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The autoantibody-mediated encephalitides: from clinical observations to molecular pathogenesis
The autoimmune encephalitis (AE) syndromes have been characterised by the detection of autoantibodies in serum and/or cerebrospinal fluid which target the extracellular domains of specific neuroglial antigens. The clinical syndromes have phenotypes which are often highly characteristic of their asso...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8068716/ https://www.ncbi.nlm.nih.gov/pubmed/31655889 http://dx.doi.org/10.1007/s00415-019-09590-9 |
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author | Ramanathan, Sudarshini Al-Diwani, Adam Waters, Patrick Irani, Sarosh R. |
author_facet | Ramanathan, Sudarshini Al-Diwani, Adam Waters, Patrick Irani, Sarosh R. |
author_sort | Ramanathan, Sudarshini |
collection | PubMed |
description | The autoimmune encephalitis (AE) syndromes have been characterised by the detection of autoantibodies in serum and/or cerebrospinal fluid which target the extracellular domains of specific neuroglial antigens. The clinical syndromes have phenotypes which are often highly characteristic of their associated antigen-specific autoantibody. For example, the constellation of psychiatric features and the multi-faceted movement disorder observed in patients with NMDAR antibodies are highly distinctive, as are the faciobrachial dystonic seizures observed in close association with LGI1 antibodies. These typically tight correlations may be conferred by the presence of autoantibodies which can directly access and modulate their antigens in vivo. AE remains an under-recognised clinical syndrome but one where early and accurate detection is critical as prompt initiation of immunotherapy is closely associated with improved outcomes. In this review of a rapidly emerging field, we outline molecular observations with translational value. We focus on contemporary methodologies of autoantibody detection, the evolution and distinctive nature of the clinical phenotypes, generalisable therapeutic paradigms, and finally discuss the likely mechanisms of autoimmunity in these patients which may inform future precision therapies. |
format | Online Article Text |
id | pubmed-8068716 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-80687162021-05-05 The autoantibody-mediated encephalitides: from clinical observations to molecular pathogenesis Ramanathan, Sudarshini Al-Diwani, Adam Waters, Patrick Irani, Sarosh R. J Neurol Review The autoimmune encephalitis (AE) syndromes have been characterised by the detection of autoantibodies in serum and/or cerebrospinal fluid which target the extracellular domains of specific neuroglial antigens. The clinical syndromes have phenotypes which are often highly characteristic of their associated antigen-specific autoantibody. For example, the constellation of psychiatric features and the multi-faceted movement disorder observed in patients with NMDAR antibodies are highly distinctive, as are the faciobrachial dystonic seizures observed in close association with LGI1 antibodies. These typically tight correlations may be conferred by the presence of autoantibodies which can directly access and modulate their antigens in vivo. AE remains an under-recognised clinical syndrome but one where early and accurate detection is critical as prompt initiation of immunotherapy is closely associated with improved outcomes. In this review of a rapidly emerging field, we outline molecular observations with translational value. We focus on contemporary methodologies of autoantibody detection, the evolution and distinctive nature of the clinical phenotypes, generalisable therapeutic paradigms, and finally discuss the likely mechanisms of autoimmunity in these patients which may inform future precision therapies. Springer Berlin Heidelberg 2019-10-26 2021 /pmc/articles/PMC8068716/ /pubmed/31655889 http://dx.doi.org/10.1007/s00415-019-09590-9 Text en © The Author(s) 2019 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) ), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. |
spellingShingle | Review Ramanathan, Sudarshini Al-Diwani, Adam Waters, Patrick Irani, Sarosh R. The autoantibody-mediated encephalitides: from clinical observations to molecular pathogenesis |
title | The autoantibody-mediated encephalitides: from clinical observations to molecular pathogenesis |
title_full | The autoantibody-mediated encephalitides: from clinical observations to molecular pathogenesis |
title_fullStr | The autoantibody-mediated encephalitides: from clinical observations to molecular pathogenesis |
title_full_unstemmed | The autoantibody-mediated encephalitides: from clinical observations to molecular pathogenesis |
title_short | The autoantibody-mediated encephalitides: from clinical observations to molecular pathogenesis |
title_sort | autoantibody-mediated encephalitides: from clinical observations to molecular pathogenesis |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8068716/ https://www.ncbi.nlm.nih.gov/pubmed/31655889 http://dx.doi.org/10.1007/s00415-019-09590-9 |
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