Sturge-Weber Syndrome

Sturge-Weber syndrome is a neurological disorder characterized by seizures and a large port-wine stain birthmark. Seizures caused by this condition usually begin in infancy and may worsen with age. Most children with Sturge-Weber syndrome are born with glaucoma or will develop it later in life. Treatment of this disorder focuses on the symptoms; anticonvulsant medications may be used to treat seizures, and laser treatment may be used to lighten or remove the birthmark.

 

What Is Sturge-Weber Syndrome?

Sturge-Weber syndrome is a neurological disorder indicated at birth by seizures accompanied by a large port-wine stain birthmark on the forehead and upper eyelid on 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 the loss of nerve cells and calcification of tissue in the cerebral cortex of the brain on the same side of the body as the birthmark.
 

What Symptoms Are Associated With Sturge-Weber Syndrome?

Neurological symptoms of Sturge-Weber syndrome include seizures that begin in infancy and may worsen with age. Convulsions usually happen on the side of the body opposite the birthmark and vary in severity.
 
There may be muscle weakness on the same side. Some children will have developmental delays and mental retardation; most will have glaucoma (increased pressure within the eye) at birth or develop it later. The increased pressure within the eye can cause the eyeball to enlarge and bulge out of its socket (buphthalmos).
 
Sturge-Weber syndrome rarely affects other body organs.
 
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Written by/reviewed by: Arthur Schoenstadt, MD
Last reviewed by: Arthur Schoenstadt, MD