Sturge-Weber Syndrome Information Page
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What is Sturge-Weber Syndrome?
Is there any treatment?
What is the prognosis?
What is Sturge-Weber Syndrome?
Sturge-Weber syndrome (also called "encephalotrigeminal angiomatosis") is a congenital disorder characterized by a vascular birthmark and neurological abnormalities. Symptoms of the disorder, which vary widely among patients, may include eye and internal organ irregularities. The most apparent symptom is a facial birthmark or port wine stain which is present at birth and usually involves at least one upper eyelid and the forehead. The stain, varying from light pink to deep purple, is caused by an overabundance of capillaries just beneath the surface of the affected skin. Neurological symptoms include excessive blood vessel growth on the surface of the brain (angiomas). These angiomas are typically located on the posterior or occipital region of the brain and cause seizures, which often start before one year of age and may worsen with age. The convulsions usually appear on the side of the body opposite the port wine stain and vary in severity. A weakening or loss of use of the side of the body opposite the port wine stain (hemiparesis) may also develop. Developmental delay of motor and cognitive skills may occur. Glaucoma (increased pressure within the eye) may be present at birth or develop later. Buphthalmos (enlargement of the coatings of the eye) may also occur in the eye that is affected by the port wine stain. Sturge-Weber syndrome rarely affects other body organs.
Is there any treatment?
Treatment for Sturge-Weber syndrome is symptomatic. Laser treatment is available to lighten and/or remove port wine stains. Anticonvulsant medications may be used to control seizures. Surgery and/or eyedrops may be prescribed to control glaucoma.
What is the prognosis?
Although SWS-related seizures can sometimes place patients in potentially life-threatening situations, the disease itself is not fatal.
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