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Sturge-Weber-Syndrom

Sturge-Weber syndrome

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Zusammenfassung

Das Sturge-Weber-Syndrom (SWS) ist eine seltene, kongenitale Erkrankung, die das Gehirn, die Haut und die Augen betrifft. Es handelt sich um ein sporadisch auftretendes neurokutanes Syndrom mit Beteiligung intrazerebraler Venen (venöse Angiomatose). Die Häufigkeit wird auf 1:50.000 Geburten geschätzt [1]. Leitsymptom ist eine intrakranielle leptomeningeale Angiomatose, die meist den Okzipital- und posterioren Parietallappen betrifft und ein- aber auch beidseitig vorkommen kann. Ipsilateral treten hierzu faziale kutane vaskuläre Veränderungen in Form eines Portweinnävus auf, die üblicherweise im Versorgungsgebiet des Trigeminusastes V1 zu finden sind. Andere mit dem SWS assoziierte klinische Erscheinungen sind Anfälle, Glaukom, Kopfschmerzen, transiente neurologische „schlaganfallähnliche“ Episoden und kognitive Beeinträchtigungen. Neurologisch finden sich oft eine Hemiparese, Hemiatrophie und Hemianopsie kontralateral zu den kortikalen Veränderungen.

Abstract

Sturge-Weber syndrome (SWS) is a rare congenital disease which affects the brain, the skin and the eyes. It is a sporadically occurring neurocutaneous syndrome affecting the intracerebral veins (venous angiomatosis). The frequency is estimated to be 1 in 50,000 births [1]. The main symptom is intracranial leptomeningeal angiomatosis which mostly affects the occipital and posterior parietal lobes and can occur unilaterally and also bilaterally. Facial cutaneous vascular alterations occur ipsilaterally in the form of port wine stains (nevus flammeus) which are normally found in the catchment area of the trigeminal branch VI. Other clinical symptoms associated with SWS are seizures, glaucoma, headaches, transient neurological stroke-like episodes and cognitive impairment. Neurological cortical symptoms often include hemiparesis, hemiatrophy and hemianopsia.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. W. Reith, U. Yilmaz, A. Zimmer geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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  1. Klinik für Diagnostische und Interventionelle Neuroradiologie, Universitätsklinikum des Saarlandes, 66424, Homburg/Saar, Deutschland

    W. Reith, U. Yilmaz & A. Zimmer

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  1. W. Reith
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  2. U. Yilmaz
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Correspondence to W. Reith.

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Reith, W., Yilmaz, U. & Zimmer, A. Sturge-Weber-Syndrom. Radiologe 53, 1099–1103 (2013). https://doi.org/10.1007/s00117-013-2554-x

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