Extractions: This survey of medical eponyms and the persons behind them is meant as a general interest site only. No information found here must under any circumstances be used for medical purposes, diagnostically, therapeutically or otherwise. If you, or anybody close to you, is affected, or believe to be affected, by any condition mentioned here: see a doctor. Angioma capillare et venosum calcificans, angiomatosis encephalofacialis, cerebrocutaneous angiomatosis, congenital neuroectodermal dysplasia, cutaneocerebral angioma, cutaneodermal angioma, encephalocutaneous angiomatosis, encephalofacial angiomatosis, encephalofacial neuroangiomatosis, encephalotrigeminal syndrome, encephalotrigeminal angiomatosis, encephalotrigeminal vascular syndrome, facial-meningeal angiomas, fourth phacomatosis syndrome, meningeal capillary angiomatosis, meningofacial angiomatosis, meningo-oculofacial angiomatosis, neuroangiomatosis encephalofacialis, neurocutaneous syndrome, neuroectodermal hamartoma, neuro-oculocutaneous angiomatosis, neurooculocutaneous syndrome, naevoid amentia, trigemino-encephalo-angiomatosis, phacomatosis syndrome, vascular encephalotrigeminal syndrome.
Dorlands Medical Dictionary bacillary angiomatosis, a condition seen in immunocompromised patients, caused by encephalofacial angiomatosis, encephalotrigeminal angiomatosis, SturgeWeber syndrome. http://www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspzQzpgzEzzSzppdocszSzuszS
EMedicine - Sturge-Weber Syndrome : Article Excerpt By: James J Riviello, Jr, MD SturgeWeber Syndrome - The Sturge-Weber syndrome (SWS), also called encephalotrigeminal angiomatosis, is a neurocutaneous disorder with angiomas involving the leptomeninges (leptomeningeal Related Terms encephalotrigeminal angiomatosis, encephalofacial angiomatosis, Sturge-Weber-Dimitri http://www.emedicine.com/neuro/byname/sturge-weber-syndrome.htm
Extractions: (advertisement) Synonyms, Key Words, and Related Terms: encephalotrigeminal angiomatosis, encephalofacial angiomatosis, Sturge-Weber-Dimitri syndrome Background: The Sturge-Weber syndrome (SWS), also called encephalotrigeminal angiomatosis, is a neurocutaneous disorder with angiomas involving the leptomeninges (leptomeningeal angiomas [LAs]) and skin of the face, typically in the ophthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve. The cutaneous angioma is called a port-wine stain (PWS). In the brain, LAs demonstrated by structural neuroimaging may be unilateral or bilateral; unilateral angiomas are more common. Functional neuroimaging may demonstrate a greater area of involvement than structural neuroimaging. This is called a structural versus functional mismatch. The neurologic manifestations vary, depending on the location of the LAs, which most commonly are located in the parietal and occipital regions, and the secondary effects of the angioma. These include seizures, which may be intractable; focal deficits, such as hemiparesis and hemianopia, both of which may be transient, called "strokelike episodes"; headaches; and developmental disorders, including developmental delay, learning disorders, and mental retardation. Developmental disorders are more common when angiomas are bilateral. Seizure control is thought to improve the neurologic outcome, and epilepsy surgery may be beneficial for refractory seizures.
Extractions: (advertisement) Synonyms, Key Words, and Related Terms: SWS, Sturge-Weber-Dimitri syndrome, encephalotrigeminal angiomatosis, trigeminal angiomatosis, meningofacial angiomatosis, encephalofacial angiomatosis, Dimitris hemoangiomatosis, Jahnkes syndrome (variant without glaucoma), Kulishers syndrome, Krabbes II syndrome, Lawfords syndrome (variant with glaucoma and without increased ocular pressure), meningocutaneous syndrome, neurooculocutaneous syndrome, Parkes Webers phacomatosis, vascular encephalotrigeminal Weber-Dimitri syndrome Background: Sturge-Weber syndrome (SWS) is a congenital disorder caused by the persistence of the transitory primordial sinusoidal plexus stage of vessel development. SWS is usually sporadic and characterized by a vascular malformation, with capillary venous angiomas that involve the face, choroid of the eye, and leptomeninges. The facial angioma has a predilection for the distribution of the first division of the trigeminal nerve. In addition to the angiomatous meningeal malformation, an underlying atrophy of the cerebral hemisphere is often present. The disease process is usually unilateral. Most patients (80%) have epilepsy, and more than 50% have a mental deficiency. Pathophysiology: Cranial manifestations The pathologic lesions in SWS include angiomatous facial malformation (port-wine stain or nevus flammeus), and venous angiomas involving the leptomeninges and choroid plexus. These lesions lead to seizures, atrophy, cerebrovascular thrombosis, and dystrophic intracortical calcification. Several variants of cortical calcification have been reported; these include calcification, which is present at birth (Yeakley, 1992); bilateral calcification (15%); and calcification contralateral to the facial nevus.
