Regional variation of Guillain-Barré syndrome

Alex Y. Doets(Erasmus University Rotterdam), Christine Verboon(Erasmus University Rotterdam), Bianca van den Berg(Erasmus University Rotterdam), Thomas Harbo(Aarhus University Hospital), David R. Cornblath(Johns Hopkins University), Hugh J. Willison(University of Glasgow), Zhahirul Islam(International Centre for Diarrhoeal Disease Research), Shahram Attarian(Hôpital de la Timone), Fábio Barroso(Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia), Kathleen Bateman(University of Cape Town), Luana Benedetti, Peter Van den Bergh(Cliniques Universitaires Saint-Luc), Carlos Casasnovas(Bellvitge University Hospital), Guido Cavaletti(University of Milano-Bicocca), Govindsinh Chavada(University of Glasgow), Kristl G. Claeys(KU Leuven), Efthimios Dardiotis(University Hospital of Larissa), Amy Davidson(University of Glasgow), Pieter A. van Doorn(Erasmus University Rotterdam), T E Feasby(University of Calgary), Giuliana Galassi(University of Modena and Reggio Emilia), Kenneth C. Gorson(Boston University), Hans-Peter Hartung(Heinrich Heine University Düsseldorf), Sung‐Tsang Hsieh(National Taiwan University Hospital), Richard AC Hughes(National Hospital for Neurology and Neurosurgery), Isabel Illa(Hospital de Sant Pau), Badrul Islam(International Centre for Diarrhoeal Disease Research), Susumu Kusunoki(Kindai University), Satoshi Kuwabara(Chiba University), Helmar C. Lehmann(University Hospital Cologne), James Miller(Royal Victoria Infirmary), Quazi Deen Mohammad(National Institute of Neurosciences & Hospital), Soledad Monges(Garrahan Hospital), Eduardo Nobile‐Orazio(University of Milan), Julio Pardo(Instituto de Investigación Sanitaria de Santiago), Yann Péréon, Simon Rinaldi(University of Oxford), Luís Querol(Hospital de Sant Pau), Stephen W Reddel(Concord Repatriation General Hospital), Ricardo Reisin(University of Malaya), Nortina Shahrizaila(Odense University Hospital), Søren H. Sindrup(University of Vermont), Waqar Waheed(University of Vermont), Bart C. Jacobs(Erasmus University Rotterdam), Bart C. Jacobs(National Hospital for Neurology and Neurosurgery), Richard AC Hughes(Johns Hopkins University), David R. Cornblath(Boston University), Kenneth C. Gorson(Boston University), H.‐P. Hartung(Heinrich Heine University Düsseldorf), Susumu Kusunoki(Erasmus University Rotterdam), Pieter A. van Doorn(University of Glasgow), Hugh J. Willison(University of Glasgow), M. van Woerkom(Cliniques Universitaires Saint-Luc), B van den Berg(Erasmus University Rotterdam), Christine Verboon(Erasmus University Rotterdam), Alex Y. Doets(Erasmus University Rotterdam), Joyce Roodbol(Erasmus University Rotterdam), Bart C. Jacobs(Hospital Británico de Buenos Aires), Ricardo Reisin(Concord Repatriation General Hospital), Stephen Reddel(International Centre for Diarrhoeal Disease Research), Zhahirul Islam(International Centre for Diarrhoeal Disease Research), Badrul Islam(International Centre for Diarrhoeal Disease Research), Quazi Deen Mohammad(Cliniques Universitaires Saint-Luc), P. van den Bergh(Cliniques Universitaires Saint-Luc), T E Feasby(University of Calgary), Thomas Harbo(Aarhus University Hospital), Yann Péréon(Heinrich Heine University Düsseldorf), H.‐P. Hartung(University Hospital Cologne), Helmar C. Lehmann(University Hospital of Larissa), Efthimios Dardiotis(University of Milan), Eduardo Nobile‐Orazio(Kindai University), Susumu Kusunoki(University of Malaya), Nortina Shahrizaila(University of Malaya), Bart C. Jacobs(Cliniques Universitaires Saint-Luc), B van den Berg(Erasmus University Rotterdam), Christine Verboon(Erasmus University Rotterdam), Alex Y. Doets(University of Cape Town), Kathleen Bateman(University of Cape Town), Isabel Illa(Hospital de Sant Pau), Luís Querol(Hospital de Sant Pau), Sung‐Tsang Hsieh(National Taiwan University Hospital), Hugh J. Willison(University of Glasgow), Govindsinh Chavada(University of Glasgow), Amy Davidson(Boston University), Kenneth C. Gorson(Boston University), Jean Addington, Senda Ajroud‐Driss(University of Vermont), Henning Andersen, Giovanni Antonini, Alessandra Ariatti(Hôpital de la Timone), Shahram Attarian(Hôpital