中枢神经系统原发性血管炎(附9例报告及文献复习)

Primary angiitis of central nervous system (9 cases report with literature review)

目的 探讨中枢神经系统原发性血管炎 (PACNS)的临床、影像学及病理特点。方法 回顾分析 9例诊断为中枢神经系统原发性血管炎患者的临床表现、MRI、血管造影、病理活检以及治疗转归情况并结合文献分析其特点。结果 PACNS患者呈急性或亚急性起病 ,病程可以为进展性或缓解复发交替 ,临床表现以头痛、智能减退、偏瘫及癫痫样发作为主。实验室检查无特殊发现。MRI以大脑半球长T1长T2 信号的类圆形病变为主 ,多无占位效应 ,可见强化。病理检查以淋巴细胞性血管炎为主。 80 %患者应用皮质类固醇激素和免疫抑制剂联合治疗 6个月 ,病情控制预后良好。结论 PACNS临床表现多样 ,通过临床、影像学以及病理活检等联合应用可进行生前诊断。

Objective To investigate the clinical characteristics,neuroimaging,and pathological features.Methods In cases of 9 patients with PACNS were retroactively studied.The records of clinical manifestations, MRI, angiography,brain biopsy,therapy and prognosis wereanalyzed combining with literatures.Results All 9 patientswere attacked acutely or subacutly with progressive or relapsing-remitting courses.Headache,hemiplegia and seizure were mainly manifestations.MRI showed oval lesions which can be enhanced by Gd in cerebral hemisphere without mass effect.Brain biopsy revealed lymphocytic angiitis.Steroid combined with immunosuppressor were effective for about 80% patients Conclusion The manifestations of PACNS are various.The diagnosis relies on the combination of multiple methods.Steroid combined with immunosuppressor can improve patient's condition.