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. 2023 Apr;94(4):300-308.
doi: 10.1136/jnnp-2022-329937. Epub 2022 Nov 25.

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

Linda W G Luijten #  1   2 , Alex Y Doets #  1 , Samuel Arends  1   3 , Mazen M Dimachkie  4 , Kenneth C Gorson  5 , Badrul Islam  6 , Noah A Kolb  7 , Susumu Kusunoki  8 , Nowshin Papri  1   6 , Waqar Waheed  7 , Christa Walgaard  9 , Yuko Yamagishi  8 , Hester Lingsma  10 , Bart C Jacobs  11 ; IGOS Consortium
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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

Linda W G Luijten et al. J Neurol Neurosurg Psychiatry. 2023 Apr.

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.

Keywords: clinical neurology; guillain-barre syndrome; intensive care; neuromuscular.

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Conflict of interest statement

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.

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