Atypical Sturge-Weber Syndrome Presenting with Stroke-Like Episode / 대한간질학회지

ABSTRACT

Sturge-Weber syndrome (SWS) is a rare sporadic neurocutaneous syndrome, characterized by port-wine nevus of face, ocular vascular abnormalities and leptomeningeal angioma. A 63-year-old man developed sudden onset of right hemiparesis and global aphasia. He had a past history of epilepsy. Physical examination showed no skin lesions. Brain CT demonstrated gyriform calcification in the left frontal area. Brain MRI revealed cortical atrophy in the left hemisphere with no diffusion restrictions. Fourteen hours later, he was completely recovered from aphasia and motor weakness. We report a case of atypical SWS with episode of acute hemiparesis which highlight the difficulty in distinguishing seizure from ischemic symptoms.