Sturge—Weber综合征研究进展

Sturge—Weber综合征是一种罕见的先天性神经皮肤综合征，主要累及软脑膜、面部三叉神经支配区及眼脉络膜。患者常伴发有癫痫、青光眼、智力障碍、偏瘫及偏头痛等症状。本病发病率较低，且一般多为单发，目前对其了解尚少。近几年来，一些神经成像、临床和分子神经病理学方面的研究使我们加深了对该病的认识。本文就目前关于该病的病因学、诊断及治疗等方面的新进展作一综述。

Research advances in diagnosis and treatment of Sturge-Weber syndrome

Sturge-Weber syndrome （SWS） is a rare, congenital neurocutaneous disorder with a leptomeningeal,facial trigeminal nerve dominative area and choroidal angioma. SWS patients frequently develop seizures, glaucoma, headaches, migraines, cognitive deficits, stroke-like episodes, focal neurologic impairments, and visual problems. The incidence of this disease is low and it is usual a single shot. This disease is little understood now. Recent neuroimaging, clinical and molecularneuropathologic studies have provided new insights into the neurologic aspects of Sturge-Weber syndrome. This review highlights the recent advances in the etiology, diagnosis and management of the neurologic aspects of SWS. Supported by National Natural Science Foundation of China （Grant No.30672329）.