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原发性中枢神经系统血管炎的多学科诊治意义探讨(附1例报道并文献复习)
引用本文:黄,成,徐国政,宋,健,黄文才,任,勇,吴晓飞,熊,飞,张,鹏.原发性中枢神经系统血管炎的多学科诊治意义探讨(附1例报道并文献复习)[J].中国临床神经外科杂志,2019,0(11):665-668.
作者姓名:    徐国政      黄文才      吴晓飞        
作者单位:4360070 武汉,中国人民解放军中部战区总医院神经外科(黄 成、徐国政、宋 健),影像医学科(黄文才、熊 飞),病理科(任 勇、张 鹏),神经内科(吴晓飞)
摘    要:目的 探讨原发性中枢神经系统血管炎(PACNS)的病因、临床特点、诊断、治疗方法以及多学科协作在其诊治过程中的相关意义。方法 回顾性分析1例PACNS的多学科联合诊治过程,并复习相关文献,总结治疗经验。结果 最开始,结合临床表现、影像学表现及活检结果,考虑炎性脱髓鞘病,甲强龙冲击治疗无效;请神经内科、影像科、病理科会诊,再次活检结果仍为炎性病变,结合炎性脱髓鞘治疗过程,基本排除炎性脱髓鞘。经北京宣武医院病理科诊断为PACNS,再次请神经内科会诊,进行系统免疫治疗(甲强龙+免疫抑制剂他克莫司),3个月后复查头部MRI增强示病变明显缩小,病人神志清楚,失语症状较前改善,左侧中枢性面瘫较前好转,左侧肢体肌力较前恢复。结论 PACNS是一种罕见病,其临床表现、影像学表现无特异性,目前病理活检是其主要诊断手段,治疗过程中,多学科协作,可明显提高诊治效率,大幅提高诊治水平。

关 键 词:原发性中枢神经系统血管炎  诊断  治疗  多学科协作模式

Disscussion on the meanings of multidisciplinary way in diagnosis and treatment of primary angiitis of the central nervous system (report of one case and literature review)
HUANG Cheng,XU Guo-zheng,SONG Jian,HUANG Wen-cai,REN Yong,WU Xiao-fei,ZHANG Peng..Disscussion on the meanings of multidisciplinary way in diagnosis and treatment of primary angiitis of the central nervous system (report of one case and literature review)[J].Chinese Journal of Clinical Neurosurgery,2019,0(11):665-668.
Authors:HUANG Cheng  XU Guo-zheng  SONG Jian  HUANG Wen-cai  REN Yong  WU Xiao-fei  ZHANG Peng
Affiliation:1. Department of Neurosurgery, General Hospital, Central Theater, PLA, Wuhan 430070, China; 2. Department of Radiology, General Hospital, Central Theater, PLA, Wuhan 430070, China; 3. Department of Pathology, General Hospital, Central Theater, PLA, Wuhan
Abstract:Objective To explore the clinical characteristics, diagnosis and treatment of primary angiitis of the central nervous system (PACNS). Methods The clinical data of one patient with PACNS was analyzed retrospectively. The experiences were summarized in diagnosing and treating PACNS. Results Head MRI enhancement 0.5 and 3 months after the treatment with the hormone and immune inhibitor showed that the intracranial lesions significantly shrank. The patients was conscious, and the aphasia symptoms was improved, the central facial paralysis in the left side was relieved and the muscle power of the left limbs retained to grade Ⅴ-level 3 months after the treatment. Conclusions The PACNS is a rare disease without specific clinical manifestations and imaging features. The pathological biopsy is the main method to diagnose the PACNS
Keywords:Primary angiitis of the central nervous system  Multidisciplinary team  Diagnosis  Treatment
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