中文摘要：

目的探讨肾病综合征（NS）并发脑静脉窦血栓（CVST）患者的临床特征及预后。方法回顾性分析2007年1月—2011年1月,南京军区南京总医院神经内科及肾脏病科住院的14例NS并发CVST患者的临床资料,对患者的临床表现、影像学、实验室检查、治疗及随访结果进行分析。结果①患者年龄为13～62岁,平均为（31±16）岁。主要临床表现为头痛、呕吐、运动功能障碍、癫痫发作。②CT/MRI/MRV提示CVST阳性比为13/14,1例患者在MR不能确诊的情况下,行DSA检查确诊。病变部位主要为上矢状窦,其次为横窦。③有12例患者的血清白蛋白〈25g/L,8例血浆纤维蛋白原〉4 g/L,8例D-二聚体〉500mg/L。经抗凝治疗后,FIB和D-二聚体降低,与治疗前比较,差异有统计学意义（P〈0.05）。④对14例患者随访12～60个月,平均（29±14）个月。对8例患者复查头部MRI/MRV,有6例显示闭塞的静脉或静脉窦再通。临床痊愈13例,改良Rankin评分（mRS）均为0分;遗留头痛的患者1例,mRS为1分。无一例CVST复发。结论 NS患者若出现头痛、运动功能障碍、癫痫发作,应及时行影像学检查明确是否为CVST。对于并发CVST,早期诊断及抗凝治疗,预后良好。

英文摘要：

Objective To investigate the clinical features and prognosis in patients with nephrotic syndrome （NS） complicated with cerebral venous sinus thrombosis （CVST）. Methods The clinical data of 14 patients with NS complicated with CVST admitted in the depamnents of neurology and nephrology, Jin-ling Hospital, Nanjing, China from January 2007 to January 20ll were analyzed retrospectively. The clinical manifestations, imaging, laboratory tests, therapeutic management and follow-up results of the patients were analyzed. Results ①The age range of the patients was 13-62 years old （mean age, 31 ± 16）. Their main clinical manifestations were headache, vomiting, motor dysfunction, and seizures. ②CT/MRI/MRV suggested that the positive ratio of CVST was 13/14. Under the circumstances of failing to diagnose by MRI, 1 patient was diagnosed by DSA. The sites of lesion were mainly in the superior sagittal sinus, followed by the transverse sinus. @Serum albumin of 12 patients was less than 25 g/L, and plasma fibrinogen of 8 pa-tients was higher than 4 g/L. @14 patients were followed up for 12 -60 months （mean 29 ±14 months）. Head MRI/MRV was reexamined in 8 patients, 6 of them showed reeanalization of originally occluded vein or venous sinus. Clinical cure were happened in 13 patients （mRS 0） ; only 1 patient had headache （mRS 1 ）.There was no recurrence of CVST found. Conclusion If the patients with NS experience headache, motor dysfunction, and seizures, neuroimaging examination should be performed timely in order to identify whether it is CVST or not. Early diagnosis and early anticoagulant therapy may have a good outcome for patients compli-cated with CVST.