Answer of Previous Clinical Quiz

Sturge Weber Syndrome (Encephalotrigeminal Angiomatosis):This disease was described clinically in 1874 by W.Allen Struge and radiographically in 1923 by Parks Weber for the first time. The syndrome is presence of a unilateral port-wine nevus in the ophthalmic division of the trigeminal nerve, along with seizure, mental retardation, contralateral hemisensory and motor deficit. The nevus is present at birth, and involvement of the upper eyelid is a strong clue to cerebral involvement, that is accompanied by a leptomeningeal angiomatosis in the parietooccipital area on the same side, which is associated with underlying cortical gliosis and calcification.Radiographic finding consist of cortical calcification that gives rise to the characteristic tram line calcification seen on plain radiography, and also calcification, cortical atrophy and hypertrophied choroids plexus on CT scan.Treatment consist of symptomtheraphy, anticonvulsant, resection of focal cortical lesion, and finally cerebral hemishpherectomy in refractory cases. Out come is directly related to the extent of primary pathology and progress of disease and complications, so clinical follow up and monitoring for seizure, buphthalmus, glaucoma, facial deformity and neurologic deficit is essential.