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

Stefania Kaninia, Alexandros Grammatikos, Kathryn Urankar, Shelley A Renowden, Nikunj K Patel, Mark M Gompels, Claire M Rice

Multiple sclerosis (Houndmills, Basingstoke, England) 2021 Aug

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The cytotoxic T-lymphocyte antigen-4 (CTLA-4) pathway acts as a negative immune regulator of T-cell activation and promotes self-tolerance. 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. 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.

Citation

Stefania Kaninia, Alexandros Grammatikos, Kathryn Urankar, Shelley A Renowden, Nikunj K Patel, Mark M Gompels, Claire M Rice. CNS demyelination associated with immune dysregulation and a novel CTLA-4 variant. Multiple sclerosis (Houndmills, Basingstoke, England). 2021 Aug;27(9):1464-1467