Sturge-Weber综合征2例报告并文献复习

目的观察Sturge-weber综合征的临床特点。方法报告2例Sturge-weber综合征,复习相关文献并探讨其病因、病理、临床表现、诊断和治疗。结果2例患者均表现为头痛和肢体抽搐,头路CT见颅内钙化,MR I等见软脑膜血管畸形等表现。结论Sturge-weber综合征是一种罕见的神经皮肤综合征,主要影响头部静脉,以颜面血管畸形、肢体抽搐和青光眼为主要表现。增强MR I是首选诊断方法,可显示病变结构和SW S病变范围。但患者的临床过程、治疗和预后各不相同。

Report of two cases of Sturge-Weber syndrome with a review of relative literatures

Objective: To investigate the clinical characteristics of Sturge-Weber syndrome(SWS).Methods: Two patients with SWS were reported and the literatures on SWS were retrospectively reviewed.Results: The main radiographic findings were intracranial calcification on CT and vascular malformation of leptomeningeal angiomatosis on MRI in 2 patients who suffered from headache and limb seizures.Conclusion: Sturge-Weber syndrome(SWS) is a rare neurocutaneous syndrome affecting the cephalic venous microvasculature,whose main manifestations were facial cutaneous vascular malformations,limb seizures,and glaucoma.Enhanced magnetic resonance imaging is the best method for diagnosing this syndrome,which could show pathologic structure and range of SWS.The clinical course,treatment and prognosis are varied.