May Awareness: Sturge-Weber Syndrome

Sturge-weber-rev

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Struge-Weber Syndrome

Sturge-Weber syndrome is a neurological disorder indicated at birth by a port-wine stain birthmark on the forehead and upper eyelid of one side of the face.  The birthmark can vary in color from light pink to deep purple and is caused by an overabundance of capillaries around the trigeminal nerve just beneath the surface of the face.  Sturge-Weber syndrome is also accompanied by abnormal blood vessels on the brain surface and the loss of nerve cells and calcification of underlying tissue in the cerebral cortex of the brain on the same side of the brain as the birthmark.

Treatment for Sturge-Weber syndrome is symptomatic.  Laser treatment may be used to lighten or remove the birthmark.   Anticonvulsant medications may be used to control seizures.

Although it is possible for the birthmark and atrophy in the cerebral cortex to be present without symptoms, most infants will develop convulsive seizures during their first year of life.  There is a greater likelihood of intellectual impairment when seizures start before the age of 2 and are resistant to treatment.  Prognosis is worst in the minority of children who have both sides of the brain affected by the blood vessel abnormalities.

Information provided by:  NIH: National Institute of Neurological Disorders and Stroke

For more information on Sturge-Weber syndrome you can go to The Sturge-Weber Foundation at www.sturge-weber.org.

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