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  • Modified Erasmus GBS Respiratory Insufficiency Score: a simplified clinical tool to predict the risk of mechanical ventilation in Guillain-Barré syndrome

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Neuro-inflammation
Original research
Modified Erasmus GBS Respiratory Insufficiency Score: a simplified clinical tool to predict the risk of mechanical ventilation in Guillain-Barré syndrome
  1. Linda W G Luijten1,2,
  2. Alex Y Doets1,
  3. Samuel Arends1,3,
  4. Mazen M Dimachkie4,
  5. Kenneth C Gorson5,
  6. Badrul Islam6,
  7. Noah A Kolb7,
  8. Susumu Kusunoki8,
  9. Nowshin Papri1,6,
  10. Waqar Waheed7,
  11. Christa Walgaard9,
  12. Yuko Yamagishi8,
  13. Hester Lingsma10,
  14. Bart C Jacobs11
  15. The IGOS Consortium
    1. 1 Neurology, Erasmus Medical Center, Rotterdam, The Netherlands
    2. 2 Neurology, Elisabeth-TweeSteden Hospital, Tilburg, The Netherlands
    3. 3 Neurology, Haga Hospital, Den Haag, The Netherlands
    4. 4 Neurology, University of Kansas Medical Center, Kansas City, Kansas, USA
    5. 5 Neurology, St. Elizabeth’s Medical Center, Boston, Massachusetts, USA
    6. 6 Laboratory of Gut-Brain Signaling, Laboratory Sciences and Services Division (LSSD), International Centre for Diarrhoeal Disease Research Bangladesh, Dhaka, Bangladesh
    7. 7 Neurology, University of Vermont Medical Center, Burlington, Vermont, USA
    8. 8 Neurology, Kindai University Faculty of Medicine Graduate School of Medical Sciences, Osakasayama, Osaka, Japan
    9. 9 Neurology, IJsselland Hospital, Capelle aan den IJssel, The Netherlands
    10. 10 Public Health, Erasmus Medical Center, Rotterdam, The Netherlands
    11. 11 Neurology and Immunology, Erasmus Medical Center, Rotterdam, The Netherlands
    1. Correspondence to Professor Bart C Jacobs, Neurology and Immunology, Ee2289, Erasmus Medical Center, Rotterdam, 3015 GD, The Netherlands; b.jacobs{at}erasmusmc.nl

    Abstract

    Background This study aimed to determine the clinical and diagnostic factors associated with mechanical ventilation (MV) in Guillain-Barré syndrome (GBS) and to simplify the existing Erasmus GBS Respiratory Insufficiency Score (EGRIS) for predicting the risk of MV.

    Methods Data from the first 1500 patients included in the prospective International GBS Outcome Study (IGOS) were used. Patients were included across five continents. Patients <6 years and patients from Bangladesh were excluded. Univariable logistic and multivariable Cox regression were used to determine which prespecified clinical and diagnostic characteristics were associated with MV and to predict the risk of MV at multiple time points during disease course.

    Results 1133 (76%) patients met the study criteria. Independent predictors of MV were a shorter time from onset of weakness until admission, the presence of bulbar palsy and weakness of neck flexion and hip flexion. The modified EGRIS (mEGRIS) was based on these factors and accurately predicts the risk of MV with an area under the curve (AUC) of 0.84 (0.80–0.88). We internally validated the model within the full IGOS cohort and within separate regional subgroups, which showed AUC values of 0.83 (0.81–0.88) and 0.85 (0.72–0.98), respectively.

    Conclusions The mEGRIS is a simple and accurate tool for predicting the risk of MV in GBS. Compared with the original model, the mEGRIS requires less information for predictions with equal accuracy, can be used to predict MV at multiple time points and is also applicable in less severely affected patients and GBS variants. Model performance was consistent across different regions.

