Introduction
Scope, Purpose, and Target Audience
How to Use These Guidelines
Category of predictor/model | GRADE criteria | Point estimates of accuracy in the body of evidence | Use during counseling of patients or surrogates? | Presence of additional specific reliable or moderately predictors required for use during counseling | Suggested language during counseling of patients or surrogates | |||||
---|---|---|---|---|---|---|---|---|---|---|
Risk of bias | Inconsistency | Imprecision | Indirectness | Quality of evidence, overall | Likelihood of outcome | Disclaimer of Uncertainty during counseling | ||||
Reliable | One downgrade permitted | Downgrade not permitted | Downgrade not permitted | Downgrade not permitted | Moderate or High | High. Prediction models require AUC > 0.8, no evidence of miscalibration in external validation studies | Yes | Preferred, but not absolutely required | “Very likely” or for clinical prediction models use predicted probability of outcome | Present, but low |
Moderately reliable individual predictors | One downgrade permitted | Downgrade not permitted | One downgrade permitted | One downgrade permitted | Any | High | Yes | Yes | “Likely” | Substantial |
Moderately reliable clinical prediction models | One downgrade permitted | Downgrade not permitted | One downgrade permitted | One downgrade permitted | Any | High. Prediction models require AUC > 0.7, some miscalibration allowed in external validation studies | Yes | No | Use predicted probability of outcome | “The predicted probability is an objective estimate, subject to considerable uncertainty” |
Not reliable | Downgrade permitted | Downgrade permitted | Downgrade permitted | Downgrade permitted | Any | Any | Noa | Not applicable | Not applicable | Not applicable |
Methods
Selection of Guideline Questions
Selection of Outcomes
0 | Healthy |
1 | Minor symptoms or signs of neuropathy but capable of manual work |
2 | Able to walk without support of a stick |
3 | Able to walk with a stick, appliance of support |
4 | Confined to bed or chair bound |
5 | Requiring assisted ventilation |
6 | Death |
Systematic Review Methodology
Outcome | Predictor | Quality of Evidence | Summary of findings (narrative of effect size) | ||||
---|---|---|---|---|---|---|---|
RoB | Inconsistency | Indirectness | Imprecision | QoE summary | |||
Mechanical ventilation | Bulbar weakness | ↓ | ↓ | ↓ | Very low | OR ranging from 1.96 to 48.08 (of note, large 95% CI); in some studies, facial-bulbar not separated/cranial nerve dysfunction and bulbar weakness used interchangeably | |
Mechanical ventilation | Degree of motor weakness (limb strength less than antigravity, MRC grade) on admission or nadir | ↓ | ↓ | Low | OR ranging from 2.5 to 87 (of note, large 95% CI) depending on threshold for MRC sum score | ||
Mechanical ventilation | Rapid disease progression | ↓ | ↓ | ↓ | Very low | OR estimates not consistently performed; ranging from 1.68 to 25; different definition of rapid progression | |
Mechanical ventilation | Neck weakness | ↓ | ↓ | ↓ | ↓ | Very low | OR 4.34 to 9.86 (large 95% CI) |
Mechanical ventilation | Autonomic dysfunction | ↓↓ | ↓ | ↓ | Very low | Variable definition of autonomic dysfunction | |
Mechanical ventilation | EGRIS | ↓ | Moderate | AUC 0.82; in another validation cohort, AUC was 0.86 (0.80–0.93). Lowest AUC reported 0.63 | |||
Mechanical ventilation | Sharshar Score | ↓ | ↓ | Low | AUC 0.81 (0.72–0.90) Only one validation study | ||
Mechanical ventilation | New normogram | ↓ | ↓ | Low | AUC 0,85, C-index 0.86 (95% CI 0.77–0.94) Only one validation study | ||
Functional outcome | Need for mechanical ventilation | ↓ | ↓ | Low | OR 2.5–16 (for poor outcome) | ||
Functional outcome | Axonal electrophysiologic subtype | ↓ | ↓ | ↓ | Very low | OR 2–14.45 | |
Functional outcome | Age | ↓ | ↓ | Low | OR 1.3–10.3 (dependent on age categories or per year) | ||
Functional Outcome | Disability grade at nadir | ↓ | ↓ | Low | OR 2.9–8.88, variable scales used to assess both intake and outcome | ||
Independent ambulation | Disability grade at nadir (MRC sum score, GBSD score) | ↓ | ↓ | Low | OR to 3–4 for MRC sum score > 40 | ||
Independent ambulation | Age | ↓ | ↓ | Low | OR 1.08–3.8 (dependent on age categories or per year) | ||
Independent ambulation | Progression of weakness (speed) | ↓ | ↓ | ↓ | Very low | OR 3.8–8.6 (for poor outcome) | |
Independent ambulation | EGOS | ↓ | Moderate | AUC 0·85, 95% CI 0·81–0·89 | |||
Independent ambulation | mEGOS | ↓ | Moderate | after 1 week AUC 0.84–0.87 and at admission the AUC is 0.73–0.77. In another validation cohort, the AUC was 0.75 (0.70–0.80) after 1 week and 0.74 (0.69–0.79) at admission |
Evidence to Recommendation Criteria
Quality of Evidence/Certainty in the Evidence and Effect Size
Balance of Desirable and Undesirable Consequences
Values and Preferences
Resource Use
Good Practice Statements
Recommendations: Clinical Variables as Predictors
Outcome: Need for Mechanical Ventilation Within 14 days of Disease Onset
Outcome: Independent Ambulation at 3 months or Later
Outcome: Functional Outcome at 6 months or Later
Recommendations: clinical prediction models
Outcome: mechanical ventilation within 14 days of disease onset
Predictor | Categories | Score |
---|---|---|
Time from onset of weakness to hospital admission, days | > 7 | 0 |
4–7 | 1 | |
≤ 3 | 2 | |
Facial and/or bulbar weakness at hospital admission | Absent | 0 |
Present | 1 | |
Medical Research Council sum score at hospital admission | 51–60 | 0 |
41–50 | 1 | |
31–40 | 2 | |
21–30 | 3 | |
≤ 20 | 4 | |
Total score | 0–7 |
Outcome: Independent Ambulation Assessed at ≥ 3 months
Predictor | Categories | Score |
---|---|---|
Age at onset, years | > 60 | 1 |
41–60 | 0.5 | |
≤ 40 | 0 | |
Diarrhea (≤ 4 weeks) | Absent | 0 |
Present | 1 | |
Guillain–Barré syndrome disability score (at 2 weeks after entry) | 0 or 1 | 1 |
2 | 2 | |
3 | 3 | |
4 | 4 | |
5 | 5 | |
Total score | 1–7 |
mEGOS at hospital admission | mEGOS at day 7 of admission | ||
---|---|---|---|
Prognostic factors | Score | Prognostic factors | Score |
Age at onset, years | Age at onset, years | ||
≤ 40 | 0 | ≤ 40 | 0 |
41–60 | 1 | 41–60 | 1 |
> 60 | 2 | > 60 | 2 |
Preceding diarrhea | Preceding diarrhea | ||
Absent | 0 | Absent | 0 |
Present | 1 | Present | 1 |
MRC sum score at hospital admission | MRC sum score at day 7 of admission | ||
51–60 | 0 | 51–60 | 0 |
41–50 | 2 | 41–50 | 3 |
31–40 | 4 | 31–40 | 6 |
≤ 30 | 6 | ≤ 30 | 9 |
Total score | 0–9 | Total score | 0–12 |