NORD - National Organization For Rare Disorders, Inc. encephalofacial angiomatosis. Encephalotrigeminal Angiomatosis. Leptomeningeal Angiomatosis. Meningeal Capillary Angiomatosis http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Sturge Weber
Syndrome DB - Table Of Contents angiomatosis. encephalofacial angiomatosis. encephalofacial neuroangiomatosis. encephalotrigeminal angiomatosis http://www.nlm.nih.gov/mesh/jablonski/syndrome_toc/toc_e.html
Neuropsychology the leptomeningeal angiomatosis characteristic of encephalofacial angiomatosis (SturgeWeber Disease affected hemisphere. Angiomatosis ( Encephalofacial; Sturge-Weber Disease) This http://braincampus.learnpsychology.com/neupath/tumor/angiomat.html
Extractions: Angiomatosis This image depicts the leptomeningeal angiomatosis characteristic of encephalofacial angiomatosis (Sturge-Weber Disease). Note the mild atrophy of the affected hemisphere. Angiomatosis (Encephalofacial; Sturge-Weber Disease): This is a developmental disorder that involves a capillary-venous angioma of the face and leptomeninges of the brain. The facial angioma is also called a nevus flammeus or "port wine stain". Most lesions are unilateral. Although the angioma is present at birth, the neurological manifestations of the disorder emerge over the course of development. They may include focal seizures, contralateral spastic hemiplegia, hemisensory loss and visual field defects. There may be cortical atrophy and calcifications.
Sturge Weber Syndrome disorder subdivision(s) covered by this report. Synonyms Dimitri Disease;encephalofacial angiomatosis; Encephalotrigeminal Angiomatosis; http://my.webmd.com/hw/raising_a_family/nord306.asp
Extractions: Sturge-Weber Syndrome is composed of three major symptoms. Excessive blood vessel growths (leptomeningeal angiomas) are accompanied by accumulations of calcium inside the brain, and seizures. Facial birth marks (nevus flammeus) appear usually on one side of the face. Angiomas similar to those found in the brain can develop inside the eye, often with secondary glaucoma.
Index Encephalitis, Japanese Encephalitis, Rasmussen s Encephalocele encephalocraniocutaneouslipomatosis encephalofacial angiomatosis Encephalopathy, Hypoglycemic http://my.webmd.com/hw/index/index-topics-E.asp
Extractions: It is possible that the main title of the report is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Information on the following diseases can be found in the Related Disorders section of this report: Sturge-Weber Syndrome is composed of three major symptoms. Excessive blood vessel growths (leptomeningeal angiomas) are accompanied by accumulations of calcium inside the brain, and seizures. Facial birth marks (nevus flammeus) appear usually on one side of the face. Angiomas similar to those found in the brain can develop inside the eye, often with secondary glaucoma. Nevus Flammeus is a discoloration on the face which is the red color of port wine. In Sturge-Weber Syndrome this "port wine stain" is noted at birth and generally occurs on the same side of the head as the excessive blood vessel growths (leptomeningeal angiomatoses) in the brain which are accompanied by accumulations of calcium (intracranial calcifications). The port wine stain primarily occurs along the distribution of the trigeminal nerve in the face, although in some cases it does not appear at all. Approximately thirty seven percent of patients have portwine stains on both sides of the face. Involvement of the extremities or trunk, in addition to the face, occurs in up to thirty-six percent of patients. Although the discoloration usually affects only one side of the face, a slight extension over the midface occurs in approximately fifty percent of cases. The port wine stain tends to deepen in color with age, and nodular elevations may also develop.
Entrez PubMed Click here to read encephalofacial angiomatosis sparing the occipital lobe andwithout facial nevus on the spectrum of SturgeWeber syndrome variants? http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1
Entrez PubMed Abstract, encephalofacial angiomatosis sparing the occipital lobe and without facialnevus on the spectrum of SturgeWeber syndrome variants? J Child Neurol. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Link&db=PubMed&dbFrom=PubMed&f
Brain Tumor Brain Tumor Angiomatosis. This image depicts the leptomeningeal angiomatosischaracteristic of encephalofacial angiomatosis (SturgeWeber Disease). http://nanonline.org/nandistance/mtbi/NeuroIll/tumor/angio.html
Extractions: Angiomatosis This image depicts the leptomeningeal angiomatosis characteristic of encephalofacial angiomatosis (Sturge-Weber Disease). Note the mild atrophy of the affected hemisphere. Angiomatosis (Encephalofacial; Sturge-Weber Disease): This is a developmental disorder that involves a capillary-venous angioma of the face and leptomeninges of the brain. The facial angioma is also called a nevus flammeus or "port wine stain". Most lesions are unilateral. Although the angioma is present at birth, the neurological manifestations of the disorder emerge over the course of development. They may include focal seizures, contralateral spastic hemiplegia, hemisensory loss and visual field defects. There may be cortical atrophy and calcifications.
CNS Syndromes Associated With Vascular Malformations SturgeWeber syndrome (encephalotrigeminal or encephalofacial angiomatosis) Extensivecapillary-venous malformation results in unilateral cerebral cortical http://moon.ouhsc.edu/kfung/JTY1/NeuroHelp/ZNG0DD02.htm
Extractions: CNS Syndromes Associated with Vascular Malformations NeuroLearn NeuroHelp Malformations 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
