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Review
Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition
  1. Luigi Zuliani1,2,
  2. Francesc Graus3,
  3. Bruno Giometto1,
  4. Christian Bien4,
  5. Angela Vincent2
  1. 1Department of Neurology, Ospedale Ca'Foncello, Treviso, Italy
  2. 2Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK
  3. 3Service of Neurology, Hospital Clinic, and Institut d'Investigacio' Biomedica August Pi i Sunyer (IDIBAPS), Barcelona, Spain
  4. 4Epilepsy Centre Bethel, Krankenhaus Mara, Bielefeld, Germany
  1. Correspondence to Professor A Vincent, Nuffield Department of Clinical Neurosciences, University of Oxford, John Radcliffe Hospital, Oxford OX3 9DU, UK; angela.vincent{at}clneuro.ox.ac.uk

Abstract

The concept of antibody mediated CNS disorders is relatively recent. The classical CNS paraneoplastic neurological syndromes are thought to be T cell mediated, and the onconeural antibodies merely biomarkers for the presence of the tumour. Thus it was thought that antibodies rarely, if ever, cause CNS disease. Over the past 10 years, identification of autoimmune forms of encephalitis with antibodies against neuronal surface antigens, particularly the voltage gated potassium channel complex proteins or the glutamate N-methyl-D-aspartate receptor, have shown that CNS disorders, often without associated tumours, can be antibody mediated and benefit from immunomodulatory therapies. The clinical spectrum of these diseases is not yet fully explored, there may be others yet to be discovered and some types of more common disorders (eg, epilepsy or psychosis) may prove to have an autoimmune basis. Here, the known conditions associated with neuronal surface antibodies are briefly reviewed, some general aspects of these syndromes are considered and guidelines that could help in the recognition of further disorders are suggested.

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Footnotes

  • Funding This work was supported by personal fellowships to LZ from the European Neurological Society (ENS) and the European Federation of Neurological Societies (EFNS), and general support from the National Institutes of Health Research (NIHR) Oxford Biomedical Research Centre to AV.

  • Competing interests AV and Oxford University hold patents for MuSK-Abs and for VGKC-complex Abs, and receive royalties and payments for antibody assays.

  • Provenance and peer review Commissioned; externally peer reviewed.

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