Sturge-Weber syndrome: A rare entity

Abstract

A 10-year-old boy presented with focal seizure involving right angle of mouth and arm followed by generalized seizures, which were controlled with valproic acid. The majority of seizures were focal seizures, which were confirmed by the electroencephalography. Initiallywe started on 15mg/kg and then gradually over 3 mo seizures got control with 25 mg/kg/day and he is now seizure free over last 15 mo. He had normal developmental milestones except for his school performance which was slightly lesser than his peers. On physical examination, head circumference was within 2 standard deviations for his age. He had facial angiomas and soft tissue hypertrophy on left side (Fig. 1). Opthalmological examination was normal. In our case, there were presence of both facial and leptomeningeal angiomas, therefore he belongs to type I. Cranial computerized tomography revealed calcifications in left frontotemporo-parietal region (Fig. 2). Cranial magnetic resonance imaging revealed presence of cortical atrophy in left hemisphere and patchy areas of mild contrast enhancement suggesting presence of venous angiomas (Fig. 3). The Sturge-Weber syndrome (SWS) is also called encephalotrigeminal angiomatosis. It is a neurocutaneous disorder with angiomas involving the leptomeninges (leptomeningeal angiomas [LA]) and skin of the face, typically in the ophthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve. The cutaneous