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July 02, 2019; 93 (1) Article

Current treatment practice of Guillain-Barré syndrome

View ORCID ProfileChristine Verboon, Alex Y. Doets, Giuliana Galassi, Amy Davidson, Waqar Waheed, Yann Péréon, Nortina Shahrizaila, Susumu Kusunoki, Helmar C. Lehmann, Thomas Harbo, Soledad Monges, Peter Van den Bergh, Hugh J. Willison, David R. Cornblath, Bart C. Jacobs, on behalf of the IGOS Consortium
First published June 7, 2019, DOI: https://doi.org/10.1212/WNL.0000000000007719
Christine Verboon
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Alex Y. Doets
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Giuliana Galassi
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Amy Davidson
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Waqar Waheed
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Yann Péréon
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Nortina Shahrizaila
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Susumu Kusunoki
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Helmar C. Lehmann
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Thomas Harbo
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Soledad Monges
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Peter Van den Bergh
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Hugh J. Willison
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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David R. Cornblath
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Bart C. Jacobs
From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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From the Departments of Neurology (C.V., A.Y.D., B.C.J.) and Immunology (B.C.J.), Erasmus MC, University Medical Center Rotterdam, the Netherlands; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Neurology (A.D., H.J.W.), University of Glasgow, UK; Department of Neurology (W.W.), University of Vermont Medical Center, Burlington; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, Nantes University Hospital, France; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (S.K.), Kindai University Faculty of Medicine, Osaka, Japan; Department of Neurology (H.C.L.), Universitätsklinikum Köln, Germany; Department of Neurology (T.H.), Aarhus University Hospital, Denmark; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Department of Neurology (P.V.d.B.), University Hospital St-Luc, University of Louvain, Brussels, Belgium; and Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD.
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Current treatment practice of Guillain-Barré syndrome
Christine Verboon, Alex Y. Doets, Giuliana Galassi, Amy Davidson, Waqar Waheed, Yann Péréon, Nortina Shahrizaila, Susumu Kusunoki, Helmar C. Lehmann, Thomas Harbo, Soledad Monges, Peter Van den Bergh, Hugh J. Willison, David R. Cornblath, Bart C. Jacobs, on behalf of the IGOS Consortium
Neurology Jul 2019, 93 (1) e59-e76; DOI: 10.1212/WNL.0000000000007719

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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.

Glossary

GBS=
Guillain-Barré syndrome;
IGOS=
International GBS Outcome Study;
IQR=
interquartile range;
IVIg=
IV immunoglobulin;
MFS=
Miller Fisher syndrome;
MP=
methylprednisolone;
MRC=
Medical Research Council;
PCB=
pharyngeal-cervical-brachial weakness;
PE=
plasma exchange;
RCT=
randomized controlled trial;
SID-GBS=
Second Immunoglobulin Dose in GBS trial;
TRF=
treatment-related fluctuation

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Podcast: NPub.org/bgclmr

  • CME Course: NPub.org/cmelist

  • Received October 30, 2018.
  • Accepted in final form February 13, 2019.
  • © 2019 American Academy of Neurology
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