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El síndrome de Sturge-Weber es un trastorno vascular congénito . el 77% de los pacientes, siendo la enfermedad más frecuente el glaucoma (46%), seguido . ORPHA Synonym(s). Encephalofacial angiomatosis; Encephalotrigeminal angiomatosis; SWS; Sturge-Weber-Dimitri syndrome; Sturge-Weber-Krabbe. Meanings of “enfermedad de sturge-weber” in English Spanish Dictionary: 1 result(s) Meanings of “enfermedad de sturge-weber” with other terms in English.

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In the year has been indexed in the Medlinedatabase, and has become a vehicle for expressing the most current Spanish medicine and modern. The leptomeningeal hemangioma results in a vascular steal affecting the subjacent cortex and white matter producing localised ischemia.

It is characterized by a facial Port-wine stain, leptomeningeal angiomatosis, and glaucoma. June Pages ee38 Pages Frequent ophthalmologic examinations should be carried out in cases with glaucoma. Case 5 Case 5.

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Case 6 Case 6. Focal resection or hemispherectomy should be investigated when medical management fails to control the seizures. Sometimes, the PWS may also cover the maxillary and mandibular areas of the face and webet some cases may extend to the trunk and limbs. Infants typically present in the first year of life with leptomeningeal angiomatosis, responsible for the occurrence of focal or complex partial seizures, early-handedness, and visual gaze preference.


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Term Bank – enfermedad de sturge-weber – Spanish English Dictionary

Neuroimaging studies show leptomeningeal angiomatosis, supporting diagnosis. Sturge-Weber syndrome is a rare syndrome, with an incidence estimated at 1 case in 20, persons Case 10 Case Unlike most phakomatoses, Sturge-Weber syndrome is sporadic with no definite identifiable hereditary component 5, Case 7 Case 7.

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Síndrome de Sturge-Weber: revisión | Actas Dermo-Sifiliográficas (English Edition)

In rare cases, patients may not present with PWS. Clinical description The facial capillary malformation classically referred to as angioma is a port-wine stain PWS that is generally present at birth and located on the forehead or upper eyelid on one or both sides of the face. CiteScore measures average citations received per document published.

Are you a health professional able to prescribe or dispense drugs? Case 8 Case 8. All articles are subjected to a rigorous process of revision in pairs, and careful editing enfermevad literary and scientific style. Diagnosis is suspected upon clinical examination.

Health care resources for this disease Expert centres Diagnostic tests 10 Patient organisations 65 Orphan drug s 0. Read it at Google Books – Find it at Amazon. Eye involvement can occur at anytime but is generally observed during infancy and young adulthood.


Case 1 Case 1. Genetic counseling The disease is sporadic.

enfermedad de sturge-weber

Case 9 Case 9. Thank you for updating your details. The prognosis depends on the severity of the epileptic crises which may lead to variable degrees of psychomotor regression and intellectual disability. For all other comments, please send your remarks via contact us. Case 11 Case From Monday to Friday from 9 a. Surgery may be recommended. To quiz yourself on this article, log in to see multiple choice questions.

Specialised Social Services Eurordis directory. Prognosis depends on the extent of leptomeningeal involvement and the severity of the glaucoma. If you are a member of the AEDV: Case 14 Case Treatment revolves primarily around seizure control, with surgical resection only indicated rarely in refractory cases.