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Regional variation of Guillain-Barré syndrome

2018; Oxford University Press; Volume: 141; Issue: 10 Linguagem: Inglês

10.1093/brain/awy232

1460-2156

Alex Y. Doets, Christine Verboon, Bianca van den Berg, Thomas Harbo, David R. Cornblath, Hugh J. Willison, Zhahirul Islam, Shahram Attarian, Fábio 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, T E Feasby, Giuliana Galassi, Kenneth C. Gorson, Hans-Peter Hartung, Sung‐Tsang Hsieh, Richard AC Hughes, Isabel Illa, Badrul Islam, Susumu Kusunoki, Satoshi Kuwabara, Helmar C. Lehmann, James Miller, Quazi Deen Mohammad, Soledad Monges, Eduardo Nobile‐Orazio, Julio Pardo, Yann Péréon, Simon Rinaldi, Luís Querol, Stephen Reddel, Ricardo Reisin, Nortina Shahrizaila, Søren H. Sindrup, Waqar Waheed, Bart C. Jacobs, Bart C. Jacobs, Richard AC Hughes, David R. Cornblath, Kenneth C. Gorson, H.‐P. Hartung, Susumu Kusunoki, Pieter A. van Doorn, Hugh J. Willison, M. van Woerkom, Benno van den Berg, Christine Verboon, Alex Y. Doets, Joyce Roodbol, Bart C. Jacobs, Ricardo Reisin, Stephen Reddel, Zhahirul Islam, Badrul Islam, Quazi Deen Mohammad, P. van den Bergh, T E Feasby, Thomas Harbo, Yann Péréon, H.‐P. Hartung, Helmar C. Lehmann, Efthimios Dardiotis, Eduardo Nobile‐Orazio, Susumu Kusunoki, Nortina Shahrizaila, Bart C. Jacobs, Benno van den Berg, Christine Verboon, Alex Y. Doets, Kathleen Bateman, Isabel Illa, Luís Querol, Sung‐Tsang Hsieh, Hugh J. Willison, Govindsinh Chavada, Amy Davidson, Kenneth C. Gorson, Jean Addington, Senda Ajroud‐Driss, Henning Andersen, Giovanni Antonini, Alessandra Ariatti, Shahram Attarian, Umesh A. Badrising, Fábio Barroso, Luana Benedetti, Alessandro Beronio, Marcelle Martim Bianco, Delphine Binda, Chiara Briani, Carina Bunschoten, Jan Bürmann, I.R. Bella, T. E. Bertoríni, Ratna Bhavaraju‐Sanka, Thomas H. Brannagan, Mark Busby, S. Butterworth, Carlos Casasnovas, Guido Cavaletti, Chi‐Chao Chao, Sarentha Chetty, Kristl G. Claeys, M E Conti, Jeremy Cosgrove, Marinos C. Dalakas, Miroslawa A Derejko, Mazen M. Dimachkie, Kathrin Doppler, Charlotta Dornonville de la Cour, Andoni Echaniz‐Laguna, Filip Eftimov, Catharina G. Faber, Raffaella Fazio, T. Fujioka, E. Fulgenzi, Giuliana Galassi, Tania García‐Sobrino, Martina Garnero, Marcel P.J. Garssen, C.J. Gijsbers, James M. Gilchrist, Jonathan Goldstein, 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, Kenichi Kaida, Summer Karafiath, Hans Katzberg, Lynette Kiers, B. C. Kieseier, Kurt Kimpinski, R. P. Kleyweg, Norito Kokubun, Noah Kolb, Krista Kuitwaard, Satoshi Kuwabara, Jing Yi Kwan, Shafeeq Ladha, Lisbeth Lassen, Victoria Lawson, David Ledingham, Luciana León Cejas, S.T. Lucy, Michael P. Lunn, Armelle Magot, Hadi Manji, Cintia Marchesoni, Girolama Alessandra Marfia, C. Márquez Infante, Eugenia Martínez‐Hernández, Giorgia Mataluni, Christopher McDermott, Gregg Meekins, James Miller, M.S. Monges, Maria C Jimeno Montero, Germán Morís, J. Mozzoni, Caterina Nascimbene, R.J. Nowak, P. Orizaloa Balaguer, M. Osei-Bonsu, Elizabeth Pan, Julio Pardo, Mamatha Pasnoor, Yusuf A. Rajabally, Simon Rinaldi, Christian Ritter, Rhys Roberts, I. Rojas-Marcos, Stacy A. Rudnicki, M Ruiz, Gabriele Sachs, Johnny P.A. Samijn, L. Santoro, Angelo Schenone, Lenka Schwindling, María J. Sedano Tous, Yukari Sekiguchi, Kazim A. Sheikh, Nicholas J. Silvestri, Søren H. Sindrup, Claudia Sommer, Beth E. Shubin Stein, Amro Stino, Alex C. Spyropoulos, Jothsana Srinivasan, Hidekazu Suzuki, Hatice Tankişi, D. Tigner, Paul Twydell, Philip Van Damme, 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, Waquas Waheed, Michael Wilken, Cory Wilkerson, Paul W. Wirtz, Yuki Yamagishi, Lan Zhou, Saša Živković,

Nerve injury and regeneration

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.