TY - CHAP M1 - Book, Section TI - Sturge–Weber Syndrome A1 - Arzimanoglou, Alexis A1 - Kossoff, Eric A2 - Duchowny, Michael A2 - Cross, J. Helen A2 - Arzimanoglou, Alexis PY - 2017 T2 - Pediatric Epilepsy AB - Sturge–Weber syndrome (SWS) is a nonfamilial neurocutaneous disorder with a potentially progressive course. The syndrome consists of a nevus flammeus (port-wine stain) involving part of the face, in most of the patients all or part of the area supplied by the trigeminal nerve (V1 distribution most commonly). In all patients a venous angioma of the leptomeninges and less often, a choroidal angioma, and ipsilateral glaucoma is present. The facial and leptomeningeal angioma are usually ipsilateral, but both can be bilateral. Pial angiomatosis more frequently occurs in the occipital region, but it can be localized anywhere and can involve an entire hemisphere or even be bilateral. The extent of lesions that affect the facial skin, eyes, and central nervous system vary between patients, and in some cases only a single-organ system may be affected.1 Cases of SWS exist without the facial port-wine stain. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/04/16 UR - neurology.mhmedical.com/content.aspx?aid=1138411088 ER -