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

Doets AY 1 , Lingsma HF 1 , Walgaard C 1 , Islam B 1 , Papri N 1 , Davidson A 1 Show all authors , Yamagishi Y 1 , Kusunoki S 1 , Dimachkie MM 1 , Waheed W 1 , Kolb N 1 , Islam Z 1 , Mohammad QD 1 , Harbo T 1 , Sindrup SH 1 , Chavada G 1 , Willison HJ 1 , Casasnovas C 1 , Bateman K 1 , Miller JAL 1 , van den Berg B 1 , Verboon C 1 , Roodbol J 1 , Leonhard SE 1 , Benedetti L 1 , Kuwabara S 1 , Van den Bergh P 1 , Monges S 1 , Marfia GA 1 , Shahrizaila N 1 , Galassi G 1 , Péréon Y 1 , Bürmann J 1 , Kuitwaard K 1 , Kleyweg RP 1 , Marchesoni C 1 , Sedano Tous MJ 1 , Querol L 1 , Illa I 1 , Wang Y 1 , Nobile-Orazio E 1 , Rinaldi S 1 , Schenone A 1 , Pardo J 1 , Vermeij FH 1 , Lehmann HC 1 , Granit V 1 , Cavaletti G 1 , Gutiérrez-Gutiérrez G 1 , Barroso FA 1 , Visser LH 1 , Katzberg HD 1 , Dardiotis E 1 , Attarian S 1 , van der Kooi AJ 1 , Eftimov F 1 , Wirtz PW 1 , Samijn JPA 1 , Gilhuis HJ 1 , Hadden RDM 1 , Holt JKL 1 , Sheikh KA 1 , Karafiath S 1 , Vytopil M 1 , Antonini G 1 , Feasby TE 1 , Faber CG 1 , Gijsbers CJ 1 , Busby M 1 , Roberts RC 1 , Silvestri NJ 1 , Fazio R 1 , van Dijk GW 1 , Garssen MPJ 1 , Straathof CSM 1 , Gorson KC 1 , Jacobs BC 2 , IGOS Consortium

