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

Alex Y Doets*, Hester F Lingsma, 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 Bateman, James Al MillerBianca 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, Guido Cavaletti, Gerardo Gutiérrez-Gutiérrez, Catharina G Faber, IGOS Consortium

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Background and Objectives

The clinical course and outcome of the Guillain-Barre 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
Number of pages15
JournalNeurology
Volume98
Issue number5
Early online date22 Dec 2021
DOIs
Publication statusPublished - 1 Feb 2022

Keywords

  • INTRAVENOUS IMMUNOGLOBULIN
  • MODELS
  • POOR-PROGNOSIS

Cite this