title: [Sturge-Weber syndrome].
abstract: Sturge-Weber syndrome (SWS) is a rare neurocutaneous syndrome characterized by intracranial leptomeningeal angioma, facial port-wine nevi, and glaucoma. Diagnosis is relatively easy because of the facial angioma and MRI findings, but evaluating severity is difficult. Predictors of SWS's prognosis are epilepsy and brain dysfunction under the leptomeningeal angioma. Therefore, active research has been intensely conducted with electrophysiological, neuroimaging, and neuropsychological methods. Final goals of this research are to define the therapeutic strategy. In this review, we focus on recent advances in neuroimaging and EEG analysis to discover the epileptogenesis, the most adequate therapy, and prospective topics of investigation in SWS.
authors.affiliation: Department of Neurosurgery, Juntendo University, Bunkyo-ku, Tokyo, Japan.
authors.forename: Hidenori
authors.initials: H
authors.lastname: Sugano
authors.forename: Hajime
authors.initials: H
authors.lastname: Nakanishi
authors.forename: Madoka
authors.initials: M
authors.lastname: Nakajima
authors.forename: Takuma
authors.initials: T
authors.lastname: Higo
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Iimura
authors.forename: Kazuaki
authors.initials: K
authors.lastname: Shimoji
authors.forename: Hajime
authors.initials: H
authors.lastname: Arai
mesh_terms: D006801:Humans; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000601:surgery*