We searched the Cochrane Library, MEDLINE, and PubMed using the search term “Guillain-Barré syndrome”. Publications from January, 2015, to April, 2020, were primarily selected, but we also included older publications that provided some of the seminal works in Guillain-Barré syndrome. We also searched the reference lists of articles identified by this search strategy, and selected papers that were relevant to the subject matter. Review articles are cited to provide readers with more details and
SeminarGuillain-Barré syndrome
Introduction
Guillain-Barré syndrome is an immune-mediated polyradiculoneuropathy that accounts for an estimated 100 000 new cases annually worldwide.1 In most patients, the acute onset of neurological symptoms is preceded by an infective illness,2 followed by progressive limb weakness, which can last up to 4 weeks before reaching plateau. Several infections are associated with Guillain-Barré syndrome, but Campylobacter jejuni is the most common and extensively reported.3 In C jejuni-related Guillain-Barré syndrome, robust evidence suggests that molecular mimicry exists between nerve and microbial antigens, leading to the development of Guillain-Barré syndrome.4
The classic presentation of the syndrome does not typically pose a diagnostic challenge, but atypical variants are missed when not considered. To support diagnosis, polyradiculoneuropathy can be detected on nerve conduction studies, and cerebrospinal fluid analysis can show albumincytological dissociation, although both tests can be normal in the early stages.5 Patients with Guillain-Barré syndrome require close monitoring for disease progression, in particular for bulbar weakness, respiratory insufficiency, and autonomic dysfunction. Prognostic scales have been developed to predict patient outcome and to stratify treatment. To date, intravenous immunoglobulin and plasma exchange are the only recognised immunotherapeutic drugs that can accelerate recovery in Guillain-Barré syndrome.5 However, the syndrome is still a serious disease. Even when treated with standard immunotherapies, approximately 5% of people die, and up to 20% cannot walk independently at 1 year from disease onset.
The past 5 years have seen advances in our understanding of Guillain-Barré syndrome, which is the focus of this Seminar. We now have improved understanding of Zika virus-associated Guillain-Barré syndrome,6 improved insight into the global burden of the syndrome through the International Guillain-Barré Syndrome Outcome Study (IGOS),7 and new therapeutic drugs have entered early clinical development.
Section snippets
Epidemiology
Guillain-Barré syndrome has been reported in many countries and has a wide range of reported incidences (figure 1).1, 8 Population-based studies from North America and Europe suggest that incidence ranges from 0·81 to 1·91 cases per 100 000 person-years (median 1·11). There is a 20% increase in incidence for every 10-year increase in age, and unlike other autoimmune diseases, the risk of Guillain-Barré syndrome is higher in men than in women.1
Although not designed to study populations, IGOS
Overview
Up to the late 1980s, Guillain-Barré syndrome was considered to be a single disease entity with immune-mediated attack on myelin components, resulting in demyelination and secondary axonal damage. It subsequently became clear that Guillain-Barré syndrome could be broadly classified into acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and AMAN, depending on the site of target antigen.25, 56 This classification, together with the discovery of antiglycolipid antibodies, expanded the
Approach to treatment
The management of patients with Guillain-Barré syndrome can be stratified according to the different stages of the disease (figure 3). In the acute phase, typically within the first 2 weeks of disease onset, patients are at risk of developing complications and extensive nerve damage. In patients with potential respiratory and autonomic failure, admission to a high-dependency unit is advisable for close monitoring of disease progression. Immunotherapy should be initiated as soon as patients show
Controversies, uncertainties, and future directions
Despite the advances in the understanding of Guillain-Barré syndrome, many uncertainties remain. To date, IGOS has impressively recruited almost 2000 patients with Guillain-Barré syndrome, but most patients have been recruited from high-income countries. Published data on Guillain-Barré syndrome in Africa, the Middle East, and many parts of Asia are scarce. To fully comprehend the global burden of Guillain-Barré syndrome and factors associated with the disease, active global engagement with
Conclusion
Since its initial description in 1916 by Georges Guillain, Jean Alexandre Barré, and André Strohl, there continues to be substantial developments in Guillain-Barré syndrome. IGOS has provided some clarity on geographical variations with more information likely to follow. The transient surge in patients with Guillain-Barré syndrome during the 2016 Zika virus outbreak and emerging reports of Guillain-Barré syndrome in SARS-CoV-2 infection add to the growing list of antecedent infections. With the
Search strategy and selection criteria
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