原发性中枢神经系统血管炎9例

Clinical analysis of nine cases of primary angiitis of central nervous system

目的初步探讨原发性中枢神经系统血管炎的临床特点及全身和局部免疫抑制治疗方法。方法对北京协和医院9例诊断为原发性中枢神经系统血管炎的患者进行回顾性分析。结果患者平均发病年龄35岁,临床表现多种多样,脑脊液异常者8例,血沉和C反应蛋白升高者4例。所有患者均有头颅磁共振(MRI)或磁共振血管成像(MRA)的异常。多数患者激素及环磷酰胺治疗有效,部分患者鞘内注射地塞米松和甲氨蝶呤显效。结论原发性中枢神经系统血管炎是一种罕见的主要累及中枢神经系统的自身免疫性疾病,诊断需结合临床、实验室、影像学和病理。

Objective To discuss clinical characters,therapies of primary angiitis of the central nervous system （PACNS）. Methods Retrospectively review was made on nine PACNS patients who were inhospitalized in recent nine years in Peking Union Medical College Hospital. Results The mean age of onset was 35 years old. The presented various central nervous system（CNS） symptoms, abnormal cerebral spinal fluid was found in eight patients. Four patients had increased erythrocyte sedimentation rate（ESR） and C reactive protein（CRP）. Magnetic resonance imaging （MRI） and angio-MRI were abnormal in all the patients. Most patients showed improvement under treatment of corticostiroid and cyclophosphomide. Intrathecal injection of dexamethasone and methotrexate was also an effective therapy. Conclusion PACNS is a rare rheumatic disease which involves predominantly in CNS. Comprehensive analysis of clinical manifestations,laboratory findings, MRI or angio-MRI and histopathological results could help diagnosis.