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Sturge-Weber Syndrome

Sturge-Weber Syndrome Causes

Sturge-Weber syndrom is the result of residual embryonal blood vessels and their secondary effects on surrounding brain tissue.

Sturge-Weber Syndrome Definition

Sturge-Weber syndrome, sometimes known as encephalotrigeminal angiomatosis, is an extremely rare congenital neurological and skin disorder.

Sturge-Weber Syndrome Diagnosis

In the "incomplete" forms of SWS, CNS angiomas arise without cutaneous features and therefore, no suspicion of SWS occurs until a seizure or other neurologic problem develops. Because of this, the diagnosis of SWS is not always straightforward.

Sturge-Weber Syndrome Symptoms and Signs

Sturge-Weber syndrome is characterized at birth by seizures accompanied by a large port-wine stain birthmark on the forehead and upper eyelid of one side of the face. The birthmark can differ in color from light pink to deep purple, and is caused by an overabundance of capillaries around the ophthalmic branch of the trigeminal nerve, just beneath the surface of the face.

Sturge-Weber Syndrome Treatment

Laser treatment may be administered to lighten or remove the birthmark. Anticonvulsant medications may be utilized to control seizures. Doctors recommend yearly monitoring for glaucoma, and surgery may be performed on more severe cases. Physical therapy should be considered for infants and children experiencing muscle weakness.

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