International Guillain Barré Syndrome Outcome Study (IGOS)

Rationale:

Guillain-Barré syndrome (GBS) is a post-infectious acute inflammation of peripheral nerves with a highly variable clinical course and outcome. Standard treatments are not sufficiently effective in a substantial proportion of patients with GBS. Clinical predictors and biomarkers need to be identified to predict the clinical course in individual patients and to help develop more effective treatments.

Objectives:

The International GBS Outcome Study (IGOS) aims to identify biological and clinical determinants and predictors of the pathogenesis, disease progression and recovery in GBS. This information will be used to understand the diversity in clinical presentation and response to treatment and to develop new prognostic models to predict the clinical course and outcome in individual patients. 

Study design:

IGOS is a prospective observational international multi‐center study in which all patients with GBS, including all variants and overlap syndromes, can be included that present within two weeks after the start of symptoms. The study has a follow‐up of one year, with the option to extend this period to three years. In IGOS we collect detailed information on preceding infections, clinical features, diagnosis, treatment, course and outcome. During follow-up we also collect a biobank with DNA, cerebrospinal fluid and serial serum samples.

Expected results and planning:

Currently more than 1800 patients have been included in IGOS, resulting in an extensive data/biobank for GBS research. The IGOS Consortium is organized in various Expertise Groups each focusing on a particular aspect of GBS as indicated below. An overall goal is to define the processes of disease onset, progression and recovery in individual patients and to develop prognostic models, conduct selective therapeutic trials and personalize treatment.

Objectives:.

Expertise Groups from the IGOS Consortium each focus on specific GBS research areas, including:

• Prognostic modelling: validation of existing prognostic models for GBS and development of new models to predict the clinical course and outcome
• Treatment interventions: defining treatment practice, effects and side-effects
• Pharmacokinetics of IVIg: defining serum IgG levels after IVIg in relation to outcome
• Electrophysiology: pathogenesis, diagnostic and prognostic relevance of electrophysiological classification
• Preceding events: infections/vaccinations in relation to pathogenesis, GBS subtype and prognosis
• Anti-neural antibodies: characterisation of serum antibodies related to the pathogenesis and outcome of GBS
• CSF biomarkers: proteomic studies in relation to the pathogenesis, diagnosis and outcome of GBS
• Genetic markers: genetic polymorphisms related to GBS pathogenesis and outcome
• Paediatric GBS: characterisation of clinical presentation, disease course and outcome in children with GBS
• Long-term outcome: residual disability and impact 2 and 3 years after disease onset
• Outcome measures: development of clinical outcome measures to monitor GBS

The IGOS is funded by the GBS-CIDP Foundation International, gain, Erasmus MC, Glasgow University, CSL Behring, Grifols, Annexon, Hansa Biopharma, Prinses Beatrix Spierfonds, EU Horizon 2020 Research and Innovation Programme (No. 734584, ZikaPLAN Consortium)

Internal collaborations:

• Dept. Neurology (Prof. dr. B.C. Jacobs, Prof. dr. P.A. van Doorn)
• Dept. Immunology (Prof. dr. B.C. Jacobs, Dr. H. Huizinga)
• Dept. Public Health (Dr. H. Lingsma, Prof. dr. E. Steyerberg)
• Dept. Medical Microbiol Inf Dis (Prof. dr. H. Endtz)
• Dept. Virology (Dr. A. Baltissen-van Eijk, Dr. C. Geurts-van Kessel)
• Dept. Paediatric Inf Diseases (Prof. A. van Rossum, Dr. W. Unger)

External collaborations:

Islam,Z. PhD

International Centre for Diarrhoeal Disease Research, Bangladesh (icddr,b), Dhaka, Bangladesh

Gorson, K.C. MD

St. Elizabeth’s Medical Centre, Tufts University School of Medicine, Boston, USA

Nobile-Orazio, E. MD PhD

Milan University, Milan, Italy

Willison, H.J.MD, PhD

University of Glasgow, Glasgow, UK

Harbo, T. MD PhD

Aarhus University Hospital, Aarhus, Denmark

1. IGOS protocol: Jacobs, B. C., van den Berg, B. , Verboon, C. , et al. International Guillain‐Barré Syndrome Outcome Study: protocol of a prospective observational cohort study on clinical and biological predictors of disease course and outcome in Guillain‐Barré syndrome. J Peripher Nerv Syst, 22: 68-76; 2017. doi:10.1111/jns.12209
2. Regional variation: Alex Y Doets, Christine Verboon, Bianca van den Berg, et al. Regional variation of Guillain-Barré syndrome. Brain, 41(10): Pages 2866–2877; 2018. https://doi.org/10.1093/brain/awy232
3. Guillain-Barré syndrome in Denmark: a population-based study on epidemiology, diagnosis and clinical severity. Al-Hakem, H., Sindrup, S.H., Andersen, H. et al. J Neurol (2019) 266: 440. https://doi.org/10.1007/s00415-018-9151-x
4. Second IVIg course in Guillain-Barré syndrome with poor prognosis: the non-randomised ISID study.Verboon C, van den Berg B, Cornblath DR, et al. J Neurol Neurosurg Psychiatry. 2020 Feb;91(2):113-121. doi: 10.1136/jnnp-2019-321496. Epub 2019 Oct 5.
5. Current treatment practice of Guillain-Barré syndrome: Verboon C, Doets AY, Galassi et al. Neurology. 2019 Jul 2;93(1):e59-e76. doi: 10.1212/WNL.0000000000007719. Epub 2019 Jun https://doi.org/10.1212/WNL.0000000000007719

Principal investigator

Prof. dr. Bart C. Jacobs

Postdoc researcher

Dr. Melissa Mandarakas

PhD-students

drs. Bianca van den Berg

drs. Christine Verboon

drs. Joyce Roodbol

drs. Alex Doets

drs. Sonja Leonhard

drs. Linda Luijten

Biobank and immunological research

Dr. Ruth Huizinga

Wouter van Rijs, BSc

Anne Tio-Gillen, BSc

Prof. dr. Bart C. Jacobs

Research-coordinator

Marieke van Woerkom

Corresponding address