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Guidelines for Neuroprognostication in Adults with Guillain–Barré Syndrome

Neurocritical Care Society · 2023 · USA

Source and licence

Authors
Katharina M Busl, Herbert Fried, Susanne Muehlschlegel et al.
Title
Guidelines for Neuroprognostication in Adults with Guillain–Barré Syndrome
Journal
Neurocritical Care, 2023
DOI
10.1007/s12028-023-01707-3
Licence
CC BY 4.0
Changes
Verbatim excerpts; formatting adapted

Full text retrieved 2026-10-27 from Europe PMC · PMC10241707

Licence statement of the original publication

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This page reproduces excerpts of the original publication; the original is authoritative. It has not been reviewed by a physician, does not state a recommendation of Medelix and does not replace medical advice. The guideline may have been updated since.

Open the original publication →Full text on Europe PMC

Recommendations

Verbatim, with grade of recommendation or strength of consensus where given.

Outcome: mechanical ventilation within 14 days of disease onset

  • Question 1: When counseling patients or surrogates of critically ill patients with GBS, should the EGRIS be considered a reliable predictor of the need for mechanical ventilation within 14 days of disease onset? Description of the predictor: The EGRIS estimates the risk of respiratory failure, defined by the need for mechanical ventilation within the first week from hospital admission. The score was developed in 397 patients from a randomized trial and validated in a Dutch GBS cohort study [38]. It was further validated in three smaller cohorts [88–90] as well as in the large International GBS Outcome Study (IGOS) of 1023 patients [91]. Its prediction is based on three clinical variables determined at hospital admission (see Table 4). The sum score ranges from 0 to 7, corresponding to a predicted risk of respiratory failure from 1 to 90%. The EGRIS is also available as an online tool that can be accessed at https://gbstools.erasmusmc.nl/prognosis-tool/0/0. Recommendation: When counseling family members and/or surrogates of patients with acute GBS, we suggest that the EGRIS be considered a moderately reliable prognostic model for the probability of needing mechanical ventilation within 14 days of disease onset (weak recommendation; moderate-quality evidence). Rationale: The body of evidence was downgraded for risk of bias, with various studies demonstrating potential bias in the PROBAST domains of participant selection, study attrition, prognostic factor measurement, and calibration. In the IGOS cohort, AUC values were > 80% for all validation subgroups, but observed proportions of mechanical ventilation were lower (10%) than predicted risks (21%), requiring recalibration [91]. Of note, in a Peruvian cohort study, the EGRIS showed only moderate discrimination capacity, with an AUC of 0.63 [90]. In addition, only prediction of mechanical ventilation within 1 week from hospital admission is available. However, only 3% of patients in the development cohort were intubated after the first week of admission.
  • Question 2: When counseling patients or surrogates of critically ill patients with GBS, should the Sharshar model be considered a reliable predictor of the need for mechanical ventilation within 14 days of disease onset? Description of the predictor: The Sharshar model was developed in 722 patients enrolled in two randomized clinical trials by the French Cooperative Group on Plasma Exchange in GBS [44] and validated in 92 patients with GBS admitted consecutively to the neurology service of a tertiary care teaching hospital in India [92]. Sharshar et al. identified six clinical variables, including admission within 7 days of onset, inability to lift head and elbows, inability to stand, ineffective cough, and elevated liver enzyme levels. Each variable was given 1 point if present, and the sum score ranges from 0 to 6. Mechanical ventilation was required in > 85% of patients with at least four predictors [44]. Recommendation: When counseling family members and/or surrogates of patients with acute GBS, we suggest that the Sharshar model not be considered a reliable prognostic model for the probability of needing mechanical ventilation within 14 days of disease onset (weak recommendation; low-quality evidence). Rationale: The body of evidence was downgraded for risk of bias, with the development and validation studies demonstrating potential bias in the PROBAST domains of participant selection, study attrition, prognostic factor measurement, outcome measurement, and statistical analysis and reporting, rendering the body of evidence of low quality. The body of evidence was further limited by imprecision. The body of evidence on this prognostic model is also insufficient, with only one small validation study reporting calibration results [92].
  • Question 3: When counseling patients or surrogates of critically ill patients with GBS, should the Ning Score (MVN) be considered a reliable predictor of the need for mechanical ventilation within 14 days of disease onset? Description of the predictor: The Ning Score (MVN) was developed and validated In two Chinese cohorts of 312 and 114 patients with GBS and consists of the following variables: hospital stay > 14 days, glossopharyngeal and vagal nerve deficits, Hughes functional grading scale scores at admission, and the neutrophil/lymphocyte ratio [93]. Recommendation: When counseling family members and/or surrogates of patients with acute GBS, we suggest that the Ning Score (MVN) not be considered a reliable prognostic model for the probability of needing mechanical ventilation within 14 days of disease onset (weak recommendation; low-quality evidence). Rationale: The body of evidence was downgraded for risk of bias, with the development and validation studies demonstrating potential bias in the PROBAST domains of study attrition, prognostic factor measurement, outcome measurement, and statistical analysis and reporting, rendering the body of evidence of low quality. The body of evidence on this prognostic model is insufficient, with only one validation study reporting calibration results [93].

