ObjectiveTo analyze the efficacy of ketogenic diet (KD) in the treatment of epilepsy syndrome associated with Electrical status epilepticus during sleep (ESES) and the changes of electroencephalogram (EEG). MethodsThe clinical data of 58 children with ESES-related epilepsy syndrome and cognitive impairment admitted to the outpatient and inpatient department of Xuzhou Children’s Hospital from March 2020 to June 2022 were collected. They were divided into observation group (group A) and control group (group B) by random number table method, with 29 cases in each group. On the basis of not changing the original anti-seizure drugs, group A was treated with ketogenic diet, and group B was treated with hormone. The clinical efficacy, cognitive function and Spike-wave index (SWI) of children with ESES-related epilepsy syndrome were observed before and 3 and 6 months after ketogenic diet and hormone treatment. Results The total clinical effective rate and control rate of group A after 3 months of treatment; The total effective rate and significant efficiency of EEG and the verbal quotient, operation quotient and total intelligence quotient of Wechsler Intelligence Scale for children were significantly lower than those of group B, and the differences were statistically significant (P<0.05). However, the total clinical effective rate and control rate of group A after 6 months of treatment; The total effective rate and significant efficiency of electroencephalogram and the verbal quotient, operation quotient and total intelligence quotient measured by Wechsler Intelligence Scale for children were similar to those of group B, and the difference was not statistically significant (P>0.05). There were no cases of EEG SWI and recurrent clinical symptoms in group A during the follow-up period; In group B, the recurrence rate of EEG SWI was 55.2%, and the recurrence rate of clinical symptoms was 37.9% after 6 months of treatment. There were no serious adverse reactions in the two groups during the treatment period. In group A, about 27.6% (8 cases) of the children had gastrointestinal symptoms, but they were mild and quickly relieved, and no serious adverse reactions occurred in the maintenance period. However, 89.7% (26 cases) of the children in group B showed different degrees of weight gain, and 69% (20 cases) of them showed kexing features such as full moon face and buffalo back. ConclusionsKetogenic diet can improve the curative effect and EEG changes of children with ESES related epilepsy syndrome, and improve their cognition. Compared with hormone pulse therapy, the recurrence rate of ESES related epilepsy syndrome in the treatment of ketogenic diet is lower, and the incidence of adverse reactions is relatively mild and low.
Anti-seizure medications (ASMs) are the most important and basic treatment for epilepsy, and are also the first choice for epilepsy treatment, but about one-third of patients have drug resistance. Perampanel (PER), as a novel third generation ASMs, inhibits the α-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid receptor (AMPAR) through non-competitive inhibition. AMPA plays an anti-seizure role. Since its approval in China in 2021, it has been mainly used in the treatment of focal epilepsy (with or without general seizure) as a single drug or addition, and has good safety, effectiveness and tolerability. Self-limited epilepsy with centrotemporal spikes (SeLECTS) is a common childhood focal epilepsy syndrome, accounting for 15% ~ 25% of various childhood epilepsies, PER has important advantages in clinical studies and has shown certain curative effect. At the same time, the overall effect of PER on cognition was neutral, with no systemic cognitive deterioration or improvement. In view of the relatively short application time of PER, which is still a new drug, this article will review the mechanism of action, dose, add-on (single drug) treatment, adverse events and, in order to provide clinicians with more drug choices and facilitate the individualized diagnosis and treatment of epilepsy.
ObjectiveTo improve the clinicians’ understanding and treatment level of electrical status epilepticus during sleep by analyzing the clinical data of patients with ESES retrospectively.MethodsWe collected 20 cases from 2018 to 2019 diagnosed in our hospital by clinical and 24-hour video EEG confirmed and analyzed the clinical manifestations, EEG features, seizure type, classification of epilepsy syndrome as well as the imaging findings.ResultsAmong the 20 patients, there were 12 males and 8 females, the mean age was (10.96±2.68) years old, and the first onset age was (8.90±1.93) years old. Epilepsy seizure as the first symptom is most common, the EEG showed a broad or localized sustained discharge, generalized tonic clonic seizure is the main form of seizure type in most patients, ECSWS is the most prominent in the epilepsy syndrome, for small lesion through MR can be found early.ConclusionMost patients have partial or generalized seizures, which have a range of cognitive impairment, mental decline or other issues. It is important to improve the cognitive function, behavior, neuropsychology of patients by long term video EEG monitoring in the early stage.