title: [Sturge-Weber syndrome].
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.
authors.affiliation: Klinik für Diagnostische und Interventionelle Neuroradiologie, Universitätsklinikum des Saarlandes, 66424, Homburg/Saar, Deutschland, Wolfgang.Reith@uniklinikum-saarland.de.
authors.forename: W
authors.initials: W
authors.lastname: Reith
authors.forename: U
authors.initials: U
authors.lastname: Yilmaz
authors.forename: A
authors.initials: A
authors.lastname: Zimmer
mesh_terms: D000293:Adolescent; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009422:Nervous System Diseases / Q000175:diagnosis / Q000628:therapy*; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000628:therapy*; D014057:Tomography, X-Ray Computed / Q000379:methods