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 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; Guido Cavaletti; Gerardo Gutiérrez-Gutiérrez; Catharina G Faber; IGOS Consortium

doi:10.1212/WNL.0000000000013139

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 journal › Article › Academic › peer-review

11 Citations (Web of Science)

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 language	English
Pages (from-to)	E518-E532
Number of pages	15
Journal	Neurology
Volume	98
Issue number	5
Early online date	22 Dec 2021
DOIs	https://doi.org/10.1212/WNL.0000000000013139
Publication status	Published - 1 Feb 2022

Keywords

INTRAVENOUS IMMUNOGLOBULIN
MODELS
POOR-PROGNOSIS

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10.1212/WNL.0000000000013139Licence: CC BY-NC-ND

Cite this

Doets, A. Y., Lingsma, H. F., Walgaard, C., Islam, B., Papri, N., Davidson, A., Yamagishi, Y., Kusunoki, S., Dimachkie, M. M., Waheed, W., Kolb, N., Islam, Z., Mohammad, Q. D., Harbo, T., Sindrup, S. H., Chavada, G., Willison, H. J., Casasnovas, C., Bateman, K., ... IGOS Consortium (2022). Predicting Outcome in Guillain-Barré Syndrome: International Validation of the Modified Erasmus GBS Outcome Score. Neurology, 98(5), E518-E532. Advance online publication. https://doi.org/10.1212/WNL.0000000000013139

@article{619a15adf91b4e13ab93823ed3071163,

title = "Predicting Outcome in Guillain-Barr{\'e} Syndrome: International Validation of the Modified Erasmus GBS Outcome Score",

abstract = "Background and ObjectivesThe 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.MethodsWe 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.ResultsFor 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.DiscussionmEGOS 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 EvidenceThis study provides Class II evidence that the mEGOS accurately predicts the inability to walk unaided at 4 and 26 weeks in patients with GBS.",

keywords = "INTRAVENOUS IMMUNOGLOBULIN, MODELS, POOR-PROGNOSIS",

author = "Doets, {Alex Y} and Lingsma, {Hester F} and Christa Walgaard and Badrul Islam and Nowshin Papri and Amy Davidson and Yuko Yamagishi and Susumu Kusunoki and Dimachkie, {Mazen M} and Waqar Waheed and Noah Kolb and Zhahirul Islam and Mohammad, {Quazi Deen} and Thomas Harbo and Sindrup, {Soren H} and Govindsinh Chavada and Willison, {Hugh J} and Carlos Casasnovas and Kathleen Bateman and Miller, {James Al} and {van den Berg}, Bianca and Christine Verboon and Joyce Roodbol and Leonhard, {Sonja E} and Luana Benedetti and Satoshi Kuwabara and {Van den Bergh}, Peter and Soledad Monges and Marfia, {Girolama A} and Nortina Shahrizaila and Giuliana Galassi and Yann P{\'e}r{\'e}on and Jan B{\"u}rmann and Krista Kuitwaard and Kleyweg, {Ruud P} and Cintia Marchesoni and {Sedano Tous}, {Mar{\'i}a J} and Luis Querol and Isabel Illa and Yuzhong Wang and Eduardo Nobile-Orazio and Simon Rinaldi and Angelo Schenone and Julio Pardo and Vermeij, {Frederique H} and Lehmann, {Helmar C} and Volkan Granit and Guido Cavaletti and Gerardo Guti{\'e}rrez-Guti{\'e}rrez and Faber, {Catharina G} and {IGOS Consortium}",