    • guillain-barre syndrome
    • clinical neurology
    • intensive care
    • neuromuscular

    Data availability statement

    Data of included patients in IGOS will be used for future studies and may be made available on reasonable request after consulting the IGOS Steering Committee.

    http://dx.doi.org/10.1136/jnnp-2022-329937

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      Data availability statement

      Data of included patients in IGOS will be used for future studies and may be made available on reasonable request after consulting the IGOS Steering Committee.

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      Footnotes

      • LWGL and AYD contributed equally.

      • Collaborators Richard A.C. Hughes, David R. Cornblath, Hans-Peter Hartung, Pieter A. van Doorn, Hugh J. Willison, Bianca van den Berg, Christine Verboon, Joyce Roodbol, Sonja E. Leonhard, Robin C.M. Thomma, Laura C. de Koning, Melissa Mandarakas, Marieke van Woerkom, Stephen W. Reddel, Zhahirul Islam, Quazi Deen Mohammad, Peter van den Bergh, Tom E. Feasby, Yuzhong Wang, Thomas Harbo, Yann Péréon, Helmar C. Lehmann, Efthimios Dardiotis, Eduardo Nobile-Orazio, Nortina Shahrizaila, Kathleen Bateman, Luis Querol, Paolo Ripellino, Sung-Tsang Hsieh, Govindsinh Chavada, Amy Davidson, James M. Addington, Senda Ajroud-Driss, Lucas Alessandro, Henning Andersen, Giovanni Antonini, Shahram Attarian, Umesh A. Badrising, Fabio A. Barroso, Isabelita R. Bella, Luana Benedetti, Tulio E. Bertorini, Ratna Bhavaraju-Sanka, Mariangela Bianco, Thomas H. Brannagan, Chiara Briani, Jan Bürmann, Mark Busby, Stephen Butterworth, Carlos Casasnovas, Guido Cavaletti, Chi-Chao Chao, Shan Chen, Kristl G. Claeys, Maria Eugenia Conti, Jeremy S. Cosgrove, Marinos C. Dalakas, Charlotte Dornonville de la Cour, Andoni Echaniz-Laguna, Filip Eftimov, Catharina G. Faber, Raffaella Fazio, Janev Fehmi, Chris Fokke, Toshiki Fujioka, Ernesto A. Fulgenzi, Giuliana Galassi, T. García-Sobrino, Marcel P.J. Garssen, Cees J. Gijsbers, James M. Gilchrist, H. Jacobus Gilhuis, Jonathan M. Goldstein, Namita A. Goyal, Volkan Granit, Stefano G. Grisanti, Gerardo Gutiérrez-Gutiérrez, Lauri Gutmann, Robert D.M. Hadden, Jakob V. Holbech, James K.L. Holt, Min Htut, Korné Jellema, Ivonne Jericó Pascual, María C. Jimeno-Montero, Kenichi Kaida, Summer Karafiath, Hans D. Katzberg, Henk Kerkhoff, Mohammad ali M. Khoshnoodi, Lynette Kiers, Ruud P. Kleyweg, Norito Kokubun, Hans Kramers, Krista Kuitwaard, Satoshi Kuwabara, Justin Y. Kwan, Shafeeq S. Ladha, Lisbeth Landschoff Lassen, Victoria Lawson, Edward Lee Pan, Luciana León Cejas, Cintia Lorena Marchesoni, Michael P. T. Lunn, Armelle Magot, Hadi Manji, Gerola A. Marfia, Celedonio Márquez-Infante, Lorena Martin Aguilar, Eugenia Martinez-Hernandez, Giorgia Mataluni, Marcelo G. Mattiazzi, Chris J. McDermott, Gregg D. Meekins, James A.L. Miller, Maria Soledad Monges, Germán Morís de la Tassa, Julieta Mozzoni, Caterina Nascimbene, Richard J. Nowak, Michael Osei-Bonsu, Julio Pardo, Robert M. Pascuzzi, Valeria Prada, Michael T. Pulley, Simon Rinaldi, Rhys C. Roberts, Iñigo Rojas-Marcos, Stacy A. Rudnicki, George M. Sachs, Johnny P.A. Samijn, Lucio Santoro, Angelo Schenone, Lenka Schwindling, Maria J. Sedano Tous, Kazim A. Sheikh, Nicholas J. Silvestri, Soren H. Sindrup, Claudia L. Sommer, Beth Stein, Amro M. Stino, Cheng-Yin Tan, Hatice Tankisi, Paul T. Twydell, Philip Van Damme, Anneke J. van der Kooi, Taco C. van der Ree, Gert W. van Dijk, Rinske van Koningsveld, Jay D. Varrato, Valentina Vélez-Santamaría, Frederique H. Vermeij, Jan J.G.M. Verschuuren, Leo H. Visser, Michal V. Vytopil, Paul W. Wirtz, Guorong Zhang, Lan Zhou, Sasha Zivkovic.

