Regional Variation of Guillain-Barré Syndrome

Alex Y. Doets; Christine Verboon; Bianca van den Berg; Thomas Harbo; David R. Cornblath; Hugh J. Willison; Zhahirul Islam; Shahram Attarian; Fabio A. Barroso; Kathleen Bateman; Luana Benedetti; Peter van den Bergh; Carlos Casasnovas; Guido Cavaletti; Govindsinh Chavada; Kristl G. Claeys; Efthimios Dardiotis; Amy Davidson; Pieter A. van Doorn; Tom E. Feasby; Giuliana Galassi; Kenneth C. Gorson; Hans-Peter Hartung; Sung-Tsang Hsieh; Richard A.C. Hughes; Isabel Illa; Badrul Islam; Susumu Kusunoki; Satoshi Kuwabara; Helmar C. Lehmann; James A.L. Miller; Quazi Deen Mohammad; Soledad Monges; Eduardo Nobile Orazio; Julio Pardo; Yann Pereon; Simon Rinaldi; Luis Querol; Stephen W. Reddel; Ricardo C. Reisin; Nortina Shahrizaila; Soren H. Sindrup; Waheed Waqar; Bart C. Jacobs; the IGOS Consortium

Disclosures

Brain. 2018;141(10):2866-2877. 

In This Article

Abstract and Introduction

Abstract

Guillain-Barré syndrome is a heterogeneous disorder regarding the clinical presentation, electrophysiological subtype and outcome. Previous single country reports indicate that Guillain-Barré syndrome may differ among regions, but no systematic comparative studies have been conducted. Comparative studies are required to identify factors determining disease susceptibility, variation and prognosis, and to improve diagnostic criteria. The International Guillain-Barré Syndrome Outcome Study is a prospective, observational cohort study including all patients within the diagnostic spectrum, aiming to describe the heterogeneity of Guillain-Barré syndrome worldwide. The current study was based on the first 1000 inclusions with a follow-up of at least 1 year and confirmed the variation in clinical presentation, course and outcome between patients. The full clinical spectrum of Guillain-Barré syndrome was observed in patients from all countries participating in the International Guillain-Barré Syndrome Outcome Study, but the frequency of variants differed between regions. We compared three regions based on geography, income and previous reports of Guillain-Barré syndrome subtypes: 'Europe/Americas', 'Asia' (without Bangladesh), and 'Bangladesh'. We excluded 75 (8%) patients because of alternative diagnoses, protocol violations, or missing data. The predominant clinical variant was sensorimotor in Europe/Americas (n = 387/562, 69%) and Asia (n = 27/63, 43%), and pure motor in Bangladesh (n = 74/107, 69%). Miller Fisher syndrome and Miller Fisher-Guillain-Barré overlap syndrome were more common in Asia (n = 14/63, 22%) than in the other two regions (Europe/Americas: n = 64/562, 11%; Bangladesh: n = 1/107, 1%) (P < 0.001). The predominant electrophysiological subtype was demyelinating in all regions (Europe/Americas: n = 312/573, 55%; Asia: n = 29/65, 45%; Bangladesh: n = 38/94, 40%). The axonal subtype occurred more often in Bangladesh (n = 34/94, 36%) than in Europe/Americas (n = 33/573, 6%) and other Asian countries (n = 4/65, 6%) (P < 0.001). In all regions, patients with the axonal subtype were younger, had fewer sensory deficits, and showed a trend towards poorer recovery compared to patients with the demyelinating subtype. The proportion of patients able to walk unaided after 1 year varied between Asia (n = 31/34, 91%), Europe/Americas (n = 334/404, 83%) and Bangladesh (n = 67/97, 69%) (P = 0.003). A similar variation was seen for mortality, being higher in Bangladesh (n = 19/114, 17%) than in Europe/Americas (n = 23/486, 5%) and Asia (n = 1/45, 2%) (P < 0.001). This study showed that factors related to geography have a major influence on clinical phenotype, disease severity, electrophysiological subtype, and outcome of Guillain-Barré syndrome.

Introduction

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy that yearly affects ~100 000 people worldwide (Sejvar et al., 2011a). While GBS is an established clinical syndrome with defined diagnostic criteria (Asbury and Cornblath, 1990; Sejvar et al., 2011b), patients differ considerably in clinical presentation, disease course, and outcome. Patients may have clinical variants of GBS, including Miller Fisher syndrome (MFS) and pure motor, paraparetic, or pharyngeal-cervical-brachial forms (Willison et al., 2016). The electrophysiological characteristics of GBS are likewise heterogeneous and include two major subtypes with demyelinating or axonal features (Willison et al., 2016). Some patients are mildly affected and recover spontaneously, but others develop tetraplegia and respiratory or autonomic failure requiring intensive care and remain severely disabled or die despite treatment (van den Berg et al., 2014). The time to improvement is reduced with plasma exchange or intravenous immunoglobulin (IVIg) (Hughes et al., 2007, 2014; Chevret et al., 2017) but most patients in low-income countries receive supportive care only (Islam et al., 2016).

Comparison of previous studies conducted in single countries suggests that the variation of GBS may be influenced by factors related to the geographical origin of patients, such as endemic infections or unusual epidemics like the recent GBS peaks related to Zika virus (Cao-Lormeau et al., 2016; Parra et al., 2016). These studies illustrate a wide variability in prevalence of clinical variants and electrophysiological subtypes of GBS between regions, suggesting that sensorimotor and demyelinating GBS predominate in Europe and North America, whereas pure motor and axonal GBS are more frequent in Asian and South American countries (Lyu et al., 1997; Hadden et al., 1998; Bogliun et al., 2004; Hughes and Cornblath, 2005; Islam et al., 2010; Sekiguchi et al., 2012; Kuwabara and Yuki, 2013; Mitsui et al., 2015; Willison et al., 2016; Liu et al., 2018). However, these single country studies had different study designs, inclusion criteria and definitions of GBS variants (Ho et al., 1995; Hadden et al., 1998). Therefore, although valuable, these studies have intrinsic limitations and do not describe the full spectrum and geographical variation of GBS. Demonstrating the geographical variation is required to clarify the role of environmental and host factors in severity and subtypes of GBS, and point to the need for different diagnostic criteria and treatments in various parts of the world.

The International GBS Outcome Study (IGOS) is a multicentre, prospective, observational cohort study investigating factors that determine and predict the clinical course, subtype, and outcome of GBS (Jacobs et al., 2017). The aim of the current study was to use the collected data from the first 1000 patient inclusions in IGOS with a follow-up of 1 year to describe the heterogeneity of GBS and to compare the clinical presentation, electrophysiological subtypes, disease course, and outcome between patients from different geographical regions.

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