Objective To study the effect of methylprednisolone shock therapy on electrical status epilepticus during sleep (ESES) in children and the changes of immune function before and after methylprednisolone shock therapy. Methods Thirty-five children hospitalized in pediatric neurology ward of Shengjing Hospital of China Medical University from October 2017 to October 2018 were selected, including 19 children with newly diagnosed temporal lobe epilepsy (TLE group) and 16 children with initial diagnosis of TLE with ESES (ESES group), and 21 healthy children in the same period were selected as normal control group.A total of 32 cases with ESES who were taken oral administration of more than 2 anti-epileptic drugs(AEDs)ineffective were given methylprednisolone shock treatment for 3 days.Changes of lymphocyte subsets and cytokines among groups were compared. Results The longer the course of disease, the worse the prognosis of children with ESES.Compared with normal control group, the NK cell activity were significantly lower in children of ESES group(P<0.05). The levels of IL-6 were higher in children of ESES group compared with that in children of TLE group, and there was significant difference between two groups(P<0.05). In children with ESES who had poor therapeutic effect of oral AEDs, the proportion of peripheral blood B lymphocytes increased after methylprednisolone shock treatment, and the proportion of T lymphocyte subsets and NK cells decreased, and the differences were statistically significant(P<0.05). The levels of IL-2, IL-4 and IL-10 were higher than those before treatment, the levels of IL-6, IL-17, INF-γ and TNF were lower than those before treatment, and the difference between the level of IL-6 before and after treatment was statistically significant(P<0.05). EEG spike waves index decreased significantly after treatment, some children′s growth and development and cognitive function improved, all children had no serious infection, high blood pressure, electrolyte imbalance and other adverse reactions.The correlation between therapeutic effect and changes of immune indexes was statistically significant in levels of NK cells(r=0.50, P<0.01), B lymphocytes (r=0.35, P=0.04) and IL-6(r=0.46, P=0.01), and all of them were positively correlated(P<0.05). Conclusion Children with ESES have immune dysfunction and may have excessive inactivation of NK cells and elevated levels of IL-6.Congenital immunity and adaptive immunity may play a role in the pathogenesis and pathophysiology of ESES syndrome.Methylprednisolone shock therapy could significantly reduce the non-rapid eye movement discharge index in children with ESES.The mechanism may be related to the significant decrease of IL-6 level.It may also be related to redistribution of lymphocyte subsets, affecting cell differentiation and balancing various cytokines. Key words: Electrical status epilepticus during sleep; Lymphocyte; Cytokines; Methylprednisolone