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中华诊断学电子杂志 ›› 2023, Vol. 11 ›› Issue (03) : 198 -211. doi: 10.3877/cma.j.issn.2095-655X.2023.03.010

指南与规范

吉兰-巴雷综合征诊治的10个步骤(Diagnosis and management of Guillain-Barré syndrome in ten steps全译)
Sonja E Leonhard, Melissa R Mandarakas, Francisco AA Gondim, Kathleen Bateman, Maria LB Ferreira, David R Cornblath, Pieter A van Doorn, Mario E Dourado, Richard AC Hughes, Badrul Islam, Susumu Kusunoki, Carlos A Pardo, Ricardo Reisin, James J Sejvar, Nortina Shahrizaila, Cristiane Soares, Thirugnanam Umapathi, 王玉忠, Eppie M Yiu, Hugh J Willison, Bart C Jacobs()   
  1. 3015 CE 鹿特丹市,伊拉斯姆斯大学医疗中心神经内科
    60430-370 塞阿拉州,福塔莱萨联邦大学沃尔特·坎迪奥大学医院
    7925 开普敦市,开普敦大学格鲁特·舒尔医院
    52171-011 伯南布哥州,累西腓市康复医院神经内科
    21218 马里兰州,约翰·霍普金斯大学医学院神经病学系
    59012-300 纳塔尔市,北里奥格兰德州联邦大学奥诺弗雷卢佩斯医院综合医学部
    WC1N 3BG 伦敦市,伦敦大学学院皇后广场神经病学研究所
    1212 达卡市,国际腹泻病研究中心
    589-8511 大阪市,金代大学医学院
    C1280AEB 布宜诺斯艾利斯市,不列颠医院
    30329 乔治亚州,亚特兰大市疾病预防控制中心
    50603 吉隆坡市,马来亚大学神经病学系
    20221-903 里约热内卢市,埃斯塔多联邦医院
    308433 新加坡,国家神经科学研究所
    272029 济宁,济宁医学院附属医院神经内科
    3052 维多利亚州,墨尔本皇家儿童医院神经内科;3052 维多利亚州,默多克儿童研究所神经科学研究组;3052 维多利亚州,墨尔本大学儿科学系
    G61 1QH 格拉斯哥市,格拉斯哥大学医学、兽医学与生命科学学院
    3015 CE 鹿特丹市,伊拉斯姆斯大学医疗中心神经内科;3015 GD 鹿特丹市,伊拉斯姆斯大学医疗中心免疫科
  • 收稿日期:2023-02-27 出版日期:2023-08-26
  • 通信作者: Bart C Jacobs

Diagnosis and management of Guillain-Barré syndrome in ten steps

Sonja E Leonhard, Melissa R Mandarakas, Francisco AA Gondim, Kathleen Bateman, Maria LB Ferreira, David R Cornblath, Pieter A van Doorn, Mario E Dourado, Richard AC Hughes, Badrul Islam, Susumu Kusunoki, Carlos A Pardo, Ricardo Reisin, James J Sejvar, Nortina Shahrizaila, Cristiane Soares, Thirugnanam Umapathi, Wang Yuzhong, Eppie M Yiu, Hugh J Willison, Bart C Jacobs()   

  1. Department of Neurology, Erasmus University Medical Center, Rotterdam 3015 CE, Netherlands
    Hospital Universitário Walter Cantidio, Universidade Federal do Ceará, Fortaleza, Ceará 60430-370, Brazil
    Groote Schuur Hospital, University of Cape Town, Cape Town 7925, South Africa
    Department of Neurology, Hospital da Restauração, Recife, Pernambuco 52171-011, Brazil
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21218, USA
    Department of Integrative Medicine, Hospital Universitário Onofre Lopes, Universidade Federal do Rio Grande do Norte, Natal 59012-300, Brazil
    UCL Queen Square Institute of Neurology, University College London, London WC1N 3BG, UK
    International Centre for Diarrhoeal Disease Research, Bangladesh, Dhaka 1212, Bangladesh
    Kindai University Faculty of Medicine, Osaka 589-8511, Japan
    Hospital Británico, Buenos Aires C1280AEB, Argentina
    Centers for Disease Control and Prevention, Atlanta, GA 30329, USA
    Department of Neurology, University of Malaya, Kuala Lumpur 50603, Malaysia
    Hospital Federal dos Servidores do Estado, Rio de Janeiro 20221-903, Brazil
    National Neuroscience Institute, Singapore 308433, Singapore
    Department of Neurology, Affiliated Hospital of Jining Medical University, Jining 272029, China
    Department of Neurology, The Royal Children′s Hospital Melbourne, Melbourne, VIC 3052, Australia; Neurosciences Research, Murdoch Children′s Research Institute, Melbourne, VIC 3052, Australia; Department of Paediatrics, The University of Melbourne, Melbourne, VIC 3052, Australia
    College of Medicine, Veterinary and Life Sciences, University of Glasgow, Glasgow G61 1QH, UK
    Department of Neurology, Erasmus University Medical Center, Rotterdam 3015 CE, Netherlands; Department of Immunology, Erasmus University Medical Center, Rotterdam 3015 GD, Netherlands
  • Received:2023-02-27 Published:2023-08-26
  • Corresponding author: Bart C Jacobs
引用本文:

Sonja E Leonhard, Melissa R Mandarakas, Francisco AA Gondim, Kathleen Bateman, Maria LB Ferreira, David R Cornblath, Pieter A van Doorn, Mario E Dourado, Richard AC Hughes, Badrul Islam, Susumu Kusunoki, Carlos A Pardo, Ricardo Reisin, James J Sejvar, Nortina Shahrizaila, Cristiane Soares, Thirugnanam Umapathi, 王玉忠, Eppie M Yiu, Hugh J Willison, Bart C Jacobs. 吉兰-巴雷综合征诊治的10个步骤(Diagnosis and management of Guillain-Barré syndrome in ten steps全译)[J]. 中华诊断学电子杂志, 2023, 11(03): 198-211.

