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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Detalles Bibliográficos
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
Descripción
Sumario: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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