Affiliations 

  • 1 From the Departments of Neurology (A.Y.D., C.W., B.v.d.B., C.V., J.R., S.E.L., K.K., B.C.J.), Public Health (H.F.L.), and Immunology (B.C.J.), Erasmus MC, University Medical Centre Rotterdam; Department of Neurology (C.W., J.P.A.S.), Maasstad Hospital, Rotterdam, the Netherlands; Laboratory of Gut-Brain Signaling (B.I., N.P., Z.I.), Laboratory Sciences and Services Division, Dhaka, Bangladesh; Department of Neurology (A.D., G. Chavada, H.J.W.), College of Medical, Veterinary and Life Sciences, University of Glasgow, UK; Department of Neurology (Y.Y., S. Kusunoki), Kindai University Faculty of Medicine, Osaka-Sayama City, Osaka, Japan; Department of Neurology (M.M.D.), University of Kansas Medical Center, Kansas City; Department of Neurology (W.W., N.K.), University of Vermont Medical Centre, Burlington; National Institute of Neurosciences and Hospital (Q.D.M.), Agargoan, Bangladesh; Department of Neurology (T.H.), Aarhus University Hospital; Department of Neurology (S.H.S.), Odense University Hospital and University of Southern Denmark, Odense, Denmark; Department of Neurology, Neuromuscular Unit (C.C.), Bellvitge University Hospital-IDIBELL, CIBERER, Barcelona, Spain; Department of Neurology (K.B.), Groote Schuur Hospital, University of Cape Town, South Africa; Department of Neurology (J.A.L.M.), Royal Victoria Infirmary, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle, UK; Department of Neurology (B.v.d.B., C.J.G.), Franciscus Vlietland Hospital, Schiedam, the Netherlands; Department of Neurology (L.B.), IRCCS Ospedale Policlinico San Martino, Genova, Italy; Department of Neurology (S. Kuwabara), Chiba University, Japan; Department of Neurology (P.V.d.B.), Neuromuscular Reference Centre, University Hospital Saint-Luc, University of Louvain, Brussels, Belgium; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Dysimmune Neuropathies Unit, Department of Systems Medicine (G.A.M.), Tor Vergata University Hospital, Rome, Italy; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Clinical Neurophysiology, Reference Centre for NMD (Y.P.), CHU Nantes, France; Department of Neurology (J.B.), Saarland University Medical School, Homburg-Saarland (previous affiliation); MVZ Pfalzklinikum (J.B.), Kusel, Germany (current affiliation); Department of Neurology (K.K., R.P.K.), Albert Schweitzer Hospital, Dordrecht, the Netherlands; Department of Neurology (C.M.), Hospital Británico, Buenos Aires, Argentina; Department of Neurology (M.J.S.T.), Hospital Marques de Valdecilla, Santander; Department of Neurology (L.Q., I.I.), Hospital de la Santa Creu i Santa Pau, U.A.B. CIBERER and ERN-NMD, Barcelona, Spain; Department of Neurology (Y.W.), Affiliated Hospital of Jining Medical University, Shandong Province, China; Neuromuscular and Neuroimmunology Service (E.N.-O.), IRCCS Humanitas Clinical and Research Institute, Milan University, Rozzano, Italy; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust, UK; Department of Neurosciences, Ophthalmology, Rehabilitation, Genetics and Maternal Sciences (A.S.), University of Genova; IRCCS San Martino Hospital (A.S.), Genova, Italy; Department of Neurology (J.P.), Hospital Clínico de Santiago, Santiago de Compostela (A Coruña), Spain; Department of Neurology (F.H.V.), Franciscus Vlietland Hospital (location: Franciscus Gasthuis), Rotterdam, the Netherlands; Department of Neurology (H.C.L.), University Hospital of Cologne, Germany; Department of Neurology (V.G.), Montefiore Medical Centre, Bronx, NY; Department of Neurology (G. Cavaletti), University Milano-Bicocca, Monza, Italy; Department of Neurology (G.G.-G.), Hospital Universitario Infanta Sofía, San Sebastián de los Reyes, Spain; Department of Neurology (F.A.B.), Instituto de Investigaciones Neurológicas Raúl Carrea, Buenos Aires, Argentina; Department of Neurology (L.H.V.), St. Elisabeth-TweeSteden Hospital, Tilburg, the Netherlands; Department of Neurology (H.D.K.), University Health Network, University of Toronto, Canada; Department of Neurology (E.D.), University Hospital of Larissa, Greece; Department of Neurology, Reference Centre for NMD (S.A.), CHU Timone, Marseille, France; Department of Neurology (A.J.v.d.K., F.E.), Amsterdam University Medical Centre, University of Amsterdam, Neuroscience Institute, Netherlands Neuromuscular Centre, Euro-NMD; Department of Neurology (P.W.W.), Haga Hospital, Den Haag; Department of Neurology (H.J.G.), Reinier de Graaf Hospital, Delft, the Netherlands; Department of Neurology (R.D.M.H.), King's College Hospital, London; Department of Neurology (J.K.L.H.), The Walton Centre, Liverpool, UK; Department of Neurology (K.A.S.), University of Texas Health Science Centre at Houston; Department of Neurology (S. Karafiath), University of Utah School of Medicine, Salt Lake City; Department of Neurology (M.V.), Lahey Hospital and Medical Center, Tufts University School of Medicine, Burlington, MA; Department of Neurology (G.A.), Mental Health and Sensory Organs (NESMOS), University of Rome "Sapienza," Sant'Andrea Hospital, Rome, Italy; Department of Clinical Neurosciences (T.E.F.), University of Calgary, Canada; Department of Neurology (C.G.F.), Maastricht University Medical Centre, the Netherlands; Department of Neurology (M.B.), Leeds Teaching Hospitals; Department of Neurology (R.C.R.), Addenbrooke's Hospital, Cambridge, UK; Department of Neurology (N.J.S.), University at Buffalo Jacobs School of Medicine and Biomedical Sciences, NY; Department of Neurology (R.F.), Scientific Institute San Raffaele, Milan, Italy; Department of Neurology (G.W.v.D.), Canisius Wilhelmina Hospital, Nijmegen; Department of Neurology (M.P.J.G.), Jeroen Bosch Hospital, 's-Hertogenbosch; Department of Neurology (C.S.M.S.), Leiden University Medical Centre, the Netherlands; and Department of Neurology (K.C.G.), St. Elizabeth's Medical Centre, Tufts University, School of Medicine, Boston, MA
