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Antiglycolipid antibodies in Guillain-Barré and Fisher syndromes: discovery, current status and future perspective
  1. Susumu Kusunoki1,
  2. Hugh J Willison2,
  3. Bart C Jacobs3
  1. 1Department of Neurology, Kindai University Faculty of Medicine, Osakasayama, Japan
  2. 2Institute of Infection, Immunity and Inflammation, University of Glasgow, Glasgow, UK
  3. 3Department of Neurology and Immunology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands
  1. Correspondence to Professor Susumu Kusunoki, Department of Neurology, Kindai University Faculty of Medicine Hospital, Osakasayama 589-8511, Japan; kusunoki-tky{at}umin.ac.jp

Abstract

Guillain-Barré syndrome (GBS) and Fisher syndrome (FS) are acute autoimmune neuropathies, often preceded by an infection. Antiglycolipid antibody titres are frequently elevated in sera from the acute-phase patients. Particularly, IgG anti-GQ1b antibodies are positive in as high as 90% of FS cases and thus useful for diagnosis. The development of animal models of antiglycolipid antibody-mediated neuropathies proved that some of these antibodies are directly involved in the pathogenetic mechanisms by binding to the regions where the respective target glycolipid is specifically localised. Discovery of the presence of the antibodies that specifically recognise a new conformational epitope formed by two different gangliosides (ganglioside complex) in the acute-phase sera of some patients with GBS suggested the carbohydrate–carbohydrate interaction between glycolipids. This finding indicated the need for further research in basic glycobiological science. Antiglycolipid antibodies, in particular antigangliosides antibodies, are mostly detected in acute motor axonal neuropathy type of GBS and in FS, and less frequently in the acute inflammatory demyelinating polyneuropathy (AIDP) type of GBS or in central nervous system (CNS) diseases. In the future, the search for the putative antibodies in AIDP and those that might be present in CNS diseases should continue. In addition, more efficient standardisation of antiglycolipid antibody detection methods and use as biomarkers in daily clinical practice in neurology is needed.

  • guillain-barre syndrome
  • neuroimmunology
  • neuropathy
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Footnotes

  • Contributors SK wrote the first draft, which was revised critically by HJW and BCJ. All authors approved the final version.

  • Funding This work was supported by Japan Society for the Promotion of Science (Grants-in-Aid for Scientific Research, 18H02745) and the Ministry of Health, Labour and Welfare of Japan (Health and Labour Sciences Research Grant on Rare and Intractable Diseases, 20FC0201).

  • Competing interests SK reports grants from Teijin, grants from Japan Blood Product Organisation, grants from Nihon Pharmaceutical, personal fees from Teijin, personal fees from Japan Blood Product Organisation, personal fees from Nihon Pharmaceutical, personal fees from CSL Behring, outside the submitted work; HJW reports other from LFB Biotechnologies, grants from European Commission (EC), grants from The Wellcome Trust, personal fees from Hansa Medical AB, grants and personal fees from Argenxy BVBA and SE, grants and personal fees from Annexon, other from GSK (USA), grants from Polyneuron Pharma AG, from Janssen NL, other from Buhlmann Labs AG, personal fees from UCB Pharma BE, outside the submitted work; BCJ reports grants from Baxalta, grants from Grifols, grants from CSL-Behring, grants from Annexon, grants from Prinses-Beatrix Spierfonds, grants from GBS-CIDP Foundation International, grants from Hansa Biopharma, grants from Horizon 2020, EU, outside the submitted work.

  • Patient consent for publication Not required.

  • Provenance and peer review Commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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