Outcome: Independent Ambulation Assessed at ≥ 3 months

  • Question 1: When counseling patients or surrogates of critically ill patients with GBS, should the EGOS within 2 weeks of admission be considered a reliable predictor of the need for independent ambulation at 3 months or later? Description of the predictor: The EGOS was developed and validated in 374 patients from two randomized controlled trials as well as one pilot study and validated in a set of 379 patients from another randomized trial [63] as well as one smaller single-center cohort. The score predicts the risk of being unable to walk independently at 6 months of GBS onset based on the age, preceding diarrhea, and GBS disability score at 2 weeks after entry, with scores ranging from 1 to 7 (see Table 5). Recommendation: When counseling family members and/or surrogates of patients with acute GBS, we suggest that the EGOS within 2 weeks of admission be considered a moderately reliable predictor for the probability of independent ambulation by 3 months or later (weak recommendation; moderate-quality evidence). Rationale: The body of evidence was downgraded for risk of bias, with various studies demonstrating potential bias in the PROBAST domains of participant selection, study attrition, prognostic factor measurement, and calibration. In addition, only outcome prediction at 6 months is available.
  • Question 2: When counseling patients or surrogates of critically ill patients with GBS, should the mEGOS assessed on admission and during the early course of hospital admission be considered a reliable predictor of independent ambulation by 3 months or later? Description of the predictor: The mEGOS was developed and validated from a Dutch GBS cohort study [94] and further validated in two smaller cohorts [88, 89] as well the large IGOS consisting of 809 patients [32]. The score predicts the risk of being unable to walk independently at 4 weeks, 3 months, and 6 months of GBS onset based on the variables age, muscle strength, and preceding diarrhea and can be assessed either at hospital admission or at day 7 of admission (see Table 6). The mEGOS is also available as an online tool that can be accessed at https://gbstools.erasmusmc.nl/prognosis-tool/0/0. Recommendation: When counseling family members and/or surrogates of patients with acute GBS, we suggest that the mEGOS at hospital admission and at 1 week be considered a moderately reliable predictor for the probability of independent ambulation by 3 months or later (weak recommendation; moderate-quality evidence). Rationale: The body of evidence was downgraded for risk of bias, with various studies demonstrating potential bias in the PROBAST domains of participant selection, study attrition, prognostic factor measurement, and calibration. In the IGOS cohort, AUC values were > 70% for all validation subgroups, but observed outcomes were either worse (in Europe/North America) or better (Asia) than predicted. Recalibration improved model accuracy and led to a region-specific version. Furthermore, predictive accuracy is lower if the mEGOS is assessed on hospital admission compared with at day 7 of admission [32].

