Atypical Sturge-Weber Syndrome: A case report

ABSTRACT

Sturge-Weber syndrome is a congenital neurocutaneous disorder of the vessels of the face, the leptomeninges and the brain. Clinically SWS consists of symptoms and signs including a facial nevus (port-wine stain), seizure, hemiparesis, mental retardation. But only a few reports related to atypical Sturge-Weber syndrome without facial nevus have been published. We report a patient with atypical Sturge-Weber syndrome without any clinical feature except hemiparesis. In neuroimaging studies using brain CT scan and MRI, leptomeningeal angiomatosis was demonstated that is the characteristic feature of Sturge- Weber syndrome. In atypical Sturge-Weber syndrome, there may be late- developed complications such as hemiparesis. In conclusion, neuroimaging study is recommended to confirm diagnosis in suspicious atypical SWS patient.