Regional variation of Guillain-Barré syndrome


Alex Y. Doets, Erasmus MC
Christine Verboon, Erasmus MC
Bianca van den Berg, Erasmus MC
Thomas Harbo, Aarhus Universitetshospital
David R. Cornblath, Johns Hopkins University
Hugh J. Willison, University of Glasgow
Zhahirul Islam, International Centre for Diarrhoeal Disease Research Bangladesh
Shahram Attarian, Hopital La Timone
Fabio A. Barroso, Instituto de Investigaciones Neurologicas Raul Carrera
Kathleen Bateman, Groote Schuur Hospital
Luana Benedetti, Azienda Ospedaliera Sant'Andrea
Peter van den Bergh, Cliniques Universitaires Saint-Luc
Carlos Casasnovas, Hospital Universitari de Bellvitge
Guido Cavaletti, University of Milano - Bicocca
Govindsinh Chavada, University of Glasgow
Kristl G. Claeys, KU Leuven– University Hospital Leuven
Efthimios Dardiotis, University Hospital of Larissa
Amy Davidson, University of Glasgow
Pieter A. van Doorn, Erasmus MC
Tom E. Feasby, University of Calgary
Giuliana Galassi, Azienda Ospedaliero - Universitaria di Modena Policlinico
Kenneth C. Gorson, Tufts University School of Medicine
Hans Peter Hartung, Heinrich-Heine-Universität Düsseldorf
Sung Tsang Hsieh, National Taiwan University Hospital
Richard A.C. Hughes, National Hospital for Neurology and Neurosurgery
Isabel Illa, Hospital de La Santa Creu I Sant Pau
Badrul Islam, International Centre for Diarrhoeal Disease Research Bangladesh
Susumu Kusunoki, Kindai University
Satoshi Kuwabara, Chiba University
Helmar C. Lehmann, Uniklinik Köln
James A.L. Miller, Royal Victoria Infirmary

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© 2018 The Author(s). 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.

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