“Sturge – Weber Syndrome: A Case Report and Review of Literature”

Abstract

Sturge-Weber syndrome is a rare, sporadically occurring neurocutaneous syndrome characterized by port wine stain (facial nevus flammeus), congenital glaucoma, and anomalous leptomeningeal angiomatous malformation. Port wine stain is usually the first component of the syndrome. Seizures of the side contralateral to the port wine stain occur early in infancy and worsen with age. Radiological investigations, like computed tomography and magnetic resonance imaging are most useful, playing a pivotal role in demonstrating the cerebral changes. We report the case of a three year old child presenting with port wine stain over left half of the body, weakness of right upper and lower limb with an episode of seizure. The child was known to have seizures from 6 months of age and was on irregular anticonvulsant treatment. This case highlights the various neurological manifestations of Sturge-Weber syndrome and how imaging helps to characterize each.