      • Contributors LWGL and AYD contributed to the design of the study and had a major role in collection of data, analysis, interpretation of the data and drafting of the manuscript. BCJ contributed to the design of the study, interpretation of the data and drafting of the manuscript and is responsible for the overall content as guarantor. HL contributed to the design of the study, interpretation of the data and drafting of the manuscript. MMD, KCG, BI, NAK, SK, NP, WW, CW, YY and SA contributed to the design of the study and had a major role in the collection of data, interpretation of the data and drafting the manuscript. The IGOS consortium members had a major role in the collection of data and critically revised the manuscript for intellectual content.

      • Funding IGOS is financially supported by the GBS-CIDP Foundation International, Gain, Erasmus MC University Medical Center Rotterdam, Glasgow University, CSL Behring, Grifols, Annexon, Hansa Biopharma and Roche.

      • Competing interests BCJ received grants from Grifols, CSL-Behring, Annexon, Prinses Beatrix Spierfonds, Baxalta, Hansa Biopharma, Roche, Horizon 2000 and GBS-CIDP Foundation International and is on the Global Medical Advisory Board of the GBS CIDP Foundation International. BCJ received consultance fees from Roche for activities outside the current study. KCG provides consulting services for Annexon, Argenx, Janssen, Pfizer, Roche and UCB Pharma, and participates on date safety monitoring boards or advisory boards from Annexon, Argenx, Janssen, Immunopharma and Roche. MMD received grants from Alexion, Alnylam Pharmaceuticals, Amicus, Biomarin, Bristol-Myers Squibb, Catalyst, Corbus, CSL-Behring, FDA/OOPD, GlaxoSmithKline, Genentech, Grifols, Kezar, Mitsubishi Tanabe Pharma, MDA, NIH, Novartis, Octapharma, Orphazyme, Ra Pharma/UCB, Sanofi Genzyme, Sarepta Therapeutics, Shire Takeda, Spark Therapeutics, The Myositis Association, UCB Biopharma/RaPharma, Viromed/Healixmith & TMA; MMD received consult fees from Amazentis, ArgenX, Catalyst, Cello, Covance/Labcorp, CSL-Behring, EcoR1, Janssen, Kezar, Medlink, Momenta, NuFactor, Octapharma, RaPharma/UCB, Roivant Sciences Inc, Sanofi Genzyme, Shire Takeda, Scholar Rock, Spark Therapeutics, Abata/Third Rock, UCB Biopharma and UpToDate; MMD received payment or honoraria for lectures/presentations from AAN, AANEM, Medlink, SANAM2022, CATALYST, SPARK, OCTOPHARMA, CSL-Behring; MMD participated in the data safety monitoring boards of Covance/LabCorp DMC and advisory boards of Sanofi, Argenx, Takeda and Janssen. SK received grants and honoraria for speech from Teijin, Nihon Pharm and the Japan Blood Products Organization. YY received payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Teijin and CSL Behring.

      • Provenance and peer review Not commissioned; externally peer reviewed.

      • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.