Often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma (cerebral malformations and tumors).
Cardinal features:
a) localized cerebral cortical atrophy and calcifications (especially cortical layers 2 and 3, with a predilection for the occipital lobes):
● calcifications appear as curvilinear double parallel lines (“tram-tracking”) on plain x-rays
● cortical atrophy usually causes contralateral hemiparesis, hemiatrophy, and homonymous hemianopia (with occipital lobe involvement)
b) ipsilateral port-wine facial nevus (nevus flammeus) usually in distribution of 1st division of trigeminal nerve (rarely bilateral) other findings that may be present:
a) ipsilateral exophthalmos and/or glaucoma, coloboma of the iris
b) oculomeningeal capillary hemangioma
c) convulsive seizures: contralateral to the facial nevus and cortical atrophy. Present in most patients starting in infancy
d) retinal angiomas