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Pharmacological treatment other than corticosteroids, intravenous immunoglobulin and plasma exchange for Guillain-Barré syndrome.

Objective:To investigate cerebrospinal fluid findings in relation to clinical and electrodiagnostic subtypes, severity, and outcome of Guillain-Barré syndrome based on 1500 patients in the International GBS Outcome Study.Methods:Albuminocytological dissociation was defined as an increased protein level (>0.45 g/L) in absence of elevated white cell count (<50 cells/µL). We excluded 124 (8%) patients due to other diagnoses, protocol violation or insufficient data. CSF was examined in 1231 patients (89%).Results:In 846 (70%) patients CSF examination showed albuminocytological dissociation, which increased with time from weakness onset: ≤4 days 57%, >4 days 84%. High CSF protein levels were associated with a demyelinating subtype, proximal or global muscle weakness and a reduced likelihood of being able to run at week 2 (OR: 0.42 (95% CI 0.25-0.70;p= 0.001) and week 4 (OR: 0.44 (95% CI 0.27-0.72;p= 0.001). Patients with the Miller Fisher syndrome, distal predominant weakness and normal or equivocal nerve conduction studies were more likely to have lower CSF protein levels. CSF cell count was <5 cells/µL in 1005 patients (83%), 5-49 cells/µL in 200 patients (16%) and ≥50 cells/µL in 13 patients (1%).Interpretation:Albuminocytological dissociation is a common finding in Guillain-Barré syndrome, but normal protein levels do not exclude this diagnosis. High CSF protein level is associated with an early severe disease course and a demyelinating subtype. Elevated CSF cell count, rarely ≥50 cells/µL, is compatible with GBS after thorough exclusion of alternative diagnoses.Classification of evidence:This study provides Class IV evidence that CSF albuminocytologic dissociation (defined by the Brighton Collaboration) is common in patients with GBS.

Background and Objectives:The clinical course and outcome of the Guillain-Barré syndrome (GBS) are diverse and vary among regions. The modified Erasmus GBS Outcome Score (mEGOS) is a clinical model that predicts the risk of walking inability in GBS patients, and was developed with data from Dutch patients. The study objective was to validate the mEGOS in the International GBS Outcome Study (IGOS) cohort and to improve its performance and region-specificity.Methods:We used prospective data from the first 1500 patients included in IGOS, aged ≥ 6 years and unable to walk independently. We evaluated if the mEGOS at entry and week 1 could predict the inability to walk unaided at 4 and 26 weeks in the full cohort and in regional subgroups, using two measures for model performance: (1) discrimination: area under the receiver operating characteristic curve (AUC), and (2) calibration: observed versus predicted probability of being unable to walk independently. To improve the model predictions we recalibrated the model containing the overall mEGOS score, without changing the individual predictive factors. Finally, we assessed the predictive ability of the individual factors.Results:For validation of mEGOS at entry 809 patients were eligible (Europe/North America n=677, Asia n=76, other=56), and 671 for validation of mEGOS at week 1 (Europe/North America n=563, Asia n=65, other=43). AUC-values were >0.7 in all regional subgroups. In the Europe/North America subgroup observed outcomes were worse than predicted, while in Asia observed outcomes were better than predicted. Recalibration improved model accuracy and enabled the development of a region-specific version for Europe/North America (mEGOS-Eu/NA). Similar to the original mEGOS, severe limb weakness and higher age were the predominant predictors of poor outcome in the IGOS cohort.Discussion:The mEGOS is a validated tool to predict the inability to walk unaided at 4 and 26 weeks in GBS patients, also in countries outside The Netherlands. We developed a region-specific version of mEGOS for patients from Europe/North America.Classification of Evidence:This study provides Class II evidence that the mEGOS accurately predicts the inability to walk unaided at 4 and 26 weeks in GBS patients.ClinicalTrials.gov Identifier:NCT01582763