Original research: Second IVIg course in Guillain-Barré syndrome with poor prognosis: The non-randomised ISID study

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C. Verboon, B. Van Den Berg, D. R. Cornblath, E. Venema, K. C. Gorson, M. P. Lunn, H. Lingsma, P. Van Den Bergh, T. Harbo, K. Bateman, Y. Pereon, So. H. Sindrup, S. Kusunoki, J. Miller, Z. Islam, H. -P. Hartung, G. Chavada, B. C. Jacobs, R. A. C. Hughes, Addington Van Doorn P. A. J. M., (University of Virginia Md, USA) Charlottesville, on October 7 S. Consortia. Protected by copyright., Downloaded from 8 Verboon C 2019 at Uppsala Universitet BIBSAM http://jnnp. bmj. com/ J Neurol Neurosurg Psychiatry: first published as 10. 1136/jnnp-2019-321496 on 5 October 2019., J Neurol Neurosurg Psychiatry 2019 Et Al., 1136/jnnp-2019-321496 Neuromuscular Ajroud-Driss 0:1–9. doi:10., (Northwestern University Feinberg Md, USA) Chicago, Antonini G., (Mental Health and Sensory Organs (NESMOS) Md, University Sapienza, Hospital Sant’Andrea, Italy) Rome, Attarian S., Md, (CHU Timone PhD, France) Marseille, Barroso F. A., (Instituto de Investigaciones Neurológicas Raúl Carrea Md, Fleni, Aires Buenos, Argentina), Benedetti L., Md, (Ospedale Sant’ Andrea La Spezia PhD, Spezia La, Italy), Bertorini T. E., (The University of Tennessee Health Science Center (UTHSC) Md, USA) Memphis, Brannagan T. H., (Columbia University Md, York City New, USA), Briani C., (University of Padova Md, Italy) Padova, Bhavaraju-Sanka R., (University Hospital/University of Texas Health Science Center Md, Antonio Texas San, USA), Butterworth S., (Pinderfields Hospital Md, UK) Wakefield, Casasnovas C., Md, Ciberer PhD (Bellvitge University Hospital – IDIBELL Neurometabolic Diseases Group., Spain) Barcelona, Cavaletti G., (University Milano-Bicocca Md, Italy) Monza, Chen S., Md, (Rutgers PhD, Wood Johnson University Hospital Robert, Brunswick New, USA), Claeys K. G., Md, University Hospitals Leuven PhD (1., Belgium Leuven, KU Leuven 2., Belgium) Leuven, Cosgrove J. S., (Leeds General Infirmary Md, UK) Leeds, Davidson A., (University of Glasgow Md, UK) Glasgow, Dardiotis E., (University of Thessaly Md, of Larissa Hospital, Greece) Larissa, Dornonville de la Cour C., (National Hospital Copenhagen Md, Denmark) Copenhagen, Faber C. G., Md, (Maastricht University Medical Centre PhD, Maastricht, Netherlands) The, Feasby T. E., (University of Calgary Md, Canada) Calgary, Fujioka T., (Toho University Medical Center Md, Japan) Tokyo, Galassi G., (University Hospital of Modena Md, Italy) Modena, Gilchrist J. M., (Soulthern Illinois University School of Medicine Md, USA) Springfield, Goyal N. A., (University of California Md, USA) Irvine, Granit V., (Montefiore Medical Md, Center, York New, USA), Gutiérrez-Gutiérrez G., (Hospital Universitario Infanta Sofia Md, Sebastian San, Spain), Hadden R. D. M., Md, (King’s College Hospital PhD, UK) London, Holt J. K. L., PhD, (The Walton Centre Frcp, UK) Liverpool, Htut M., George’s Hospital MD (St., UK) London, Jericó Pascual I., Md, (Complejo Hospitalario de Navarra PhD, Spain) Pamplona, Karafiath S., (University of Utah School of Medicine Md, Lake City Salt, USA), Katzberg H. D., (University of Toronto Md, Canada) Toronto, Kiers L., (University of Melbourne Md, Melbourne Hospital Royal, Australia) Parkville, Kieseier B. C., (Heinrich Heine University Md, Germany) Düsseldorf, Kimpinski K., (University Hospital Md, London-Ontario Lhsc, Canada), Kuwabara S., Md, (Chiba University PhD, Japan) Chiba, Kwan J. Y., (University of Maryland School of Medicine Md, USA) Baltimore, Ladha S. S., (Barrow Neurology Clinics Md, Arizona Phoenix, USA), Lawson V., (Wexner Medical Center at The Ohio State University Md, USA) Columbus, Lehmann H., Md, (University Hospital of Cologne PhD, Köln Universitätsklinikum, Germany) Cologne, Manji H., Md, (Ipswich Hospital Frcp, UK) Ipswich, Marfia G. A., (Neurological Clinic
ISSN: 0022-3050

Objective 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. Methods 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. Results Of 237 eligible patients, 199 patients received a single IVIg course. Twenty patients received an α early' second IVIg course (1-2 weeks after start of the first IVIg course) and 18 patients a α 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. Conclusions 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.

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