  • 2 From the Departments of Neurology (A.Y.D., C.W., B.v.d.B., C.V., J.R., S.E.L., K.K., B.C.J.), Public Health (H.F.L.), and Immunology (B.C.J.), Erasmus MC, University Medical Centre Rotterdam; Department of Neurology (C.W., J.P.A.S.), Maasstad Hospital, Rotterdam, the Netherlands; Laboratory of Gut-Brain Signaling (B.I., N.P., Z.I.), Laboratory Sciences and Services Division, Dhaka, Bangladesh; Department of Neurology (A.D., G. Chavada, H.J.W.), College of Medical, Veterinary and Life Sciences, University of Glasgow, UK; Department of Neurology (Y.Y., S. Kusunoki), Kindai University Faculty of Medicine, Osaka-Sayama City, Osaka, Japan; Department of Neurology (M.M.D.), University of Kansas Medical Center, Kansas City; Department of Neurology (W.W., N.K.), University of Vermont Medical Centre, Burlington; National Institute of Neurosciences and Hospital (Q.D.M.), Agargoan, Bangladesh; Department of Neurology (T.H.), Aarhus University Hospital; Department of Neurology (S.H.S.), Odense University Hospital and University of Southern Denmark, Odense, Denmark; Department of Neurology, Neuromuscular Unit (C.C.), Bellvitge University Hospital-IDIBELL, CIBERER, Barcelona, Spain; Department of Neurology (K.B.), Groote Schuur Hospital, University of Cape Town, South Africa; Department of Neurology (J.A.L.M.), Royal Victoria Infirmary, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle, UK; Department of Neurology (B.v.d.B., C.J.G.), Franciscus Vlietland Hospital, Schiedam, the Netherlands; Department of Neurology (L.B.), IRCCS Ospedale Policlinico San Martino, Genova, Italy; Department of Neurology (S. Kuwabara), Chiba University, Japan; Department of Neurology (P.V.d.B.), Neuromuscular Reference Centre, University Hospital Saint-Luc, University of Louvain, Brussels, Belgium; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Dysimmune Neuropathies Unit, Department of Systems Medicine (G.A.M.), Tor Vergata University Hospital, Rome, Italy; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Clinical Neurophysiology, Reference Centre for NMD (Y.P.), CHU Nantes, France; Department of Neurology (J.B.), Saarland University Medical School, Homburg-Saarland (previous affiliation); MVZ Pfalzklinikum (J.B.), Kusel, Germany (current affiliation); Department of Neurology (K.K., R.P.K.), Albert Schweitzer Hospital, Dordrecht, the Netherlands; Department of Neurology (C.M.), Hospital Británico, Buenos Aires, Argentina; Department of Neurology (M.J.S.T.), Hospital Marques de Valdecilla, Santander; Department of Neurology (L.Q., I.I.), Hospital de la Santa Creu i Santa Pau, U.A.B. CIBERER and ERN-NMD, Barcelona, Spain; Department of Neurology (Y.W.), Affiliated Hospital of Jining Medical University, Shandong Province, China; Neuromuscular and Neuroimmunology Service (E.N.-O.), IRCCS Humanitas Clinical and Research Institute, Milan University, Rozzano, Italy; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust, UK; Department of Neurosciences, Ophthalmology, Rehabilitation, Genetics and Maternal Sciences (A.S.), University of Genova; IRCCS San Martino Hospital (A.S.), Genova, Italy; Department of Neurology (J.P.), Hospital Clínico de Santiago, Santiago de Compostela (A Coruña), Spain; Department of Neurology (F.H.V.), Franciscus Vlietland Hospital (location: Franciscus Gasthuis), Rotterdam, the Netherlands; Department of Neurology (H.C.L.), University Hospital of Cologne, Germany; Department of Neurology (V.G.), Montefiore Medical Centre, Bronx, NY; Department of Neurology (G. Cavaletti), University Milano-Bicocca, Monza, Italy; Department of Neurology (G.G.-G.), Hospital Universitario Infanta Sofía, San Sebastián de los Reyes, Spain; Department of Neurology (F.A.B.), Instituto de Investigaciones Neurológicas Raúl Carrea, Buenos Aires, Argentina; Department of Neurology (L.H.V.), St. Elisabeth-TweeSteden Hospital, Tilburg, the Netherlands; Department of Neurology (H.D.K.), University Health Network, University of Toronto, Canada; Department of Neurology (E.D.), University Hospital of Larissa, Greece; Department of Neurology, Reference Centre for NMD (S.A.), CHU Timone, Marseille, France; Department of Neurology (A.J.v.d.K., F.E.), Amsterdam University Medical Centre, University of Amsterdam, Neuroscience Institute, Netherlands Neuromuscular Centre, Euro-NMD; Department of Neurology (P.W.W.), Haga Hospital, Den Haag; Department of Neurology (H.J.G.), Reinier de Graaf Hospital, Delft, the Netherlands; Department of Neurology (R.D.M.H.), King's College Hospital, London; Department of Neurology (J.K.L.H.), The Walton Centre, Liverpool, UK; Department of Neurology (K.A.S.), University of Texas Health Science Centre at Houston; Department of Neurology (S. Karafiath), University of Utah School of Medicine, Salt Lake City; Department of Neurology (M.V.), Lahey Hospital and Medical Center, Tufts University School of Medicine, Burlington, MA; Department of Neurology (G.A.), Mental Health and Sensory Organs (NESMOS), University of Rome "Sapienza," Sant'Andrea Hospital, Rome, Italy; Department of Clinical Neurosciences (T.E.F.), University of Calgary, Canada; Department of Neurology (C.G.F.), Maastricht University Medical Centre, the Netherlands; Department of Neurology (M.B.), Leeds Teaching Hospitals; Department of Neurology (R.C.R.), Addenbrooke's Hospital, Cambridge, UK; Department of Neurology (N.J.S.), University at Buffalo Jacobs School of Medicine and Biomedical Sciences, NY; Department of Neurology (R.F.), Scientific Institute San Raffaele, Milan, Italy; Department of Neurology (G.W.v.D.), Canisius Wilhelmina Hospital, Nijmegen; Department of Neurology (M.P.J.G.), Jeroen Bosch Hospital, 's-Hertogenbosch; Department of Neurology (C.S.M.S.), Leiden University Medical Centre, the Netherlands; and Department of Neurology (K.C.G.), St. Elizabeth's Medical Centre, Tufts University, School of Medicine, Boston, MA. b.jacobs@erasmusmc.nl
Neurology, 2022 Feb 01;98(5):e518-e532.
PMID: 34937789 DOI: 10.1212/WNL.0000000000013139

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.

TRIAL REGISTRATION INFORMATION: NCT01582763.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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