Sturge-Weber Syndrome

The port-wine birthmark varies in size and color, ranging from light pink to deep purple. It usually appears on the forehead and upper eyelid of one eye but may also include the lower eyelid and face. If both eyelids are involved, people are much more likely to have an angioma in the tissues covering the brain.

Seizures Seizures in Children Seizures are a periodic disturbance of the brain’s electrical activity, resulting in some degree of temporary brain dysfunction. When older infants or young children have seizures, they often... read more occur in about 75 to 90% of people and typically start by the time children are 1 year old. Usually, seizures occur on only one side of the body, opposite the birthmark, but they may affect the whole body. About 25 to 50% of people have weakness or paralysis on the side opposite the birthmark. Coordination may be lost. The weakness or paralysis sometimes worsens, especially if seizures cannot be controlled.

About 50% of people have some intellectual disability Intellectual Disability Intellectual disability is significantly below average intellectual functioning present from birth or early infancy, causing limitations in the ability to conduct normal activities of daily... read more . Intellectual disability is more severe in people whose seizures start before 2 years of age and cannot be controlled with drugs. Development of motor and language skills may be delayed.

Glaucoma Glaucoma Glaucomas are a group of eye disorders characterized by progressive optic nerve damage (often, but not always, associated with increased eye pressure) that can lead to irreversible loss of vision... read more may damage the optic nerve (which connects the brain to the eye), causing loss of vision, in the eye on the same side as the birthmark. Glaucoma may be present at birth or develop later. The eyeball may enlarge and bulge out.

Doctors suspect Sturge-Weber syndrome in children with the characteristic birthmark.

Magnetic resonance imaging (MRI) Magnetic Resonance Imaging (MRI) Magnetic resonance imaging (MRI) is a type of medical imaging that uses a strong magnetic field and very high frequency radio waves to produce highly detailed images. During an MRI, a computer... read more is used to check for angiomas in the tissues covering the brain. In older children and adults, computed tomography (CT) Computed Tomography (CT) Computed tomography (CT) is a type of medical imaging that combines a series of x-rays to create cross-sectional, detailed images of internal structures. In computed tomography (CT), which used... read more of the head is done if MRI is not available.

A neurologic examination Neurologic Examination When a neurologic disorder is suspected, doctors usually evaluate all of the body systems during the physical examination, but they focus on the different parts of the nervous system. Examination... read more is done to check for evidence of weakness and lack of coordination and/or paralysis. An eye examination is done to check for eye problems.

Treatment of Sturge-Weber syndrome focuses on relieving symptoms.

Antiseizure drugs Antiseizure drugs In seizure disorders, the brain's electrical activity is periodically disturbed, resulting in some degree of temporary brain dysfunction. Many people have unusual sensations just before a seizure... read more to control seizures and drugs to treat glaucoma Medications Glaucomas are a group of eye disorders characterized by progressive optic nerve damage (often, but not always, associated with increased eye pressure) that can lead to irreversible loss of vision... read more are used. Surgery for glaucoma Surgery Glaucomas are a group of eye disorders characterized by progressive optic nerve damage (often, but not always, associated with increased eye pressure) that can lead to irreversible loss of vision... read more or surgery for seizures Surgery In seizure disorders, the brain's electrical activity is periodically disturbed, resulting in some degree of temporary brain dysfunction. Many people have unusual sensations just before a seizure... read more that return despite drug treatment may be required.

People are usually given low doses of aspirin because aspirin may reduce the risk of strokes. Also, aspirin may improve blood flow in the part of the brain under the angioma. However, there is no proof that aspirin is effective.

Laser treatment Using Lasers to Treat Skin Problems may be used to lighten or remove the birthmark.