Objective To summarize the characteristics of primary central nervous system vasculitis from clinical, imaging and pathological aspects by retrospective study. Methods From March 2012 to December 2014, the data of the inpatients of primary central nervous system vasculitis in Beijing Tiantan Hospital were collected, and their clinical manifestation, imaging and pathological characteristics were analyzed by using a descriptive method. Results There were 9 patients, 5 males (55.56%) and 4 females (44.44%) separatively. The age ranges from 10 years old to 47 year old, with the median age of 30 years old. There were 4 cases (44.44%) of epileptic seizure, 3 cases (33.33%) of abnormal behavior and cognition, 5 cases (55.56%) with sensorimotor abnormalities, 2 cases (22.22%) with dizziness, 2 cases (22.22%) with headache, 1 case (11.11%) with facial pain, 1 case (11.11%) with blurred vision and 1 case (11.11%) with unstable walking. There were 4 patients (44.44%) with cerebral spinal lfuid (CSF) abnormalities. There were 6 cases (66.67%) with bilateral lesions, 3 cases (33.33%) with unilateral lesions, 9 cases (100%) involved the frontal lobe, 5 cases (55.56%) involved the parietal lobe,4 cases (44.44%) involved the temporal and occipital lobe, 6 cases (66.67%) combined with subcortical white matter involvement, 3 cases (33.33%) combined with meningeal involvement, 1 case (11.11%) complicated with basal ganglia involvement and 1 case (11.11%) complicated with spinal cord involvement. The lesions were 8 cases (88.89%) with unclear border and 1 case (11.11%) with clear border. There were 3 cases (33.33%) with cortical atrophy. There were 6 cases (66.67%) with the enhancement of the lesions and meningeal. 57.14% (4/7 patients) showed that the lesions were low signal in T2*/susceptibility weighted imaging (SWI) sequence of magnetic resonance imaging (MRI). Conclusion The clinical manifestation and imaging in primary central nervous system vasculitis are diverse. It is important characteristics of MRI that the meningeals with lesion are enhanced and the lesions in T2*/SWI sequence are low signal. The brain biopsy is still the important diagnostic method.%目的：总结原发性中枢神经系统血管炎临床、磁共振成像（magnetic resonance imaging，MRI）和病理学特点。方法收集2012年3月～2014年12月首都医科大学附属北京天坛医院神经病学中心收治的原发性中枢神经系统血管炎患者资料，采用描述性方法对其临床表现、MRI及病理学特点进行分析。结果共收集9例患者，其中男性5例（55.56%），女性4例（44.44%），年龄范围10～47岁，中位年龄30岁。痫性发作4例（44.44%），行为认知异常3例（33.33%），局灶性感觉运动异常5例（55.56%），头晕2例（22.22%），钝性头痛2例（22.22%），面部疼痛1例（11.11%），视物模糊1例（11.11%），行走不稳1例（11.11%）。腰穿异常4例（44.44%）。MRI表现为双侧病灶6例（66.67%），单侧病灶3例（33.33%），其中受累部位分别为额叶9例（100%），顶叶5例（55.56%），颞枕叶4例（44.44%），合并皮层下白质受累6例（66.67%），合并脑膜/脊膜受累3例（33.33%），合并基底节受累1例（11.11%），合并脊髓受累1例（11.11%）；病变边界不清8例（88.89%），边界清1例（11.11%）；皮层萎缩伴脑室扩大3例（33.33%）；病灶及脑脊膜强化6例（66.67%）；7例患者行磁共振T2*或磁敏感加权序列（susceptibility weighted imaging，SWI），其中病灶表现低信号4例（57.14%）。2例行脑病理学检查，表现为脑实质水肿，血管周围炎性细胞浸润，血管壁坏死和胶质增生。结论原发性中枢神经系统血管炎临床表现及影像学多样，病灶合并脑膜和（或）脊膜强化，以及磁共振T2*或SWI序列低信号是其重要影像学特征，脑活检仍是目前重要的确诊手段。
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