A Case of Incomplete form cf Sturge-Weber Syndrome

ABSTRACT

Sturge-Weber syndrome is a neurocutaneous syndrome characterized by portwine nevus of the face with angiomatosis of the ipsilateral cerebral leptomeninges and extensive calcification in the underlying cerebral cortex. Associated features include mental retardation, generalized or focal seizures, hemiparesis, choroidal angioma, & buphthalmos or glaucoma. We experienced a case of incomplete form of Sturge-Weber syndrome in a 12 year-old boy, who had generalized seizure, a homonymous hemianopsia, intracranial calficication and leptomeningeal angiomatosis without facial nevus or mental retardation. So we presented a case with a brief review of related literatures.