneuronet.org/livello2/scope_of_work2.html

Index of Diseases BACK to scope of work ACQUIRED METABOLIC DISORDERS MIGRAINE AND HEADACHE ALCOHOL AND ALCOHOLISM ... OTHERS ACQUIRED METABOLIC DISORDERS back to index Ischemic-Hypoxic encephalopathy Carbon Monoxide Poisoning High-Altitude sickness Hypercapnic pulmonary disease Hypoglycemic encephalopathy Hyperglycemia Hepatic stupor and coma (hepatic or portal-systemic encephalopathy) Uremic encephalopathy Encephalopathy associated with sepsis and burns Disorders of sodium, potassium and water balance Central pontine myelinolysis Chronic acquired (Non-Wilsonian) hepatocerebral degeneration Kernicterus Hypoparathyroidism Cerebellar ataxia associated with myxedema Effects of Hyperthermia on the Cerebellum Cerebellar syndromes associated with celiac-sprue and Jejunoileal bypass Cushing disease and corticosteroid psychoses Thyroid encephalopathies Pancreatic encephalopathy

39. Pages 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 Translate this page encephalofacial angiomatosis, æõÑÇãñ æöÚóÇÆöíøñ ÏöãÇÛöíøñ æóÌúåöíøñ ( ãõÊóáÇÒöãóÉõÓÊíÑÌ- æíÈÑ) º. encephalotrigeminal angiomatosis, æõÑÇãñ æöÚóÇÆöíøñ http://www.emro.who.int/umd/BrowsingDic.asp?PageNo=11&Char=A

40. Maladies Rares, Maladies Orphelines encephalofacial angiomatosis see SturgeWeber Syndrome Enchondromatosis seeOlliers Disease Endocardial Fibroelastosis see Metabolic Diseases http://www.fmo.easynetonline.net/anciensite/listcaf.html

Maladies rares maladies orphelines ATTENTION : VOUS ETES SUR L'ANCIEN SITE DE LA FEDERATION DES MALADIES ORPHELINES Vous pouvez vous diriger vers le nouveau site en cliquant - ici Retour Liste Contact A Family 2 Hydroxglutaric Aciduria see Metabolic Diseases 4 Hydroxybutyric Aciduria see Metabolic Diseases 2-Methylacetoacetyl-CoA Thiolase Deficiency see Metabolic Diseases 3-Hydroxy Acyl CoA Dehydrogenase Deficiency see Metabolic Diseases 3-Hydroxy-Methylglutaril CoA Lyase Deficiency see Organic Acidaemias 3-Methylglutaconic Aciduria see Metabolic Diseases 4p-Syndrome see Wolf-Hirschhorn Syndrome 5 Alpha Reductase Deficiency see Metabolic Diseases 5-Oxoprolinuria see Metabolic Diseases 21 Hydroxylase Deficiency see Congenital Adrenal Hyperplasia aAA see Acquired Aplastic Anaemia ADA see Primary Immune Deficiencies ADD see Attention Deficit Hyperactivity Disorder ADHD see Attention Deficit Hyperactivity Disorder ADL see Adrenoleukodystrophy AIDS see HIV Infection and AIDS AMC see Arthrogryposis ASD see Heart Defects ATR-X see Alpha Thalassaemia/Mental Retardation on the X Chromosome Abdominal Exstrophies Abetalipoproteinaemia see Metabolic Diseases Achondroplasia see Restricted Growth Acne Acne Vulgaris see Acne Acquired Aplastic Anaemia Acrodermatitis Enteropathica see Metabolic Diseases Acrofacial dysostosis see Nager Syndrome Acute Idiopathic Polyneuritis see Guillain-Barré Syndrome and other Neuropathies Acute Inflammatory Polyneuropathy see Guillain-Barré Syndrome and other Neuropathies

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