CNS demyelination associated with immune dysregulation and a novel CTLA-4 variant.
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ABSTRACT: 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.
SUBMITTER: Kaninia S
PROVIDER: S-EPMC8358566 | biostudies-literature | 2021 Aug
REPOSITORIES: biostudies-literature
Publications
CNS demyelination associated with immune dysregulation and a novel <i>CTLA-4</i> variant.
Kaninia Stefania S Grammatikos Alexandros A Urankar Kathryn K Renowden Shelley A SA Patel Nikunj K NK Gompels Mark M MM Rice Claire M CM
Multiple sclerosis (Houndmills, Basingstoke, England) 20210607 9
<h4>Background</h4>The cytotoxic T-lymphocyte antigen-4 (CTLA-4) pathway acts as a negative immune regulator of T-cell activation and promotes self-tolerance.<h4>Case</h4>We report the first case of biopsy-proven central nervous system inflammatory demyelination in the context of primary immunodeficiency and a novel <i>CTLA-4</i> variant.<h4>Conclusion</h4>This case has significant implications for the development of novel treatments for autoimmune conditions including multiple sclerosis and fur ...[more]
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