note = "{\textcopyright} 2021 American Academy of Neurology.",

year = "2022",

month = feb,

day = "1",

doi = "10.1212/WNL.0000000000013139",

language = "English",

volume = "98",

pages = "E518--E532",

journal = "Neurology",

issn = "0028-3878",

publisher = "LIPPINCOTT WILLIAMS & WILKINS",

number = "5",

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Doets, AY, Lingsma, HF, Walgaard, C, Islam, B, Papri, N, Davidson, A, Yamagishi, Y, Kusunoki, S, Dimachkie, MM, Waheed, W, Kolb, N, Islam, Z, Mohammad, QD, Harbo, T, Sindrup, SH, Chavada, G, Willison, HJ, Casasnovas, C, Bateman, K, Miller, JA, van den Berg, B, Verboon, C, Roodbol, J, Leonhard, SE, Benedetti, L, Kuwabara, S, Van den Bergh, P, Monges, S, Marfia, GA, Shahrizaila, N, Galassi, G, Péréon, Y, Bürmann, J, Kuitwaard, K, Kleyweg, RP, Marchesoni, C, Sedano Tous, MJ, Querol, L, Illa, I, Wang, Y, Nobile-Orazio, E, Rinaldi, S, Schenone, A, Pardo, J, Vermeij, FH, Lehmann, HC, Granit, V, Cavaletti, G, Gutiérrez-Gutiérrez, G, Faber, CG & IGOS Consortium 2022, 'Predicting Outcome in Guillain-Barré Syndrome: International Validation of the Modified Erasmus GBS Outcome Score', Neurology, vol. 98, no. 5, pp. E518-E532. https://doi.org/10.1212/WNL.0000000000013139

TY - JOUR

T1 - Predicting Outcome in Guillain-Barré Syndrome

T2 - International Validation of the Modified Erasmus GBS Outcome Score

AU - Doets, Alex Y

AU - Lingsma, Hester F

AU - Walgaard, Christa

AU - Islam, Badrul

AU - Papri, Nowshin

AU - Davidson, Amy

AU - Yamagishi, Yuko

AU - Kusunoki, Susumu

AU - Dimachkie, Mazen M

AU - Waheed, Waqar

AU - Kolb, Noah

AU - Islam, Zhahirul

AU - Mohammad, Quazi Deen

AU - Harbo, Thomas

AU - Sindrup, Soren H

AU - Chavada, Govindsinh

AU - Willison, Hugh J

AU - Casasnovas, Carlos

AU - Bateman, Kathleen

AU - Miller, James Al

AU - van den Berg, Bianca

AU - Verboon, Christine

AU - Roodbol, Joyce

AU - Leonhard, Sonja E

AU - Benedetti, Luana

AU - Kuwabara, Satoshi

AU - Van den Bergh, Peter

AU - Monges, Soledad

AU - Marfia, Girolama A

AU - Shahrizaila, Nortina

AU - Galassi, Giuliana

AU - Péréon, Yann

AU - Bürmann, Jan

AU - Kuitwaard, Krista

AU - Kleyweg, Ruud P

AU - Marchesoni, Cintia

AU - Sedano Tous, María J

AU - Querol, Luis

AU - Illa, Isabel

AU - Wang, Yuzhong

AU - Nobile-Orazio, Eduardo

AU - Rinaldi, Simon

AU - Schenone, Angelo

AU - Pardo, Julio

AU - Vermeij, Frederique H

AU - Lehmann, Helmar C

AU - Granit, Volkan

AU - Cavaletti, Guido

AU - Gutiérrez-Gutiérrez, Gerardo

AU - Faber, Catharina G

AU - IGOS Consortium

PY - 2022/2/1

Y1 - 2022/2/1

N2 - Background and ObjectivesThe 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.MethodsWe 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.ResultsFor 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.DiscussionmEGOS 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 EvidenceThis study provides Class II evidence that the mEGOS accurately predicts the inability to walk unaided at 4 and 26 weeks in patients with GBS.

AB - Background and ObjectivesThe 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.MethodsWe 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.ResultsFor 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.DiscussionmEGOS 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 EvidenceThis study provides Class II evidence that the mEGOS accurately predicts the inability to walk unaided at 4 and 26 weeks in patients with GBS.

KW - INTRAVENOUS IMMUNOGLOBULIN

KW - MODELS

KW - POOR-PROGNOSIS

U2 - 10.1212/WNL.0000000000013139

DO - 10.1212/WNL.0000000000013139

M3 - Article

C2 - 34937789

SN - 0028-3878

VL - 98

SP - E518-E532

JO - Neurology

JF - Neurology

IS - 5

ER -