de la Timone), Umesh A. Badrising(Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia), Fábio Barroso(Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia), Luana Benedetti, Alessandro Beronio, Marcelle Martim Bianco(University of Vermont), Delphine Binda, Chiara Briani, Carina Bunschoten(Erasmus University Rotterdam), Jan Bürmann(International Centre for Diarrhoeal Disease Research), I.R. Bella(Concord Repatriation General Hospital), T. E. Bertoríni, Ratna Bhavaraju‐Sanka(Boston University), Thomas H. Brannagan, Mark Busby, S. Butterworth(Bellvitge University Hospital), Carlos Casasnovas(Bellvitge University Hospital), Guido Cavaletti(University of Milano-Bicocca), Chi‐Chao Chao, Sarentha Chetty(KU Leuven), Kristl G. Claeys(Royal Victoria Infirmary), M E Conti, Jeremy Cosgrove, Marinos C. Dalakas, Miroslawa A Derejko(National Institute of Neurosciences & Hospital), Mazen M. Dimachkie(Chiba University), Kathrin Doppler, Charlotta Dornonville de la Cour, Andoni Echaniz‐Laguna(University of Calgary), Filip Eftimov(Boston University), Catharina G. Faber, Raffaella Fazio, T. Fujioka(University of Vermont), E. Fulgenzi(University of Modena and Reggio Emilia), Giuliana Galassi(University of Modena and Reggio Emilia), Tania García‐Sobrino, Martina Garnero, Marcel P.J. Garssen(Erasmus University Rotterdam), C.J. Gijsbers, James M. Gilchrist, Jonathan Goldstein(Erasmus University Rotterdam), Volkan Granit, Aude‐Marie Grapperon, Gerardo Gutiérrez‐Gutiérrez, Robert D. M. Hadden, Jakob Vormstrup Holbech, James K. L. Holt, Christian Homedes Pedret, M. Htut, I. Jericó Pascual(Chiba University), Kenichi Kaida, Summer Karafiath, Hans Katzberg(University of Malaya), Lynette Kiers, B. C. Kieseier(Chiba University), Kurt Kimpinski, R. P. Kleyweg, Norito Kokubun, Noah Kolb(Chiba University), Krista Kuitwaard(Chiba University), Satoshi Kuwabara(Chiba University), Jing Yi Kwan(University of Modena and Reggio Emilia), Shafeeq Ladha(University of Malaya), Lisbeth Lassen(Erasmus University Rotterdam), Victoria Lawson(University of Vermont), David Ledingham(University of Malaya), Luciana León Cejas(Concord Repatriation General Hospital), S.T. Lucy, Michael P. Lunn, Armelle Magot(University of Vermont), Hadi Manji, Cintia Marchesoni, Girolama Alessandra Marfia, Celedonio Márquez‐Infante(University of Vermont), Eugenia Martínez‐Hernández, Giorgia Mataluni(Erasmus University Rotterdam), Christopher McDermott, Gregg Meekins(Royal Victoria Infirmary), James Miller(Garrahan Hospital), M.S. Monges, Maria C Jimeno Montero, G Morís de la Tassa(Erasmus University Rotterdam), J. Mozzoni, Caterina Nascimbene, R.J. Nowak(Odense University Hospital), P. Orizaloa Balaguer, M. Osei-Bonsu, Elizabeth Pan(Instituto de Investigación Sanitaria de Santiago), Julio Pardo(Instituto de Investigación Sanitaria de Santiago), Mamatha Pasnoor, Yusuf A. Rajabally(University of Oxford), Simon Rinaldi(University of Oxford), Christian Ritter(University Hospital Cologne), Rhys Roberts, I. Rojas-Marcos, Stacy A. Rudnicki, M Ruiz, Gabriele Sachs, Johnny P.A. Samijn(University of Malaya), L. Santoro, Angelo Schenone(University of Malaya), Lenka Schwindling, María J. Sedano Tous, Yukari Sekiguchi(Chiba University), Kazim A. Sheikh, Nicholas J. Silvestri(Odense University Hospital), Søren H. Sindrup(Odense University Hospital), Claudia Sommer(Erasmus University Rotterdam), Beth E. Shubin Stein, Amro Stino, Alex C. Spyropoulos(Erasmus University Rotterdam), Jothsana Srinivasan(University of Vermont), Hidekazu Suzuki(University of Vermont), Hatice Tankişi(University of Vermont), D. Tigner, Paul Twydell(Odense University Hospital), Philip Van Damme(Erasmus University Rotterdam), Anneke J. van der Kooi, Gert W. van Dijk, T. van der Ree, Rinske van Koningsveld, J.D. Varrato, Frédérique H Vermeij, Leo H. Visser, M.V. Vytopil(University of Vermont), Waquas Waheed, Michael Wilken, Cory Wilkerson, Paul W. Wirtz, Yuki Yamagishi(University of Malaya), Lan Zhou, Saša Živković
Brain
August 10, 2018
10.1093/brain/awy232
Cited by 325Open Access
Full Text

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.

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