Effect of peripheral blood cell subtypes on immunopatogenesis in west syndrome

West Syndrome is an epileptic encephalopathy that typically occurs in infants and is characterized by hypsarrhythmia, infantile spasms, and neurodevelopmental prognosis. In some autoimmune diseases accompanied by seizures, autoimmunity-related antibodies and cytokines in the serum and cerebrospinal fluids of patients are thought to trigger epileptiform activity in infancy epileptic encephalopathies, depending on the immune response mediated by monocytes, T and B cells. Our aim is to reveal the immune pathogenesis and especially inflammasome activity in WS. Eight WS cases treated with synacthen and 11 age- and sex-matched healthy volunteers were included in our study. Peripheral blood mononuclear cells were isolated from the blood of the patients and immunophenotyping was performed, the expressions of NFκB transcription and NLRP3 inflammasome factor were evaluated before and after treatment. In our study, there was no difference in the B, T, NK and NKT cells, while an increase in pre-treatment naive B cells (CD19+IgD+CD27-) was found compared to the controls (p=0.018). Before treatment, a decrease was determined in transformed memory B cells (CD19+IgD-CD27+) compared to controls (p=0.0108), the increase in plasma (CD19+CD38+CD138+) cells was significant (p=0.004), the increase in plasmablasts (CD19+CD38+CD138-) was close to significance (p=0.055). A significant increase was observed in pre-treatment CD8+ cytotoxic T cells compared to controls (p=0.04). When evaluating the percentages of NLRP3 and NFkB, only a significant (p=0.0225) increase was observed in the NKT cells compared to controls in the post-treatment group (p=0.0954), and a close to significant (p=0.0225) increase was observed in the pre-treatment group compared to controls. A significant difference was found in CD4+ helper T cells (p=0.0313) after treatment in patients. A decrease in CD8+ cytotoxic T cells and Breg (CD19+CD38++24++) cells was close to significance (p=0.0625; p= 0.0625). Our findings support the views that inflammation-related mechanisms may cause seizures in WS cases and play a role in the pathogenesis of epilepsy, and are also important in terms of elucidating the pathophysiology of the disease and creating new treatment options in treatment-resistant patients. Key Words: Epileptic Encephalopathy, Infantile spasm, Immunophenotyping, Inflammasome The present work was supported by the Research Fund of Istanbul University. Project No: 37173