Intracranial variant of encephalotrigeminal angiomatosis – A case report

Abstract

Encephalotrigeminal Angiomatosis is a rare developmental phakomatoses characterized by the occurrence of nevus flammeus (port-wine stain) along the distribution of branches of trigeminal nerve, vascular angiomas in the eye, and leptomeningeal angiomas affecting 1 in 1,00,000 South Asian population. Herewith, such a rare case of such encephalotrigeminal angiomatosis in a 24-year-old male is described.

Keywords

• hemianopsia
• phakomatoses
• port-wine stain
• status epilepticus
• trigeminal nerve