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

Doets, Alex Y Lingsma, Hester F Walgaard, Christa Islam, Badrul Papri, Nowshin Davidson, Amy Yamagishi, Yuko Kusunoki, Susumu Dimachkie, Mazen M Waheed, Waqar Kolb, Noah Islam, Zhahirul Mohammad, Quazi Deen Harbo, Thomas Sindrup, Soren H Chavada, Govindsinh Willison, Hugh J Casasnovas, Carlos Bateman, Kathleen Miller, James A L van den Berg, Bianca Verboon, Christine Roodbol, Joyce Leonhard, Sonja E Benedetti, Luana Kuwabara, Satoshi Van den Bergh, Peter [UCL] Monges, Soledad Marfia, Girolama A Shahrizaila, Nortina Galassi, Giuliana Péréon, Yann Bürmann, Jan Kuitwaard, Krista Kleyweg, Ruud P Marchesoni, Cintia Sedano Tous, María J Querol, Luis Illa, Isabel Wang, Yuzhong Nobile-Orazio, Eduardo Rinaldi, Simon Schenone, Angelo Pardo, Julio Vermeij, Frederique H Lehmann, Helmar C Granit, Volkan Cavaletti, Guido Gutiérrez-Gutiérrez, Gerardo Barroso, Fabio A Visser, Leo H Katzberg, Hans D Dardiotis, Efthimios Attarian, Shahram van der Kooi, Anneke J Eftimov, Filip Wirtz, Paul W Samijn, Johnny P A Gilhuis, H Jacobus Hadden, Robert D M Holt, James K L Sheikh, Kazim A Karafiath, Summer Vytopil, Michal Antonini, Giovanni Feasby, Thomas E Faber, Catharina G Gijsbers, Cees J Busby, Mark Roberts, Rhys C Silvestri, Nicholas J Fazio, Raffaella van Dijk, Gert W Garssen, Marcel P J Straathof, Chiara S M Gorson, Kenneth C Jacobs, Bart C IGOS Consortium et al.[show all]

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. TRIAL REGISTRATION INFORMATION: NCT01582763.