Sonja E Leonhard, Melissa R Mandarakas, Francisco AA Gondim, Kathleen Bateman, Maria LB Ferreira, David R Cornblath, Pieter A van Doorn, Mario E Dourado, Richard AC Hughes, Badrul Islam, Susumu Kusunoki, Carlos A Pardo, Ricardo Reisin, James J Sejvar, Nortina Shahrizaila, Cristiane Soares, Thirugnanam Umapathi, Wang Yuzhong, Eppie M Yiu, Hugh J Willison, Bart C Jacobs. Diagnosis and management of Guillain-Barré syndrome in ten steps[J]. Chinese Journal of Diagnostics(Electronic Edition), 2023, 11(03): 198-211.

吉兰-巴雷综合征(GBS)是一种少见但可危及生命的、自身免疫介导的周围神经病。GBS通常由感染引发,发病率可在感染性疾病暴发期间增加,如2013年法属波利尼西亚和2015年拉丁美洲的寨卡病毒流行期间,该病的发病率明显上升。由于GBS的临床表现和病程多种多样,其诊疗存在一定的复杂性,而目前尚无可用的国际性临床诊疗指南。本文作者撰写了一份适用于全球的GBS诊疗指南,特别是在疾病暴发期间,以期为临床医生提供指导。本指南根据当前文献和专家共识撰写而成,采用10步式结构,以便在临床实践中更好地应用。本文首先介绍了GBS的诊断标准、临床变异和鉴别诊断;其次,通过10个步骤介绍了GBS的早期识别和诊断、收治重症监护室的指征、治疗指征和选择、疾病进展的监测和处理、临床病程和结局的预测,以及并发症和后遗症的处理。

Guillain-Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicableguideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.

图1 吉兰-巴雷综合征诊疗的10个步骤注:本要点总结概述了指南中描述的10个步骤。*为应根据临床变化确定监测频率,并对个体患者进行评估。CSF为脑脊液;EGRIS为Erasmus GBS呼吸功能不全评分(附录3);GBS为吉兰-巴雷综合征;ICU为重症监护病房;mEGOS为改良版Erasmus GBS结局评分(补充表3);MFS为米勒-费希尔综合征
图2 吉兰-巴雷综合征各种变异的症状形式注:图示为GBS不同临床变异中观察到的典型症状形式。症状可能为纯运动型、纯感觉型(罕见)或运动-感觉混合型。米勒-费希尔综合征患者可出现共济失调,Bickerstaff脑干脑炎患者可同时出现意识水平降低和共济失调。症状可局限于身体的特定部位,GBS不同变异型之间的临床表现可以不同。尽管不符合GBS的诊断标准,双侧面神经麻痹伴感觉异常、纯感觉性变异型和Bickerstaff脑干脑炎也包含在GBS谱系中。经许可,本图改编自参考文献[112];GBS为吉兰-巴雷综合征
表1 吉兰-巴雷综合征的变异型
附录1 吉兰-巴雷综合征的诊断标准
附录2 GBS的鉴别诊断
中枢神经系统
脑干炎症或感染(如结节病、干燥综合征、视神经脊髓炎或髓鞘少突胶质细胞糖蛋白抗体相关疾病),需注意Bickerstaff脑干脑炎
脊髓炎症或感染(如结节病、干燥综合征或急性横贯性脊髓炎)
恶性肿瘤(如软脑膜转移或神经淋巴瘤病)
脑干或脊髓卡压
脑干卒中
维生素缺乏(如维生素B1缺乏造成的韦尼克脑病a或维生素B12缺乏造成的脊髓亚急性联合变性)
前角细胞
急性弛缓性脊髓炎(如由脊髓灰质炎病毒、肠病毒D68或A71、西尼罗河病毒、日本脑炎病毒或狂犬病毒引起)
神经根
感染(如莱姆病、巨细胞病毒、HIV、EB病毒或水痘带状疱疹病毒)
压迫
软脑膜恶性肿瘤
周围神经
慢性炎症性脱髓鞘性多发性神经病
代谢或电解质紊乱(如低血糖、甲状腺功能减退、卟啉病或铜缺乏)
维生素缺乏(如维生素B1、B12或E缺乏)
中毒(如药物、酒精、维生素B6、铅、铊、砷、有机磷酸酯、乙二醇、二甘醇、甲醇或正己烷)
危重症多发性神经病
神经痛性肌萎缩
血管炎
感染(如白喉或HIV)
神经肌肉接头
重症肌无力
兰伯特-伊顿肌无力综合征
神经毒素(如肉毒杆菌中毒、破伤风、蜱虫病或蛇咬伤中毒)
有机磷中毒
肌肉
代谢或电解质紊乱(如低钾血症、甲状腺毒性低钾型周期性瘫痪、低镁血症或低磷酸盐血症)
炎症性肌病
急性横纹肌溶解
药物诱导性肌病(如秋水仙碱、氯喹、依米丁或他汀类药物诱导)
线粒体疾病
其他
转换障碍或功能性障碍
补充表1 吉兰-巴雷综合征的布莱顿协作组分层诊断标准
附录3 Erasmus GBS呼吸功能不全评分
补充表2 吉兰-巴雷综合征残疾评分
表2 吉兰-巴雷综合征中重要的并发症
补充表3 改良的Erasmus GBS结局评分(mEGOS)
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