ANGIOMATOSE DE STURGE WEBER PDF

Sturge — beschrieben. Augenerkrankungen z. Im Computertomogramm sieht man den Gewebsschwund im Gehirn Hirn atrophie. In der Regel ist eine Augensymptomatik an der Seite festzustellen, an der das Angiom im Gesicht besteht. Literatur[ Bearbeiten Quelltext bearbeiten ] W.

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Syndrome de Sturge-Weber. The current neuro-imaging data Sturge-Weber syndrome SWS is a rare congenital sporadic disease with neuro-ocular and cutaneous vascular findings. Clinically, the full-blown condition consists of a facial port-wine stain PWS involving the V1 facial trigeminal skin area, alone or in combination with V2 and V3PWS, seizures and ocular abnormalities glaucoma and choroidal angioma. Radiologically, a leptomeningeal pial capillary and venous malformation, mostly located in the parieto-occipital area, cerebral atrophy and calcifications are demonstrated.

An ipsilateral enlarged choroid plexus may be an early anatomic symptom. Developmental venous anomalies DVA of the brain are sometimes associated. MR with gadolinium enhancement is the optimal neuro-diagnostic imaging technique for the screening of infants with an at-risk V1PWS, as well as for the follow-up of patients with evidence SWS.

Accelerated myelination in the involved hemisphere may be an early diagnostic feature before 6months of age. Later, hyperintensity of white matter on T2 is considered a symptom of gliosis. Clinically, progression of the diseases is associated with anatomic changes and correlates with the extent of the pial vascular anomaly, extent and severity of cerebral atrophy, and white matter abnormalities. Key words : Sturge-Weber syndrome. Le SSW peut faire partie de la phacomatose pigmento-vasculaire.

Les tableaux sont exclusivement disponibles en format PDF. Figure 1. Port-wine stain in the region of the ophtalmic nerve V1 that extended to the zone innerved by the second branch of the trigeminal nerve.

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Síndrome de Sturge-Weber

Syndrome de Sturge-Weber. The current neuro-imaging data Sturge-Weber syndrome SWS is a rare congenital sporadic disease with neuro-ocular and cutaneous vascular findings. Clinically, the full-blown condition consists of a facial port-wine stain PWS involving the V1 facial trigeminal skin area, alone or in combination with V2 and V3PWS, seizures and ocular abnormalities glaucoma and choroidal angioma. Radiologically, a leptomeningeal pial capillary and venous malformation, mostly located in the parieto-occipital area, cerebral atrophy and calcifications are demonstrated. An ipsilateral enlarged choroid plexus may be an early anatomic symptom.

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Syndroom van Sturge-Weber

Ook in de hersenen bestaan er hemangiomen van de leptomeningen zachte hersenvliezen , die aanleiding geven tot epilepsie en atrofie. Verder kunnen optreden: ontwikkelingsstoornissen, gezichtsveldstoornissen, en andere neurologische uitval zoals verlammingen. Alle symptomen zijn direct of indirect een gevolg van de abnormale bloedvatontwikkeling. Van het syndroom is sprake als de eerste twee kenmerken aanwezig zijn. Een wijnvlek zonder verdere afwijkingen binnen de schedel valt er niet onder.

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