Diagnosis and management of Guillain\u2013Barr\xe9 syndrome in ten steps

Sonja E Leonhard ,Carlos A Pardo ,Thirugnanam Umapathi ,Mário Emílio Dourado ,Ricardo Reisin ,Badrul Islam ,Maria Lúcia Brito Ferreira ,James J Sejvar ,Kathleen Bateman ,Nortina Shahrizaila ,Eppie M Yiu ,Susumu Kusunoki ,David R Cornblath ,Cristiane Soares ,Melissa R Mandarakas ,Francisco De Assis Aquino Gondim ,Yu‐Zhong Wang ,Richard Hughes ,Hugh J Willison ,Pieter A Van Doorn ,Bart C Jacobs

doi:10.1038/s41582-019-0250-9

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.

Highlights

Guillain–Barré syndrome (GBS) is an inflammatory disease of the PNS and is the most common cause of acute flaccid paralysis, with an annual global incidence of approximately 1–2 per 100,000 person-years[1]
This involvement of the autonomic nervous system contributes to mortality, which is estimated at 3–10% for patients with GBS even with the best medical care available[7,8,9]
GBS is thought to be caused by an aberrant immune response to infections that results in damage to peripheral nerves, the pathogenesis is not fully understood

Summary

Introduction

Guillain–Barré syndrome (GBS) is an inflammatory disease of the PNS and is the most common cause of acute flaccid paralysis, with an annual global incidence of approximately 1–2 per 100,000 person-years[1]. Disease progression can be rapid, and most patients with GBS reach their maximum disability within 2 weeks. Cardiac arrhythmias and blood pressure instability can occur owing to involvement of the autonomic nervous system[6] This involvement of the autonomic nervous system contributes to mortality, which is estimated at 3–10% for patients with GBS even with the best medical care available[7,8,9]. Classic Guillain–Barré syndrome (GBS) is an acute-onset ascending sensorimotor neuropathy, but the disease can present atypically or as a clinical variant. Abnormal results in electrophysiological studies and a combination of an increased protein level and normal cell count in cerebrospinal fluid are classic features of GBS, but patients with GBS can have normal results in both tests, especially early in the disease course. Respiratory function should be monitored in all patients as respiratory failure can occur without symptoms of dyspnoea

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