Distribution of peripheral blood mononuclear cell subtypes in patients with West syndrome: Impact of synacthen treatment

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Soylu S., Cherkezzade M., Akbayır E., Korkmaz H., Koral G., Şanlı E., ...More

IMMUNOLOGY LETTERS, vol.261, pp.17-24, 2023 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 261
  • Publication Date: 2023
  • Doi Number: 10.1016/j.imlet.2023.07.007
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, Aquatic Science & Fisheries Abstracts (ASFA), BIOSIS, CAB Abstracts, Chemical Abstracts Core, EMBASE, Veterinary Science Database
  • Page Numbers: pp.17-24
  • Keywords: Epileptic encephalopathy, Immunophenotyping, Infantile spasm, Synacthen, West syndrome
  • Istanbul University Affiliated: Yes


Background: West Syndrome (WS) is an epileptic encephalopathy that typically occurs in infants and is characterized by hypsarrhythmia, infantile spasms, and neurodevelopmental impairment. Demonstration of autoantibodies and cytokines in some WS patients and favorable response to immunotherapy have implicated inflammation as a putative trigger of epileptiform activity in WS. Our aim was to provide additional support for altered inflammatory responses in WS through peripheral blood immunophenotype analysis. Methods: Eight WS cases treated with synacthen and 11 age- and sex-matched healthy volunteers were included. Peripheral blood mononuclear cells (PBMC) were isolated and immunophenotyping was performed in pretreatment baseline (8 patients) and 3 months post-treatment (6 patients) samples. The analysis included PBMC expressing NF & kappa;B transcription and NLRP3 inflammasome factors. Results: In pre-treatment baseline samples, switched memory B cells (CD19+IgD-CD27+) were significantly reduced, whereas plasma cells (CD19+CD38+CD138+) and cytotoxic T cells (CD3+CD8+) were significantly increased. Regulatory T and B cell ratios were not significantly altered. Synacthen treatment only marginally reduced helper T cell ratios and did not significantly change other T, B, NK and NKT cell and monocyte ratios. Conclusions: Our findings lend further support for the involvement of inflammation-related mechanisms in WS. New-onset WS patients are inclined to display increased plasma cells in the peripheral blood. Synacthen treatment does not show a beneficial effect on most effector acquired and innate immunity subsets.