Cell-surface central nervous system autoantibodies: clinical relevance and emerging paradigms.

The recent discovery of several potentially pathogenic autoantibodies has helped identify patients with clinically distinctive central nervous system diseases that appear to benefit from immunotherapy. The associated autoantibodies are directed against the extracellular domains of cell-surface-expre...

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Main Authors: Irani, SR, Gelfand, J, Al-Diwani, A, Vincent, A
Format: Journal article
Language:English
Published: 2014
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author Irani, SR
Gelfand, J
Al-Diwani, A
Vincent, A
author_facet Irani, SR
Gelfand, J
Al-Diwani, A
Vincent, A
author_sort Irani, SR
collection OXFORD
description The recent discovery of several potentially pathogenic autoantibodies has helped identify patients with clinically distinctive central nervous system diseases that appear to benefit from immunotherapy. The associated autoantibodies are directed against the extracellular domains of cell-surface-expressed neuronal or glial proteins such as LGI1, N-methyl-D-aspartate receptor, and aquaporin-4. The original descriptions of the associated clinical syndromes were phenotypically well circumscribed. However, as availability of antibody testing has increased, the range of associated patient phenotypes and demographics has expanded. This in turn has led to the recognition of more immunotherapy-responsive syndromes in patients presenting with cognitive and behavioral problems, seizures, movement disorders, psychiatric features, and demyelinating disease. Although antibody detection remains diagnostically important, clinical recognition of these distinctive syndromes should ensure early and appropriate immunotherapy administration. We review the emerging paradigm of cell-surface-directed antibody-mediated neurological diseases, describe how the associated disease spectrums have broadened since the original descriptions, discuss some of the methodological issues regarding techniques for antibody detection and emphasize considerations surrounding immunotherapy administration. As these disorders continue to reach mainstream neurology and even psychiatry, more cell-surface-directed antibodies will be discovered, and their possible relevance to other more common disease presentations should become more clearly defined.
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spelling oxford-uuid:550792b9-46b7-411f-afe6-5b6a458816dc2022-03-26T16:41:27ZCell-surface central nervous system autoantibodies: clinical relevance and emerging paradigms.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:550792b9-46b7-411f-afe6-5b6a458816dcEnglishSymplectic Elements at Oxford2014Irani, SRGelfand, JAl-Diwani, AVincent, AThe recent discovery of several potentially pathogenic autoantibodies has helped identify patients with clinically distinctive central nervous system diseases that appear to benefit from immunotherapy. The associated autoantibodies are directed against the extracellular domains of cell-surface-expressed neuronal or glial proteins such as LGI1, N-methyl-D-aspartate receptor, and aquaporin-4. The original descriptions of the associated clinical syndromes were phenotypically well circumscribed. However, as availability of antibody testing has increased, the range of associated patient phenotypes and demographics has expanded. This in turn has led to the recognition of more immunotherapy-responsive syndromes in patients presenting with cognitive and behavioral problems, seizures, movement disorders, psychiatric features, and demyelinating disease. Although antibody detection remains diagnostically important, clinical recognition of these distinctive syndromes should ensure early and appropriate immunotherapy administration. We review the emerging paradigm of cell-surface-directed antibody-mediated neurological diseases, describe how the associated disease spectrums have broadened since the original descriptions, discuss some of the methodological issues regarding techniques for antibody detection and emphasize considerations surrounding immunotherapy administration. As these disorders continue to reach mainstream neurology and even psychiatry, more cell-surface-directed antibodies will be discovered, and their possible relevance to other more common disease presentations should become more clearly defined.
spellingShingle Irani, SR
Gelfand, J
Al-Diwani, A
Vincent, A
Cell-surface central nervous system autoantibodies: clinical relevance and emerging paradigms.
title Cell-surface central nervous system autoantibodies: clinical relevance and emerging paradigms.
title_full Cell-surface central nervous system autoantibodies: clinical relevance and emerging paradigms.
title_fullStr Cell-surface central nervous system autoantibodies: clinical relevance and emerging paradigms.
title_full_unstemmed Cell-surface central nervous system autoantibodies: clinical relevance and emerging paradigms.
title_short Cell-surface central nervous system autoantibodies: clinical relevance and emerging paradigms.
title_sort cell surface central nervous system autoantibodies clinical relevance and emerging paradigms
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