Sturge-Weber syndrome： a case report and review of literatures

Sturge-Weber syndrome （SWS）, or encephalo- trigeminal angiomatosis, is a rare, congenitalneurocutaneous syndrome characterized by unilateral facial cutaneous vascular malformation （nevus flammeus or port-wine stain） in association with ipsilateral leptomeningeal angiomatosis. Prevalence is approxima...

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Bibliographic Details
Published in:Chinese medical journal 2010-01, Vol.123 (1), p.117-121
Main Authors: Zhou, Jing, Li, Nan-yun, Zhou, Xiao-jun, Wang, Jian-dong, Ma, Heng-hui, Zhang, Ru-song
Format: Article
Language:English
Subjects:
Adult
Female
Humans
Male
Sturge-Weber Syndrome - diagnosis
Sturge-Weber Syndrome - pathology
SWS
Young Adult
三叉神经
综合征
脑组织
面部皮肤
鲜红斑痣
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Summary:Sturge-Weber syndrome （SWS）, or encephalo- trigeminal angiomatosis, is a rare, congenitalneurocutaneous syndrome characterized by unilateral facial cutaneous vascular malformation （nevus flammeus or port-wine stain） in association with ipsilateral leptomeningeal angiomatosis. Prevalence is approximately one per 50 000 live births. Males and females are equally affected and there is no racial bias. 1,2 The common clinical manifestations of SWS include progressive seizures, unilateral cutaneous vascular nevus following the ophthalmic divisions of the trigeminal nerve, ipsilateral glaucoma, contralateral hemiparesis, hemiatrophy, hemianopia and mental retardation. The radiographic hallmark of SWS is ＂tram-line＂ or gyriform calcifications usually involving the occipital and parietal lobes. Histologic studies have revealed that intracranial lesions of SWS display as leptomeningeal angiomatosis, and gyriform calcifications, neuronal loss, astrogliosis in underlying brain tissue.
ISSN:0366-6999
2542-5641
DOI:10.3760/cma.j.issn.0366-6999.2010.01.023