Early recognition of poor prognosis in Guillain-Barre syndrome

Abstract

Background: Guillain-Barré syndrome (GBS) has a highly diverse clinical course and outcome, yet patients are treated with a standard therapy. Patients with poor prognosis may benefit from additional treatment, provided they can be identified early, when nerve degeneration is potentially reversible and treatment is most effective. We developed a clinical prognostic model for early prediction of outcome in GBS, applicable for clinical practice and future therapeutic trials.

Methods: Data collected prospectively from a derivation cohort of 397 patients with GBS were used to identify risk factors of being unable to walk at 4 weeks, 3 months, and 6 months. Potential predictors of poor outcome (unable to walk unaided) were considered in univariable and multivariable logistic regression models. The clinical model was based on the multivariable logistic regression coefficients of selected predictors and externally validated in an independent cohort of 158 patients with GBS.

Results: High age, preceding diarrhea, and low Medical Research Council sumscore at hospital admission and at 1 week were independently associated with being unable to walk at 4 weeks, 3 months, and 6 months (all p 0.05-0.001). The model can be used at hospital admission and at day 7 of admission, the latter having a better predictive ability for the 3 endpoints; the area under the receiver operating characteristic curve (AUC) is 0.84-0.87 and at admission the AUC is 0.73-0.77. The model proved to be valid in the validation cohort.

Conclusions: A clinical prediction model applicable early in the course of disease accurately predicts the first 6 months outcome in GBS.

Figure 1. Predicted fraction of patients unable to walk independently according to modified Erasmus GBS Outcome Score (mEGOS)

Predicted fraction of patients unable to walk independently at 4 weeks (black lines), 3 months (red lines), and 6 months (green lines) on the basis of the mEGOS at hospital admission (A) and at day 7 of admission (B). The gray areas around the colored lines represent 90% confidence intervals.

Figure 2. Comparing 3 therapeutic study populations with respect to prognostic factors at hospital admission using modified Erasmus GBS Outcome Score (mEGOS)

Points represent the percentages of patients with a specific mEGOS in a therapeutic trial comparing plasma exchange vs IV immunoglobulin (IVIg) (green), a therapeutic trial comparing IVIg/placebo vs IVIg/methylprednisolone (red), and an observational study (black). Smoothed lines represent the distribution of the study population over the total mEGOS.