重症肌无力危象前状态管理专家共识(2024)  

Expert Consensus on the Management of Impending Myasthenic Crisis(2024)

作　　者：罗苏珊 周昊[2,15] 岳耀先 卜碧涛 常婷[5,15] 邓晖 段瑞生 笪宇威[8,15] 冯慧宇 管宇宙 王玉鸽[11,15] 杨春生 张华[13,15] 周红雨 赵重波[1,15] 杨欢 LUO Su-shan;ZHOU Hao;YUE Yao-xian;BU Bi-tao;CHANG Ting;DENG Hui;DUAN Rui-sheng;DA Yu-wei;FENG Hui-yu;GUAN Yu-zhou;WANG Yu-ge;YANG Chun-sheng;ZHANG Hua;ZHOU Hong-yu;ZHAO Chong-bo;YANG Huan(Department of Neurology,Huashan Hospital Affiliated to Fudan University,National Center for Neurological Diseases(NCND),Shanghai 200040,China;Department of Neurology,Xiangya Hospital,Central South University,Changsha 410008,China;Department of Neurology,Qilu Hospital,Shandong University,Qingdao 266035,China;Department of Neurology,Tongji Hospital Afiliated to Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430030,China;Department of Neurology,the Second Affliated Hospital of Air Force Military Medical University,Xi'an 710038,China;Department of Neurology,the First Bethune Hospital of Jilin University,Changchun 130021,China;Department of Neurology,the First Afiliated Hospital of Shandong University,Jinan 250014,China;Department of Neurology,Xuanwu Hospital,Capital Medical University,Beijing 100053,China;Department of Neurology,the First Affiliated Hospital of Sun Yatsen University,Guangzhou 510080,China;Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Neurology,the Third Affiliated Hospital of Sun Yat-sen University,Guangzhou 510635,China;Department of Neurology,Tianjin Medical University,General Hospital,Tianjin 300052,China;Department of Neurology,Beijing Hospital,Beijing 100730,China;Department of Neurology,West China Hospital of Sichuan University,Chengdu 610041,China;Chinese Myasthenia Gravis Collaborative Group(CMGCG),Beijing 100022,China)

机构地区：[1]复旦大学附属华山医院神经内科,国家神经疾病医学中心,上海200040 [2]中南大学湘雅医院神经内科,长沙410008 [3]山东大学齐鲁医院(青岛)神经内科,青岛266035 [4]华中科技大学同济医学院附属同济医院神经内科,武汉430030 [5]空军军医大学第二附属医院神经内科,西安710038 [6]吉林大学白求恩第一医院神经内科,长春130021 [7]山东第一医科大学第一附属医院神经内科,济南250014 [8]首都医科大学宣武医院神经内科,北京100053 [9]中山大学附属第一医院神经内科,广州510080 [10]中国医学科学院北京协和医院神经内科,北京100730 [11]中山大学附属第三医院神经内科,广州510635 [12]天津医科大学总医院神经内科,天津300052 [13]北京医院神经内科,北京100730 [14]四川大学华西医院神经内科,成都610041 [15]中国罕见病联盟/北京罕见病诊疗与保障学会重症肌无力协作组,北京100022

出　　处：《中国临床神经科学》2024年第3期241-251,共11页Chinese Journal of Clinical Neurosciences

摘　　要：重症肌无力(MG)是一种自身免疫性神经-肌肉接头疾病,肌无力危象(MC)是MG患者的最严重状态,显著增加患者死亡风险和疾病负担,如能及时识别和干预MG危象前状态,阻止疾病向MC进展,其临床意义重大。然而MG危象前状态的诊断尚无统一标准,对其治疗管理亦缺乏高级别证据。为此,中国MG协作组专家基于新近研究结果和临床实践经验,经过反复讨论,围绕MG危象前状态的早期识别、诊断标准、治疗干预及随访管理等形成共识,以期为临床改善MG危象前状态的管理及预后提供参考。Myasthenia gravis(MG)is an autoimmune neuromuscular disease,and myasthenic crisis(MC)is the most severe state of MG,significantly increasing the risk of death and disease burden for patients.If the impending myasthenic crisis of MG can be identified and intervened in time,it can prevent the disease from progressing to MC.However,there is no concensus for the diagnosis of impending MC,and there is also a lack of high-level evidence for its treatment and management.Therefore,based on recent research results and clinical practice experience,experts from the Chinese Myasthenia Gravis Collaborative Group(CMGCG),after repeated discussions,formed a consensus on the early identification,diagnostic criteria,therapeutic intervention,and follow-up management of impending MC,in order to provide a reference for improving the management and prognosis of the impending MC in clinical practice.

关 键 词：重症肌无力 肌无力危象 危象前状态 管理 共识 

分 类 号：R746.1[医药卫生—神经病学与精神病学]