Predicting Outcome in Guillain-Barré Syndrome International Validation of the Modified Erasmus GBS Outcome Score

Alex Y. Doets, Hester F. Lingsma, the IGOS Consortium, Christa Walgaard, Badrul Islam, Nowshin Papri, Amy Davidson, Yuko Yamagishi, Susumu Kusunoki, Mazen M. Dimachkie, Waqar Waheed, Noah Kolb, Zhahirul Islam, Quazi Deen Mohammad, Thomas Harbo, Soren H. Sindrup, Govindsinh Chavada, Hugh J. Willison, Carlos Casasnovas, Kathleen BatemanJames A.L. Miller, Bianca van den Berg, Christine Verboon, Joyce Roodbol, Sonja E. Leonhard, Luana Benedetti, Satoshi Kuwabara, Peter van den Bergh, Soledad Monges, Girolama A. Marfia, Nortina Shahrizaila, Giuliana Galassi, Yann Péréon, Jan Bürmann, Krista Kuitwaard, Ruud P. Kleyweg, Cintia Marchesoni, María J. Sedano Tous, Luis Querol, Isabel Illa, Yuzhong Wang, Eduardo Nobile-Orazio, Simon Rinaldi, Angelo Schenone, Julio Pardo, Frederique H. Vermeij, Helmar C. Lehmann, Volkan Granit, Johnny P.A. Samijn, Gert W. van Dijk, Bart C. Jacobs*

*Corresponding author for this work

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Abstract

Background and Objectives The clinical course and outcome of the Guillain-Barré syndrome (GBS) are diverse and vary among regions. The modified Erasmus GBS Outcome Score (mEGOS), developed with data from Dutch patients, is a clinical model that predicts the risk of walking inability in patients with GBS. The study objective was to validate the mEGOS in the International GBS Outcome Study (IGOS) cohort and to improve its performance and region specificity. Methods We used prospective data from the first 1,500 patients included in IGOS, aged ≥6 years and unable to walk independently. We evaluated whether the mEGOS at entry and week 1 could predict the inability to walk unaided at 4 and 26 weeks in the full cohort and in regional subgroups, using 2 measures for model performance: (1) discrimination: area under the receiver operating characteristic curve (AUC) and (2) calibration: observed vs predicted probability of being unable to walk independently. To improve the model predictions, we recalibrated the model containing the overall mEGOS score, without changing the individual predictive factors. Finally, we assessed the predictive ability of the individual factors. Results For validation of mEGOS at entry, 809 patients were eligible (Europe/North America [n = 677], Asia [n = 76], other [n = 56]), and 671 for validation of mEGOS at week 1 (Europe/North America [n = 563], Asia [n = 65], other [n = 43]). AUC values were >0.7 in all regional subgroups. In the Europe/North America subgroup, observed outcomes were worse than predicted; in Asia, observed outcomes were better than predicted. Recalibration improved model accuracy and enabled the development of a region-specific version for Europe/North America (mEGOS-Eu/NA). Similar to the original mEGOS, severe limb weakness and higher age were the predominant predictors of poor outcome in the IGOS cohort. Discussion mEGOS is a validated tool to predict the inability to walk unaided at 4 and 26 weeks in patients with GBS, also in countries outside the Netherlands. We developed a region-specific version of mEGOS for patients from Europe/North America. Classification of Evidence This study provides Class II evidence that the mEGOS accurately predicts the inability to walk unaided at 4 and 26 weeks in patients with GBS.

Original languageEnglish
Pages (from-to)E518-E532
JournalNeurology
Volume98
Issue number5
DOIs
Publication statusPublished - 1 Feb 2022

Bibliographical note

Funding Information:
The IGOS is funded by the GBS-CIDP Foundation International, gain, Erasmus University Medical Centre, Glasgow University, CSL Behring, Grifols, Annexon and Hansa Biopharma.

