Abstract
Neuroinflammation has been implicated in the pathogenesis of West syndrome (WS). Inflammatory cytokines, including interleukin-1β(IL-1β), have been reported to be associated with epilepsy. However, the assessment of cytokine changes in humans is not always simple or deterministic. This study aimed to elucidate the immunological mechanism of WS. We examined the intracellular cytokine profiles of peripheral blood cells collected from 13 patients with WS, using flow cytometry, and measured their serum cytokine levels. These were compared with those of 10 age-matched controls. We found that the WS group had significantly higher percentages of inter IL-1β, interleukin-1 receptor antagonist (IL-1RA)-positive monocytes, and interferon gamma (IFN-γ) in their CD8+ T cells than the control group. Interestingly, the group with sequelae revealed significantly lower levels of intracellular IFN-γ and IL-6 in their CD8+ T and CD4+ T cells, respectively, than the group without sequelae. There was no correlation between the ratios of positive cells and the serum levels of a particular cytokine in the WS patients. These cytokines in the peripheral immune cells might be involved in the neuroinflammation of WS, even in the absence of infectious or immune disease. Overall, an immunological approach using flow cytometry analysis might be useful for immunological studies of epilepsy.
Highlights
West syndrome (WS) is a severe epileptic encephalopathy, which causes spasm attacks and hypsarrhythmia on an electroencephalogram in infants and developmental delay in approximately 80–90% of cases [1]
Cytotoxic edema derived from inflammatory cytokines has been detected in WS using tensor imaging [8], and pharmacological targeting of the IL-1 receptor pathway has shown a close link between inflammatory cytokines and epileptogenesis in a mouse model [9]
An increase in IL-1β production could be observed in patients with phenotyping, and we found no difference between patients with WS and controls
Summary
West syndrome (WS) is a severe epileptic encephalopathy, which causes spasm attacks and hypsarrhythmia on an electroencephalogram in infants and developmental delay in approximately 80–90% of cases [1]. IL-1β is produced by microglia in the brain and has been reported to influence astrocytes to acquire epileptogenesis [12]. These results have not been demonstrated in West’s mouse model, nor has it been studied in humans. Shiihara et al reported decreased levels of IL-1β after ACTH therapy [13], recent studies, including our own, have not yielded equivalent findings and detected no significant differences in IL-1RA levels in a large number of samples [14]. We previously reported that serum IL-1RA levels increased after treatment, following the resolution of clinical symptoms. Some reports suggest that treatment with ACTH increases the number of CD4+ cells and CD3+ cells [15]
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