Spectrum of CT and MR findings in Sturge-Weber syndrome: A case report

Abstract

Sturge-Weber syndrome is an uncommon, 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 10-month-old infant presenting with port wine stain over face, trunk, and extremities, hemiparesis of the left side of the body, and new onset of seizure. This case highlights the various neurological manifestations of Sturge-Weber syndrome and how imaging helps to characterize each.

Keywords

• Cerebral hemiatrophy
• neurocutaneous syndrome
• phakomatoses
• port wine stain
• pial angioma
• Sturge-Weber syndrome (SWS)