Teaching NeuroImages: Sturge-Weber syndrome presenting in a 58-year-old woman with seizures.

A 58-year-old woman with a history of migraine presented with headache, aphasia, right homonymous hemianopsia, and right hemiparesis, followed by 2 seizures. On examination, she was noted to have a facial angioma (figure 1) that was present from childhood. MRI findings (figure 2, A–C) were consistent with leptomeningeal angiomatosis, making the diagnosis of Sturge-Weber syndrome. Sturge-Weber syndrome is a sporadically occurring (nonhereditary) neurocutaneous syndrome, hypothesized to be caused by somatic mutations in fibronectin expression causing capillary malformation1 and dysfunctional cortical perfusion.2 It is usually diagnosed in infancy, but it may occasionally present in adulthood with seizures or strokelike episodes.