Current treatment practice of Guillain-Barre syndrome

Authors: Verboon, C., Doets, A. Y., Galassi, G., Davidson, A., Waheed, W., Pereon, Y., Shahrizaila, N., Kusunoki, S., Lehmann, H. C., Harbo, T., Monges, S., Van den Bergh, P., Willison, H. J., Cornblath, D. R., and Jacobs, B. C.

Published: Neurology. December 2019

Affiliations: Departments of Neurology and Immunology, Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology, University Hospital of Modena, Italy; Department of Neurology, University of Glasgow, UK; Department of Neurology, University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology, Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine, University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology, Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology, Universitätsklinikum Köln, Germany; Department of Neurology, Aarhus University Hospital, Denmark; Department of Neurology, Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology, University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD

Abstract: OBJECTIVE: To define the current treatment practice of Guillain-Barré syndrome (GBS). METHODS: The study was based on prospective observational data from the first 1,300 patients included in the International GBS Outcome Study. We described the treatment practice of GBS in general, and for (1) severe forms (unable to walk independently), (2) no recovery after initial treatment, (3) treatment-related fluctuations, (4) mild forms (able to walk independently), and (5) variant forms including Miller Fisher syndrome, taking patient characteristics and hospital type into account. RESULTS: We excluded 88 (7%) patients because of missing data, protocol violation, or alternative diagnosis. Patients from Bangladesh (n = 189, 15%) were described separately because 83% were not treated. IV immunoglobulin (IVIg), plasma exchange (PE), or other immunotherapy was provided in 941 (92%) of the remaining 1,023 patients, including patients with severe GBS (724/743, 97%), mild GBS (126/168, 75%), Miller Fisher syndrome (53/70, 76%), and other variants (33/40, 83%). Of 235 (32%) patients who did not improve after their initial treatment, 82 (35%) received a second immune modulatory treatment. A treatment-related fluctuation was observed in 53 (5%) of 1,023 patients, of whom 36 (68%) were re-treated with IVIg or PE. CONCLUSIONS: In current practice, patients with mild and variant forms of GBS, or with treatment-related fluctuations and treatment failures, are frequently treated, even in absence of trial data to support this choice. The variability in treatment practice can be explained in part by the lack of evidence and guidelines for effective treatment in these situations.