The Sturge-Weber syndrome is a congenital vascular disease (. As seizures, focal neurological deficits, mental retardation z) is characterized by a port-wine stain on the face, a leptomeningeal angioma and neurological complications.

The Sturge-Weber syndrome is a neurocutaneous syndrome that occurs in 1 in 50,000 people. Sturge-Weber syndrome is not hereditary. It is caused by a somatic mutation (a change in the DNA that occurs after conception in the precursors of the affected area) in GNAQ gene on chromosome 9q21.

The Sturge-Weber syndrome is a congenital vascular disease (. As seizures, focal neurological deficits, mental retardation z) is characterized by a port-wine stain on the face, a leptomeningeal angioma and neurological complications. The Sturge-Weber syndrome is a neurocutaneous syndrome that occurs in 1 in 50,000 people. Sturge-Weber syndrome is not hereditary. It is caused by a somatic mutation (a change in the DNA that occurs after conception in the precursors of the affected area) in GNAQ gene on chromosome 9q21. The Sturge-Weber Syndrome causes a capillary malformation, called port-wine stain (or sometimes referred to as a spot or birthmark), typically on the forehead and upper eyelid in the area of ??the first and / or second bough of the trigeminal nerve. A similar vascular lesion leptomeningeal angioma-occurs in 90% of patients if the wine stain on one side of both the upper and the lower eyelid covered, but if only one eyelid is affected in only 10-20% of cases. Usually, the nevi and leptomeningeal angiomas are unilateral, but in rare cases, patients have bilateral wine stains in the distribution of the first division of the trigeminal nerve and bilateral leptomeningeal angioma. A port wine stain may occur without leptomeningeal angioma and the accompanying neurological symptoms; in such cases, the eyes and eyelids may be involved or not. In rare cases, there is a leptomeningeal angioma without wine stain with ocular involvement. Fascial nevi flammei lateral © Springer Science + Business Media var model = {thumbnailUrl: ‘/-/media/manual/professional/images/433-facial-port-wine-stain-slide-4-springer-high_de.jpg?la= de & thn = 0 & mw = 350 ‘, imageUrl:’ /-/media/manual/professional/images/433-facial-port-wine-stain-slide-4-springer-high_de.jpg?la=de&thn=0 ‘, title: ‘fascial nevi flammei lateral’ description: ‘ u003Ca id = “v37898013 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eDiese Figure shows a child with a birthmark that the ophthalmic branch (V1) affects the trigeminal u003c / p u003e u003c / div u003e ‘credits’. © Springer Science + Business Media’

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