目的依据国际通用的诊断标新，分析比较原发性中枢神经系统血管炎（primary central nervous system vasculitis，PCNSV）诊断手段。 方法收集2012年1月1日-2015年12月31日于首都医科大学附属北京天坛医院神经病学中心神经感染与免疫科住院治疗的PACNS患者共8例，回顾性分析患者临床资料，总结其临床表现、实验室检查、神经影像学及神经病理学特征。 结果临床表现：本组8例患者中，居于前五位的首发症状为慢性头痛、痫性发作、类卒中样局灶性神经功能缺损、精神行为异常和锥体外系症状，所有患者均未发现特异性的血生化及脑脊液异常结果，头颅磁共振成像（magnetic resonance imaging，MRI）均可见颅内异常信号，磁共振血管造影（magnetic resonance angiography，MRA）及数字减影血管造影（digital subtraction angiography，DSA）均未见明显异常。患者行脑组织活检，病理结果符合PCNSV表现。 结论 PCNSV的诊断要综合考虑患者的临床表现、实验室检查和影像学检查结果，脑组织活检的广泛开展，有助于提高本病的诊断率。%Objective To analyze and compare the diagnosis method of primary central nervous system vasculitis (PCNSV) according to the international standard. Methods A total of 8 cases who were admitted to Department of Neuro-infection and Immunology of Neurological Disease Center of Beijing Tiantan Hospital, Capital Medical University during Jan.1st, 2012 to Dec.31st, 2015 and diagnosed with PCNSV were reviewed. Their clinical descriptions, laboratory examinations, neuroimaging and pathological characteristic were analyzed. Results The most common clinical symptoms were: chronic headache, seizures, stroke like focal neurological deifcits, mental and behavioral abnormalities and symptoms of the external pyramidal system. No speciifc blood biochemical and cerebral spinal lfuid (CSF) abnormalities were found in all patients. Intracranial abnormal signal was seen in magnetic resonance imaging (MRI). Magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) were not significantly abnormal. Brain biopsy was performed in 8 patients and the pathologic results were consistent with PCNSV expression. Conclusion The diagnosis of PCNSV should consider the patient's clinical manifestations, laboratory tests and imaging findings. The extensive development of brain biopsy is helpful to improve the diagnostic rate of the disease.
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