摘要
目的探讨Sturge-Weber综合征（SWS）的临床特征和影像学特点。方法选取自2012年6月至2015年11月徐州医科大学附属医院神经内科收治的符合SWS诊断标准的8例住院患者的临床及影像学资料进行回顾性分析。8例患者均予以药物治疗，其中1例行“青光眼复合式小梁切除术”，1例行“开颅血管畸形切除术”。结果SWS患者中表现为面部血管瘤样改变7例，癫痫发作5例，头痛4例，肢体偏瘫3例，青光眼3例，甲状腺功能减退1例。影像学显示头颅CT及脑血管成像显示皮层下钙化，软脑膜血管迂曲增多；磁共振常规及T1序列增强显示脑萎缩．脑回样强化；磁敏感加权成像示脑皮质多发极低信号影。眼底检查发现眼底脉络膜血管畸形及视神经萎缩。治疗后所有患者临床症状均好转。结论表现为典型的青光眼、癫痫或偏瘫的青年患者，无论有无颜面部血管瘤均需高度怀疑SWS，需进一步完善头颅CT血管成像、磁共振检查（特别是磁敏感加权成像序列及增强）以确诊，对于药物治疗无效的部分癫痫患者可手术治疗。
Objective To explore the clinical features and radiological findings of patients with Sturge-Weber syndrome （SWS）. Methods A retrospective study was performed on the course of illness, clinical data and neuroimages of 8 patients with SWS, admitted to our hospital from June 2012 to November 2015. Results The main symptoms included facial hemangioma in 7 patients, epilepsy in 5 patients, headache in 4 patients, limb hemiplegia in 3 patients, glaucoma in 3 patients, and thyroid dysfunction in one patient. Neurological imaging showed subcortical calcification, brain atrophy, gyriform enhancement and increased pial vascular tortuosity. Fundus examination revealed optic atrophy and vascular malformations of choroid. All symptoms in the patients were alleviated after treatment. Conclusions SWS should be highly suspected in young patients who present with typical glaucoma, epilepsy or hemiplegia with/without facial hemangioma. CT angiography or MR imaging （especially susceptibility-weighted imaging） is required to confirm the diagnosis. If medications fail to control seizures, surgery could be considered in some patients.
出处
《中华神经医学杂志》
CAS
CSCD
北大核心
2016年第10期1026-1031,共6页
Chinese Journal of Neuromedicine
基金
国家自然科学基金（81271268、81571469）
