TY - JOUR
T1 - Regional variation of Guillain-Barré syndrome
AU - the IGOS Consortium
AU - Doets, Alex Y.
AU - Verboon, Christine
AU - van den Berg, Bianca
AU - Harbo, Thomas
AU - Cornblath, David R.
AU - Willison, Hugh J.
AU - Islam, Zhahirul
AU - Attarian, Shahram
AU - Barroso, Fabio A.
AU - Bateman, Kathleen
AU - Benedetti, Luana
AU - van den Bergh, Peter
AU - Casasnovas, Carlos
AU - Cavaletti, Guido
AU - Chavada, Govindsinh
AU - Claeys, Kristl G.
AU - Dardiotis, Efthimios
AU - Davidson, Amy
AU - van Doorn, Pieter A.
AU - Feasby, Tom E.
AU - Galassi, Giuliana
AU - Gorson, Kenneth C.
AU - Hartung, Hans Peter
AU - Hsieh, Sung Tsang
AU - Hughes, Richard A.C.
AU - Illa, Isabel
AU - Islam, Badrul
AU - Kusunoki, Susumu
AU - Kuwabara, Satoshi
AU - Lehmann, Helmar C.
AU - Miller, James A.L.
AU - Mohammad, Quazi Deen
AU - Monges, Soledad
AU - Nobile Orazio, Eduardo
AU - Pardo, Julio
AU - Pereon, Yann
AU - Rinaldi, Simon
AU - Querol, Luis
AU - Reddel, Stephen W.
AU - Reisin, Ricardo C.
AU - Shahrizaila, Nortina
AU - Sindrup, Soren H.
AU - Waqar, Waheed
AU - Jacobs, Bart C.
AU - van Woerkom, M.
AU - Roodbol, J.
AU - Péréon, Y.
AU - Addington, J. M.
AU - Ajroud-Driss, S.
AU - Andersen, H.
N1 - Funding Information:
D.R.C is a consultant for Acetylon, Alcobra Pharma, Alnylam Pharmaceuticals, Annexon Biosciences, Akros Pharma, Biotest Pharmaceuticals, Boehringer Ingelheim, Cigna Health Management, CSL Behring, DP Clinical, Grifols, Hansa Medical, Karos Pharmaceuticals, Neurocrine Biosciences, Novartis, Octapharma, Pharnext, Seattle Genetics, Sun Pharmaceuticals, and Syntimmune. He is on the data and safety monitoring board for Sanofi, Pledpharma, Pfizer, Johnson & Johnson, Ionis Pharmaceuticals, GlaxoSmithKline, and Axovant Sciences. He has licensed technology for the Total Neuropathy Score for Acetylon, AstraZeneca, Calithera Biosciences, Genentech, Neurocrine Biosciences, Merrimack Pharmaceuticals, Seattle Genetics, and Shire Development Inc. He is on the Board of Directors for the Peripheral Nerve Society. G.C. received a honorarium from CSL Behring. P.A.v.D has received honoraria for consulting, lectures and serving on steering committees from Octapharma, Kedrion, CSL Behring, Grifols, and Hansa (all honoraria to departmental research fund), and is currently receiving grants from Prinses Beatrix Spierfonds, Sanquin Blood supply, Shire and Grifols. P.A.v.D is President Elect of the Peripheral Nerve Society, member of the Inflammatory Neuropathy Consortium, Medical Advisory Board for the GBS-CIDP Foundation International, editorial board for the Journal of the Neurological Sciences and Journal of Neuromuscular Diseases. He is PI of the RCT investigating the effect of methylprednisolone in GBS (MP/ IVIg RCT in GBS) and the RCT investigating the effect of a second dose IVIg in GBS (SID-GBS study). H.P.H received fees for consulting, serving on steering committees and speaking at symposia from Baxter, CSL Behring, Novartis and Octapharma related to work on chronic immune neuropathies. R.A.C.H has current consultancies with LFB and former consultancies with Novartis. S.K. received a research grant from Teijin, Japan Blood Products Organization, and Japan Pharmaceutical, and speech honoraria from Teijin, Japan Blood Products Organization, and Japan Pharmaceutical. E.N-O. received personal fees for lecturing or Advisory/Scientific/Safety Board Membership not related to this study from Baxter, Italy; CSL Behring, Switzerland; Kedrion Biopharma, Italy; LFB, France; Novartis, Switzerland; UCB, UK; Astellas Biopharma, the Netherlands. L.Q. has received research grants from the Instituto de Salud Carlos III, Spain and FEDER (grant FIS16/00627), has provided expert testimony for Grifols and CSL Behring and received research funds from Novartis Spain and Grifols (Spin Award). S.W.R. received funds over the last 5 years including, but not limited to, travel support, honoraria, trial payments, research and clinical support to the neurology department of which he is a member, from bodies and charities: NHMRC, NIH, USMDA, NSWMDA, MGANSW, MGAQLD, MAA, Beeren foundation, anonymous donors; and from pharmaceutical / biological companies: Aspreva, Baxter, Bayer Schering, Biogen Idec, CSL, Genzyme, Grifols, Merck, Novartis, Sanofi Aventis Genzyme, Servier, TEVA. He is cofounder/shareholder of Medical Safety Systems (grant and contracts with Genzyme >$25 000 AUD including a per patient payment, potential application to multiple drugs), part of the national IVIG Governance Advisory Council & Specialist Working Group Australia (Neurology) (paid), the Australian Technical Advisory Group on Immunisation Varicella Zoster working party (unpaid), and board member of the Nerve Research Foundation (unpaid). S.W.R receives public salary as a staff specialist neurologist from Sydney Local Health District (paid), private billings from patients and Medicare Australia reimbursement as a private practice neurologist (paid), and he is medical advisor (unpaid) to various patient and advocacy groups. B.C.J. is currently receiving funding for research projects from Prinses Beatrix Spierfonds, Horizon 2020, GBS-CIDP Foundation International, Grifols, CSL Behring and Annexon. He is on the Medical Advisory Board for the GBS-CIDP Foundation International, and a member of the Inflammatory Neuropathy Consortium.
Funding Information:
This study is funded by GBS-CIDP Foundation International, gain, Erasmus University Medical Centre, Glasgow University, CSL Behring, Grifols and Annexon.
Publisher Copyright:
© 2018 The Author(s).
PY - 2018/10/1
Y1 - 2018/10/1
N2 - 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.
AB - 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.
KW - axonal degeneration
KW - clinical course
KW - demyelination
KW - outcome
KW - polyradiculoneuropathy
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U2 - 10.1093/brain/awy232
DO - 10.1093/brain/awy232
M3 - Article
C2 - 30247567
AN - SCOPUS:85054687956
SN - 0006-8950
VL - 141
SP - 2866
EP - 2877
JO - Brain
JF - Brain
IS - 10
ER -