Diagnosis and management of Guillain-Barré syndrome in ten steps

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

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 applicable guideline 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.

Conflict of interest statement

B.C.J. has received funding from Annexon Biosciences, Baxter, CSL Behring, Hansa Biopharma and Grifols. D.R.C. has received consultancy honoraria from Annexon Biosciences, argenx, Biotest Pharmaceuticals, Cigna Health Management, CSL Behring, DP Clinical, Grifols, Hansa Biopharma, New Enterprise Associates, Octapharma, Pharnext SAS, Polyneuron Pharmaceuticals, Seattle Genetics, Stealth BioTherapeutics, and Syntimmune. D.R.C is also on a data safety monitoring board for Pfizer, Sanofi, Alnylam Pharmaceuticals, PledPharma, and Momenta Pharma. Johns Hopkins University has licensed technology to AstraZeneca Pharmaceuticals, Genentech, Levicept, Seattle Genetics, Merrimack Pharmaceuticals, Levicept and Disarm Therapeutics, for which D.R.C. receives royalties. P.A.v.D. received a grant from Sanquin Blood supply for the SID-GBS randomized controlled trial, and a grant from Grifols to conduct the I-SID GBS study. R.A.C.H. has received consultancy honoraria from Laboratoire Francais du Fractionnement et des Biotechnologies (LFB S.A.). S.K. has received speaker honoraria from Teijin Pharma, Nihon Pharmaceutical and Japan Blood Products Organization, and research support from Nihon Pharmaceutical and Japan Blood Products Organization. R.R. is supported by a grant from CSL Behring. N.S. receives research support from Hovid Berhad. The other authors declare no competing interests.

Figures

Fig. 1. Ten-step approach to the diagnosis…
Fig. 1. Ten-step approach to the diagnosis and management of Guillain–Barré syndrome.
This bullet point summary provides an overview of each of the ten steps described in the guideline. *Frequency of monitoring is dependent on the clinical picture and should be assessed in individual patients. CSF, cerebrospinal fluid; EGRIS, Erasmus GBS Respiratory Insufficiency Score (Box 3); GBS, Guillain-Barré syndrome; ICU, intensive care unit; mEGOS, modified Erasmus GBS Outcome Score (Supplementary Table 3).
Fig. 2. Pattern of symptoms in variants…
Fig. 2. Pattern of symptoms in variants of Guillain–Barré syndrome.
Graphic representation of the pattern of symptoms typically observed in the different clinical variants of Guillain–Barré syndrome (GBS). Symptoms can be purely motor, purely sensory (rare) or a combination of motor and sensory. Ataxia can be present in patients with Miller Fisher syndrome and both decreased consciousness and ataxia can be present in patients with Bickerstaff brainstem encephalitis. Symptoms can be localized to specific regions of the body, and the pattern of symptoms differs between variants of GBS. Although bilateral facial palsy with paraesthesias, the pure sensory variant and Miller Fisher syndrome are included in the GBS spectrum, they do not fulfil the diagnostic criteria for GBS. Adapted with permission from ref., ©2019 BMJ Publishing Group Limited. All rights reserved.

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