Funding Information:
A.J. van der Kooi received financial support from CSL Behring for the Immediate Myositis study, outside the submitted work. B.C. Jacobs received grants from Grifols, CSL-Behring, Annexon, Prinses Beatrix Spierfonds, Hansa Biopharma, and GBS-CIDP Foundation International and is on the Global Medical Advisory Board of the GBS CIDP Foundation International.C.G. Faber reports grants from European Union’s Horizon 2020 research and innovation programme Marie Sklodowska-Curie grant for PAIN-Net, Molecule-to-man pain network (grant no. 721841); European Union 7th Framework Programme (grant n°602273) for the PROPANE study; the Prinses Beatrix Spierfonds; Grifols and Lamepro for a trial on IVIg in small fibre neuropathy; and from steering committees for studies in small fibre neuropathy/neuropathic pain of Biogen, Vertex and Lilly. E. Nobile-Orazio received personal fees for advisory or scientific boards from Kedrion Biopharma, Italy; Baxter/Baxalta/Shire/Takeda, USA/Japan; CSL-Behring, Italy; LFB Biomedicaments and Biotechnologies, France; Astellas, the Netherlands; UCB Biopharma srl, Belgium; Argenx BVBA, Belgium; Sanofi US Services, inc., USA, outside the submitted work, and travel grants to attend scientific meetings from Baxter, Grifols, Kedrion, and Novartis, Italy. H.C. Lehmann has received personal compensations and/or grant support in the last three years from Akcea, Alnylam, Biogen, Celgene, CSL Behring, Grifols, Novartis, and Takeda. J.K.L. Holt has received reimbursement for traveling and accommodation for foreign conference attendance and payment for Advisory Boards from CSL Behring, and has receive a research grant from Grifols. K.C. Gorson provides consulting services for Annexon, Argenx, and UCB Pharma. L. Querol is funded by the Fondo de Investigaciones Sanitarias (FIS), Instituto de Salud Carlos III, Spain and FEDER FIS19/1407 and a personal grant SLT006/17/00131 of the Pla estratègic de recerca i innovació en salut (PERIS), Departament de Salut, Generalitat de Catalunya. L. Querol received speaker honoraria from Merck, Sanofi-Genzyme, Roche, Biogen, Grifols, CSL Behring, provided expert testimony for Grifols, Johnson and Johnson, Annexon Phaarmaceuticals, Alexion, Sanofi-Genzyme, Novartis and CSL Behring and received research funds from Roche and Grifols. N. Kolb serves as a consultant for Abalone Bio and is on the Advisory Board for Alexion. R.D.M. Hadden received payments from CSL Behring, Grifols, Alnylam and Argenx. S. Kusunoki reports grants from Nihon Pharmaceutical, Teijin and Japan Blood Product Organization, and personal fees from Nihon Pharmaceutical, Teijin, Japan Blood Product Organization and CSL Behring, outside the submitted work. Z. Islam reports grants from the Fogarty International Center, National Institute of Neurological Disorders and Stroke of the National Institutes of Health, USA, under Award Number K43 TW011447 and Annexon Biosciences (South San Francisco, CA 94080, USA). A.Y. Doets, H.F. Lingsma, C. Walgaard, B. Islam, N. Papri, A. Davidson, Y. Yamagishi, M.M. Dimachkie, W. Waheed, Q.D. Mohammad, T. Harbo, S.H. Sindrup, G. Chavada, H.J. Willison, C. Casasnovas, K. Bateman, J.A.L. Miller, B. van den Berg, C. Verboon, J. Roodbol, S.E. Leonhard, L. Benedetti, S. Kuwabara, P. van den Bergh, S. Monges, G.A. Marfia, N. Shahrizaila, G. Galassi, Y. Péréon, J. Bürmann, K. Kuitwaard, R.P. Kleyweg, C. Marchesoni, M.J. Sedano Tous, I. Illa, Y. Wang, S. Rinaldi, A. Schenone, J. Pardo, F.H. Vermeij, V. Granit, G. Cavaletti, G. Gutiérrez-Gutiérrez, F.A. Barroso, L.H. Visser, H.D. Katzberg, E. Dardiotis, S. Attarian, F. Eftimov, P.W. Wirtz, J.P.A. Samijn, H.J. Gilhuis, K.A. Sheikh, S. Karafiath, M. Vytopil, G. Antonini, T.E. Feasby, C.J. Gijsbers, M. Busby, R.C. Roberts, N.J. Silvestri, R. Fazio, G.W. van Dijk, M.P.J. Garssen and C.S.M. Straathof declare no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.

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© 2022 Lippincott Williams and Wilkins. All rights reserved.

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