Characteristic syndrome: cutaneous vascular port-wine nevus of the face, contralateral hemiparesis and hemiatrophy, glaucoma, seizures, mental retardation.

Neurologic: epilepsy most common neurologic manifestation, usually starting before age 1 year with focal motor, generalized major motor, or partial complex seizures. Onset of seizures before age 2 years, refractory epilepsy associated
with increased risk of intellectual impairment. Homonymous hemianopia due to occipital lobe atrophy. Behavioral problems include attention-deficit disorder.