Outcome: Functional Outcome Assessed at ≥ 6 months

  • Question 1: When counseling patients or surrogates of critically ill patients with GBS, should the EGOS as assessed during the acute course of hospitalization be considered a reliable predictor of functional outcome at 6 months or later? Recommendation: When counseling family members and/or surrogates of patients with acute GBS, we suggest that the EGOS not be considered a reliable predictor of functional outcome (weak recommendation; low-quality evidence). Rationale: The EGOS was not developed for prediction of functional outcome, and only two smaller studies have assessed the predictive value of the EGOS for functional outcome with low accuracy [88, 89].
  • Question 2: When counseling patients or surrogates of critically ill patients with GBS, should the mEGOS as assessed during the acute course of hospitalization be considered a reliable predictor of functional outcome assessed at 6 months or later? Recommendation: When counseling family members and/or surrogates of patients with acute GBS, we suggest that the mEGOS not be considered a reliable predictor of functional outcome (weak recommendation; low-quality evidence). Rationale: The mEGOS was not developed for prediction of functional outcome, and only one small study has assessed the predictive value of the mEGOS for functional outcome with low accuracy [89].
  • Question 3: When counseling patients or surrogates of critically ill patients with GBS, should the EGRIS as assessed during the acute course of hospitalization be considered a reliable predictor of functional outcome assessed at 6 months or later? Recommendation: When counseling family members and/or surrogates of patients with acute GBS, we suggest that the EGRIS not be considered a reliable predictor of functional outcome (weak recommendation; low-quality evidence). Rationale: The EGRIS was not developed for prediction of functional outcome, and only two smaller studies have assessed the predictive value of the EGRIS for functional outcome with low accuracy [88, 89].

Abstract

Background: Guillain–Barré syndrome (GBS) often carries a favorable prognosis. Of adult patients with GBS, 10–30% require mechanical ventilation during the acute phase of the disease. After the acute phase, the focus shifts to restoration of motor strength, ambulation, and neurological function, with variable speed and degree of recovery. The objective of these guidelines is to provide recommendations on the reliability of select clinical predictors that serve as the basis of neuroprognostication and provide guidance to clinicians counseling adult patients with GBS and/or their surrogates.

Methods: A narrative systematic review was completed using Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. Candidate predictors, including clinical variables and prediction models, were selected based on clinical relevance and presence of appropriate body of evidence. The Population/Intervention/Comparator/Outcome/Time frame/Setting (PICOTS) question was framed as follows: “When counseling patients or surrogates of critically ill patients with Guillain–Barré syndrome, should [predictor, with time of assessment if appropriate] be considered a reliable predictor of [outcome, with time frame of assessment]?” Additional full-text screening criteria were used to exclude small and lower quality studies. Following construction of an evidence profile and summary of findings, recommendations were based on four GRADE criteria: quality of evidence, balance of desirable and undesirable consequences, values and preferences, and resource use. In addition, good practice recommendations addressed essential principles of neuroprognostication that could not be framed in PICOTS format.

Results: Eight candidate clinical variables and six prediction models were selected. A total of 45 articles met our eligibility criteria to guide recommendations. We recommend bulbar weakness (the degree of motor weakness at disease nadir) and the Erasmus GBS Respiratory Insufficiency Score as moderately reliable for prediction of the need for mechanical ventilation. The Erasmus GBS Outcome Score (EGOS) and modified EGOS were identified as moderately reliable predictors of independent ambulation at 3 months and beyond. Good practice recommendations include consideration of both acute and recovery phases of the disease during prognostication, discussion of the possible need for mechanical ventilation and enteral nutrition during counseling, and consideration of the complete clinical condition as opposed to a single variable during prognostication.

Conclusions: These guidelines provide recommendations on the reliability of predictors of the need for mechanical ventilation, poor functional outcome, and independent ambulation following GBS in the context of counseling patients and/or surrogates and suggest broad principles of neuroprognostication. Few predictors were considered moderately reliable based on the available body of evidence, and higher quality data are needed.

Supplementary Information: The online version contains supplementary material available at 10.1007/s12028-023-01707-3.

Contents of the original publication

The section headings of the original.

  • Introduction
  • Scope, Purpose, and Target Audience
  • How to Use These Guidelines
  • Methods
  • Selection of Guideline Questions
  • Selection of Outcomes
  • Systematic Review Methodology
  • Evidence to Recommendation Criteria
  • Good Practice Statements
  • Recommendations: clinical prediction models
  • Conclusions
  • Endorsements
  • Source of Support

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