The Sturge-Weber syndrome, also called encephalotrigeminal angiomatosis, is a neurocutaneous disorder with angiomas involving the leptomeninges and skin of the face, typically in the ophthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve. The cutaneous angioma is called a port-wine stain.
The port-wine stains are present at birth. Both sexes are affected equally.
In the brain, the leptomeningiomas are usually unilateral, however, bilateral angiomas may occur. Neurologic and developmental manifestations include seizures in 75-90% of the cases, hemiparesis in about 1/3 of the patients, strokes, headaches, hemianopsia, mental retardation, and developmental abnormalities.