CNS demyelination associated with immune dysregulation and a novel CTLA-4 variant

BACKGROUND: The cytotoxic T-lymphocyte antigen-4 (CTLA-4) pathway acts as a negative immune regulator of T-cell activation and promotes self-tolerance. CASE: We report the first case of biopsy-proven central nervous system inflammatory demyelination in the context of primary immunodeficiency and a n...

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Autores principales: Kaninia, Stefania, Grammatikos, Alexandros, Urankar, Kathryn, Renowden, Shelley A, Patel, Nikunj K, Gompels, Mark M, Rice, Claire M
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2021
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8358566/
https://www.ncbi.nlm.nih.gov/pubmed/34097529
http://dx.doi.org/10.1177/1352458520963896
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author Kaninia, Stefania
Grammatikos, Alexandros
Urankar, Kathryn
Renowden, Shelley A
Patel, Nikunj K
Gompels, Mark M
Rice, Claire M
author_facet Kaninia, Stefania
Grammatikos, Alexandros
Urankar, Kathryn
Renowden, Shelley A
Patel, Nikunj K
Gompels, Mark M
Rice, Claire M
author_sort Kaninia, Stefania
collection PubMed
description BACKGROUND: The cytotoxic T-lymphocyte antigen-4 (CTLA-4) pathway acts as a negative immune regulator of T-cell activation and promotes self-tolerance. CASE: We report the first case of biopsy-proven central nervous system inflammatory demyelination in the context of primary immunodeficiency and a novel CTLA-4 variant. CONCLUSION: This case has significant implications for the development of novel treatments for autoimmune conditions including multiple sclerosis and further emphasises the need for caution with clinical use of CTLA-4 immune checkpoint inhibitors in those with a history of inflammatory demyelination.
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spelling pubmed-83585662021-08-13 CNS demyelination associated with immune dysregulation and a novel CTLA-4 variant Kaninia, Stefania Grammatikos, Alexandros Urankar, Kathryn Renowden, Shelley A Patel, Nikunj K Gompels, Mark M Rice, Claire M Mult Scler Case Report BACKGROUND: The cytotoxic T-lymphocyte antigen-4 (CTLA-4) pathway acts as a negative immune regulator of T-cell activation and promotes self-tolerance. CASE: We report the first case of biopsy-proven central nervous system inflammatory demyelination in the context of primary immunodeficiency and a novel CTLA-4 variant. CONCLUSION: This case has significant implications for the development of novel treatments for autoimmune conditions including multiple sclerosis and further emphasises the need for caution with clinical use of CTLA-4 immune checkpoint inhibitors in those with a history of inflammatory demyelination. SAGE Publications 2021-06-07 2021-08 /pmc/articles/PMC8358566/ /pubmed/34097529 http://dx.doi.org/10.1177/1352458520963896 Text en © The Author(s), 2021 https://creativecommons.org/licenses/by/4.0/This article is distributed under the terms of the Creative Commons Attribution 4.0 License (https://creativecommons.org/licenses/by/4.0/) which permits any use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Report
Kaninia, Stefania
Grammatikos, Alexandros
Urankar, Kathryn
Renowden, Shelley A
Patel, Nikunj K
Gompels, Mark M
Rice, Claire M
CNS demyelination associated with immune dysregulation and a novel CTLA-4 variant
title CNS demyelination associated with immune dysregulation and a novel CTLA-4 variant
title_full CNS demyelination associated with immune dysregulation and a novel CTLA-4 variant
title_fullStr CNS demyelination associated with immune dysregulation and a novel CTLA-4 variant
title_full_unstemmed CNS demyelination associated with immune dysregulation and a novel CTLA-4 variant
title_short CNS demyelination associated with immune dysregulation and a novel CTLA-4 variant
title_sort cns demyelination associated with immune dysregulation and a novel ctla-4 variant
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8358566/
https://www.ncbi.nlm.nih.gov/pubmed/34097529
http://dx.doi.org/10.1177/1352458520963896
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