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J Neurol Neurosurg Psychiatry. 2019 Oct 5. pii: jnnp-2019-321496. doi: 10.1136/jnnp-2019-321496. [Epub ahead of print]

Second IVIg course in Guillain-Barré syndrome with poor prognosis: the non-randomised ISID study.

Collaborators (117)

Ajroud-Driss S, Antonini G, Attarian S, Barroso FA, Benedetti L, Bertorini TE, Brannagan TH, Briani C, Bhavaraju-Sanka R, Butterworth S, Casasnovas C, Cavaletti G, Chen S, Claeys KG, Cosgrove JS, Davidson A, Dardiotis E, Dornonville de la Cour C, Faber CG, Feasby TE, Fujioka T, Galassi G, Gilchrist JM, Goyal NA, Granit V, Gutiérrez-Gutiérrez G, Hadden RDM, Holt JKL, Htut M, Jericó Pascual I, Karafiath S, Katzberg HD, Kiers L, Kieseier BC, Kimpinski K, Kuwabara S, Kwan JY, Ladha SS, Lawson V, Lehmann H, Manji H, Marfia GA, Márquez Infante C, Mattiazzi MG, McDermott CJ, Monges MS, Morís de la Tassa G, Nascimbene C, Nobile Orazio E, Nowak RJ, Osei-Bonsu M, Pardo Fernandez J, Querol Gutierrez L, Reisin R, Rinaldi S, Rojas-Marcos I, Rudnicki SA, Schenone A, Sedano Tous MJ, Shahrizaila N, Sheikh K, Silvestri NJ, Sommer CL, Varrato JD, Verschuuren J, Vytopil MV, Zhou L, Bella IR, Bunschoten C, Bürmann J, Busby M, Chao CC, Conti ME, Dalakas MC, Van Damme P, Doets A, van Dijk GW, Dimachkie MM, Doppler K, Echaniz-Laguna A, Eftimov F, Fazio R, Fokke C, Fulgenzi EA, Garssen MPJ, Gijsbers CJ, Gilhuis J, Grapperon A, Hsieh ST, Illa I, Islam B, Jellema K, Kaida K, Kokubun N, Kolb N, van Koningsveld R, van der Kooi AJ, Kuitwaard K, Landschoff Lassen L, Leonhard SE, Mandarakas M, Martinez Hernandez E, Mohammad QD, Pulley M, Rajabally YA, Reddel SW, van der Ree T, Roodbol J, Sachs GM, Samijn JPA, Santoro L, Stein B, Vermeij FH, Visser LH, Willison HJ, Wirtz P, Zivkovich SA.

Author information

Department of Neurology, Erasmus MC, Rotterdam, The Netherlands.
Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
Department of Public Health, Erasmus MC, Rotterdam, The Netherlands.
Department of Neurology, St. Elizabeth's Medical Center, Boston, Massachusetts, USA.
Department of Neurology, National Hospital for Neurology and Neurosurgery, London, UK.
Department of Neurology, University Clinic St. Luc, Leuven, Belgium.
Department of Neurology, Aarhus University Hospital, Aarhus, Denmark.
Department of Neurology, University of Cape Town, Cape Town, South Africa.
Department of Clinical Neurophysiology, Reference Centre for NMD, Nantes University Hospital, Nantes, France.
Department of Neurology, Odense University Hospital, Odense, Denmark.
Department of Neurology, Kindai University Faculty of Medicine, Osaka, Japan.
Department of Neurology, Royal Victoria Infirmary, Newcastle, UK.
Department of Laboratory Sciences and Services Division, The International Centre for Diarrhoeal Disease Research, Dhaka, Bangladesh.
Department of Neurology, Heinrich Heine University, Düsseldorf, Germany.
Department of Neurology, University of Glasgow, Glasgow, UK.
Department of Immunology, Erasmus MC, Rotterdam, The Netherlands.
MRC Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery, London, UK.
Department of Neurology, Erasmus MC, Rotterdam, The Netherlands



To compare disease course in patients with Guillain-Barré syndrome (GBS) with a poor prognosis who were treated with one or with two intravenous immunoglobulin (IVIg) courses.


From the International GBS Outcome Study, we selected patients whose modified Erasmus GBS Outcome Score at week 1 predicted a poor prognosis. We compared those treated with one IVIg course to those treated with two IVIg courses. The primary endpoint, the GBS disability scale at 4 weeks, was assessed with multivariable ordinal regression.


Of 237 eligible patients, 199 patients received a single IVIg course. Twenty patients received an 'early' second IVIg course (1-2 weeks after start of the first IVIg course) and 18 patients a 'late' second IVIg course (2-4 weeks after start of IVIg). At baseline and 1 week, those receiving two IVIg courses were more disabled than those receiving one course. Compared with the one course group, the adjusted OR for a better GBS disability score at 4 weeks was 0.70 (95%CI 0.16 to 3.04) for the early group and 0.66 (95%CI 0.18 to 2.50) for the late group. The secondary endpoints were not in favour of a second IVIg course.


This observational study did not show better outcomes after a second IVIg course in GBS with poor prognosis. The study was limited by small numbers and baseline imbalances. Lack of improvement was likely an incentive to start a second IVIg course. A prospective randomised trial is needed to evaluate whether a second IVIg course improves outcome in GBS.


Guillain-Barré syndrome; poor prognosis; second IVIg course; treatment


Conflict of interest statement

Competing interests: CV, BvdB, EV, HL, KB, SHS, ZI report no disclosures. DRC is a consultant for Acetylon, Alcobra Pharma, Alnylam Pharmaceuticals, Annexon Biosciences, Akros Pharma, Biotest Pharmaceuticals, Boehringer Ingelheim, Cigna HealthManagement, CSL Behring, DP Clinical, Grifols, Hansa Medical, Karos Pharmaceuticals, Neurocrine Biosciences, Novartis, Octapharma, Pharnext, Seattle Genetics, Sun Pharmaceuticals, and Syntimmune. He is on the data and safety monitoring board for Sanofi, Pledpharma, Pfizer, Johnson Roche, Sanofi Genzyme, Teva,TG Therapeutics and UCB. GC has received honoraria from CSL Behring. BCJ has received funding for research projects from Prinses Beatrix Spierfonds, Horizon 2020,GBS-CIDP Foundation International, Grifols, CSL Behring and Annexon. He is on the Medical Advisory Board for the GBS-CIDP Foundation International, and a member of the Inflammatory Neuropathy Consortium. RH has current consultancies with LFB and former consultancies with Novartis. PvD has received honoraria for consulting, lectures and serving on steering committees from Octapharma, Kedrion, CSL Behring, Grifols, and Hansa (all honoraria to departmental research fund), and is currently receiving grants from Prinses Beatrix Spierfonds, Sanquin Blood supply, Shire and Grifols. He is President Elect of the Peripheral Nerve Society, member of the Inflammatory Neuropathy Consortium, Medical Advisory Board for the GBS-CIDP Foundation International, editorial board for the Journal of the Neurological Sciences and Journal of Neuromuscular Diseases. He is PI of the RCT investigating the effect of methylprednisolone in GBS (MP/IVIg RCT in GBS) and the RCT investigating the effect of a second dose IVIg in GBS (SID-GBS study).

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