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Regional variation of Guillain-Barré syndrome

Doets AY 1 , Verboon C 1 , van den Berg B 1 , Harbo T 2 , Cornblath DR 3 , Willison HJ 4 Show all authors , Islam Z 5 , Attarian S 6 , Barroso FA 7 , Bateman K 8 , Benedetti L 9 , van den Bergh P 10 , Casasnovas C 11 , Cavaletti G 12 , Chavada G 4 , Claeys KG 13 , Dardiotis E 14 , Davidson A 4 , van Doorn PA 1 , Feasby TE 15 , Galassi G 16 , Gorson KC 17 , Hartung HP 18 , Hsieh ST 19 , Hughes RAC 20 , Illa I 21 , Islam B 5 , Kusunoki S 22 , Kuwabara S 23 , Lehmann HC 24 , Miller JAL 25 , Mohammad QD 26 , Monges S 27 , Nobile Orazio E 28 , Pardo J 29 , Pereon Y 30 , Rinaldi S 31 , Querol L 21 , Reddel SW 32 , Reisin RC 33 , Shahrizaila N 34 , Sindrup SH 35 , Waqar W 36 , Jacobs BC 1 , IGOS Consortium

Affiliations 

  • 1 Department of Neurology, Erasmus University Medical Centre, Dr. Molewaterplein 40, 3015 GD, Rotterdam, The Netherlands
  • 2 Department of Neurology, Aarhus University Hospital, Nörrebrogade 44, 8000, Aarhus, Denmark
  • 3 Department of Neurology, Johns Hopkins University, 733 North Broadway, 21205 MD, Baltimore, USA
  • 4 Department of Neurology, University of Glasgow, University Avenue, G12 8QQ, Glasgow, UK
  • 5 Department of Laboratory Sciences and Services Division, The International Centre for Diarrhoeal Disease Research, GBP Box 128, 1000, Dhaka, Bangladesh
  • 6 Department of Neurology, CHU Timone, 264 Rue Saint Pierre, 13005, Marseille, France
  • 7 Department of Neurology, Instituto de Investigaciones Neurológicas Raúl Carrea, FLENI, Montañeses 2325, Buenos Aires, Argentina
  • 8 Department of Neurology, Groote Schuur Hospital, University of Cape Town, Main Road, Observatory 7925, Cape Town, South Africa
  • 9 Department of Neurology, Ospedale Sant' Andrea La Spezia, Via Vittorio Veneto 197, 19121 SP, La Spezia, Italy
  • 10 Department of Neurology, University Hospital St. Luc, University of Louvain, Avenue Hippocrate 10, 1200, Brussels, Belgium
  • 11 Department of Neurology, Bellvitge University Hospital, Carrer de la Feixa Llarga 8907, Barcelona, Spain
  • 12 Department of Neurology, University Milano-Bicocca, Via Cadore 48, 20900 MB, Monza, Italy
  • 13 Department of Neurology, University Hospitals Leuven, Herestraat 49, 3000, Leuven, Belgium
  • 14 Department of Neurology, University Hospital of Larissa, POB 1425, 41110, Larissa, Greece
  • 15 Department of Clinical Neurosciences, University of Calgary, 2500 University Drive NW, T2N 1N4, Calgary, Canada
  • 16 Department of Neurology, University Hospital of Modena, Via P. Giardini 1455, 41126, Modena, Italy
  • 17 Department of Neurology, Tufts University School of Medicine, 736 Cambridge Street, 2135, Boston, USA
  • 18 Department of Neurology, Medical Faculty and Center of Neurology and Neuropsychiatry, Heinrich-Heine-University Düsseldorf, Moorenstrasse 1, 40225, Düsseldorf, Germany
  • 19 Department of Neurology, National Taiwan University Hospital, 7 Chung-Shan S Road, 10002, Taipei City, Taiwan
  • 20 MRC Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery, Queen Square, WC1N 3BG, London, UK
  • 21 Department of Neurology, Hospital de la Santa Creu I Santa Pau, C/Sant Antoni M. Claret 167, 8025, Barcelona, Spain
  • 22 Department of Neurology, Kindai University, 377-2 Ohno-Higashi, Osaka-Sayama City, Osaka 589-8511, Japan
  • 23 Department of Neurology, Chiba University, 1-8-1 Inohana, Chuo-ku, 260-8670, Chiba, Japan
  • 24 Department of Neurology, University Hospital of Cologne, Kerpenerstrasse 62, 50937, Cologne, Germany
  • 25 Department of Neurology, Royal Victoria Infirmary, Newcastle upon Tyne Hospitals NHS Foundation Trust, Queen Victoria Road, NE1 4LP, Newcastle, UK
  • 26 National Institute of Neuroscience and Hospital, Sher-E-Bangla Nagar, 1207, Dhaka, Bangladesh
  • 27 Department of Neurology, Hospital de Pediatría J.P. Garrahan, Combate de los Pozos 1881, 1245, Buenos Aires, Argentina
  • 28 Department of Neurology, Milan University, Via Manzoni 56, 20089, Rozzano, MI, Milan, Italy
  • 29 Department of Neurology, Hospital Clínico de Santiago, Travesia Choupana, S/N 15706, Santiago de Compostela (A Coruña), Spain
  • 30 Department of Clinical Neurophysiology, Reference centre for NMD, CHU Nantes, Place Alexis-Ricordeau, 44093, Nantes, France
  • 31 Department of Clinical Neurosciences, University of Oxford and Oxford University Hospitals NHS Foundation Trust, Headly Way, Headington, OX3 9DU, Oxford, UK
  • 32 Department of Neurology, Concord Hospital, Hospital Road, 2139, Sydney NSW, Australia
  • 33 Department of Neurology, Hospital Británico, Perdriel 74, 1280, Buenos Aires, Argentina
  • 34 Department of Medicine, University of Malaya, Lembah Pantai, 50603, Kuala Lumpur, Malaysia
  • 35 Department of Neurology, Odense University Hospital, Sdr. Boulevard 29, 5000, Odense, Denmark
  • 36 Department of Neurology, University of Vermont, 89 South William Street 5401, Burlington, USA
Brain, 2018 10 01;141(10):2866-2877.
PMID: 30247567 DOI: 10.1093/brain/awy232

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.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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