Diagnosis and management of Guillain-Barré syndrome in ten steps

doi:10.3877/cma.j.issn.2095-655X.2023.03.010

Chinese Journal of Diagnostics(Electronic Edition) ›› 2023, Vol. 11 ›› Issue (03): 198-211. doi: 10.3877/cma.j.issn.2095-655X.2023.03.010

• Guideline and Criterion • Previous Articles Next Articles

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^†()

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 Online:2023-08-26 Published:2023-08-24
Contact: Bart C Jacobs

Abstract

Abstract:

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.

Key words: Guillain-Barré syndrome, Diagnosis, Management

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References 119

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变异类型	频率(GBS病例)(%)^a	临床特征	参考文献
经典的感觉运动型^b	30~85	快速进展的对称性肌无力和感觉体征，腱反射减弱或消失，通常在2周	11，24，113，114
纯运动型^c	5~70	肢体无力，无感觉体征	5，11，24
下肢瘫痪型	5~10	局限于下肢的瘫痪	10，24，114
咽-颈-臂型	<5	咽、颈、臂部肌肉无力，无下肢无力	10，113，114
双侧面神经麻痹伴感觉异常型^d	<5	双侧面部无力、感觉异常和腱反射减弱	113，114，115
纯感觉型^d	<1	急性或亚急性感觉神经病，无其他症状	116，117
米勒-费希尔综合征	5~25	眼肌麻痹、共济失调和腱反射消失。可出现仅有共济失调(急性共济失调神经病)或眼肌麻痹(急性眼肌麻痹)的不完全类型^[31]；估计有15%的患者与经典的感觉运动型GBS重叠	11，24，113，115，116，117，118
Bickerstaff脑干脑炎^d	<5	眼肌麻痹、共济失调、腱反射消失、锥体束体征和意识障碍，常与感觉运动型GBS发生重叠	113，114

变异类型	频率(GBS病例)(%)^a	临床特征	参考文献
经典的感觉运动型^b	30~85	快速进展的对称性肌无力和感觉体征，腱反射减弱或消失，通常在2周	11，24，113，114
纯运动型^c	5~70	肢体无力，无感觉体征	5，11，24
下肢瘫痪型	5~10	局限于下肢的瘫痪	10，24，114
咽-颈-臂型	<5	咽、颈、臂部肌肉无力，无下肢无力	10，113，114
双侧面神经麻痹伴感觉异常型^d	<5	双侧面部无力、感觉异常和腱反射减弱	113，114，115
纯感觉型^d	<1	急性或亚急性感觉神经病，无其他症状	116，117
米勒-费希尔综合征	5~25	眼肌麻痹、共济失调和腱反射消失。可出现仅有共济失调(急性共济失调神经病)或眼肌麻痹(急性眼肌麻痹)的不完全类型^[31]；估计有15%的患者与经典的感觉运动型GBS重叠	11，24，113，115，116，117，118
Bickerstaff脑干脑炎^d	<5	眼肌麻痹、共济失调、腱反射消失、锥体束体征和意识障碍，常与感觉运动型GBS发生重叠	113，114

确诊级别1	确诊级别2	确诊级别3
双侧肢体弛缓性瘫痪	双侧肢体弛缓性瘫痪	双侧肢体弛缓性瘫痪
无力肢体腱反射减退或消失	无力肢体腱反射减退或消失	无力肢体腱反射减退或消失
单相病程，从发病到疾病高峰期的时间间隔为12 h~28 d，高峰期后有临床平台期	单相病程，从发病到疾病高峰期的时间间隔为12 h~28 d，高峰期后有临床平台期	单相病程，从发病到疾病高峰期的时间间隔为12 h~28 d，高峰期后有临床平台期
电生理符合GBS的特征	脑脊液细胞数<50个/μL(蛋白水平>正常值或正常)	肌无力无法用其他疾病解释
脑脊液蛋白水平>正常值，而细胞数<50个/μL	若无脑脊液的结果，电生理符合GBS的特征
肌无力无法用其他疾病解释	肌无力无法用其他疾病解释

确诊级别1	确诊级别2	确诊级别3
双侧肢体弛缓性瘫痪	双侧肢体弛缓性瘫痪	双侧肢体弛缓性瘫痪
无力肢体腱反射减退或消失	无力肢体腱反射减退或消失	无力肢体腱反射减退或消失
单相病程，从发病到疾病高峰期的时间间隔为12 h~28 d，高峰期后有临床平台期	单相病程，从发病到疾病高峰期的时间间隔为12 h~28 d，高峰期后有临床平台期	单相病程，从发病到疾病高峰期的时间间隔为12 h~28 d，高峰期后有临床平台期
电生理符合GBS的特征	脑脊液细胞数<50个/μL(蛋白水平>正常值或正常)	肌无力无法用其他疾病解释
脑脊液蛋白水平>正常值，而细胞数<50个/μL	若无脑脊液的结果，电生理符合GBS的特征
肌无力无法用其他疾病解释	肌无力无法用其他疾病解释

指标	类别	评分(分)
肌无力发病和入院之间的间隔天数	>7 d	0
	4~7 d	1
	≤3 d	2
入院时面部和/或延髓麻痹	无	0
	有	1
入院时MRC总分	51~60分	0
	41~50分	1
	31~40分	2
	21~30分	3
	≤20分	4

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评分	临床表现
0	正常
1	轻微的症状或体征，能跑或进行手工劳动
2	能独立行走10 m以上的距离，但不能跑或进行手工劳动
3	能在辅助支持(手杖等)下行走10 m以上的距离
4	只能卧床或坐在轮椅上
5	需要辅助通气治疗(至少在1天的某个时间段内)
6	死亡

并发症	出现以下情况时需警惕
窒息	延髓性麻痹
心律不齐	所有患者
医源性感染(如肺炎、脓毒症或尿路感染)	延髓和面神经麻痹、不能活动、膀胱功能障碍、机械通气
疼痛和触觉异常性疼痛	沟通障碍
谵妄	沟通障碍
抑郁	沟通障碍
尿潴留	所有患者
便秘	不能活动
角膜溃疡	面神经麻痹
饮食不足引发的营养不良	延髓和面神经麻痹
低钠血症	所有患者
压疮	不能活动
压迫性神经病	不能活动
肢体挛缩和骨化	长期重度无力

预测因子	分类	得分(分)
发病年龄(岁)	≤40	0
	41~60	1
	>60	2
前驱腹泻史	无	0
	有	1
入院7 d时的MRC总分^*(分)	51~60	0
	41~50	3
	31~40	6
	0~30	9

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