ObjectiveTo investigate the effect of high-dose hormone pulse therapy on children with epilepsy and sleep status epilepticus.Methods60 children were randomly divided into control group (30 cases) and study group (30 cases) according to the admission sequence. The control group was treated with antiepileptic drugs (according to clinical electroencephalogram or syndrome), and the study group was treated with methylprednisolone sodium succinate [20 mg/(kg·d) × 3 days, once a month, for 6 months]. During the period, antiepileptic drugs were taken normally. After 6 months, the curative effect, abnormal EEG and side effects were compared.ResultsAfter 6 months of treatment, the clinical conditions of the two groups were significantly improved (effective rate: control group: 19/30 vs. study group: 26/30), and the clinical efficacy (26/30) and EEG improvement (26/30) of the study group were better than those of the control group (P<0.05), weight gain can be improved by diet control and reasonable exercise.ConclusionHormone pulse therapy for children with epilepsy and sleep status epilepticus in the control of clinical seizures and improve the effect of abnormal EEG, recommended.
Severe psychomotor developmental delay resulting from early postnatal (within 3 months) seizures can be diagnosed as Early-Infantile Developmental and Epileptic encephalopathies (EIDEE). Its primary etiologies include structural, hereditary, metabolic and etc. The main pathogenesis may be related to the inhibition of normal physiological activity of the brain by abnormal electrical activity and the damage of the brain neural network. Ohtahara syndrome and Early Myoclonic Encephalopathy (EME) are typical types of EIDEE. The principle of treatment is to improve the cognitive and developmental function by controlling frequent seizures. When the seizure is difficult to control with drugs, surgical evaluation should be performed as soon as possible, and surgical treatment is the first choice for patients suitable for surgery. The types of surgery can be divided into excision surgery, dissociation surgery, neuromodulation surgery and etc. The current status of surgical treatment of EIDEE was described, and the curative effect of surgical treatment was explored, so as to help clinicians choose appropriate treatment methods.
ObjectiveTo investigate the knowledge of drug safety among parents of some children with epilepsy in Henan Province, and to provide a basic advice for drug safety and health education for children with epilepsy.sMethodUsing a questionnaire to parents of epilepsy children from the Children's Hospital in Henan Province and the First Affiliated Hospital of Zhengzhou University in July 2019, a total of 336 questionnaire were conducted by the professionals responsible for after the training, including parents and children basic situation, drug habits and attitudes, medication safety consciousness and antibiotic drug use knowledge.Results320 questionnaire were collected, 314 of which were valid questionnaires, with an effective rate of 98.1%. 66 (21%) of parents chose juice or milk for oral administration, and 87 (27.7%) of parents chose sugar water. 104 (33.1%) of the parents chose to stop the medication immediately after the child's condition improved. 126 (40.1%) of parents believe that combination of two or more antibiotics is more effective, 178 (56.7%) of parents will give their children antibiotics when they have a cold or fever, and more than 254 (80%) of parents are not aware of adverse reactions to antibiotics.ConclusionIn the investigation, the parents of children with epilepsy in Henan Province still have insufficient knowledge of safe drug use, and there are many non-standard drug use behaviors. Local medical professionals for epilepsy should be strengthened to guide and monitor the children and their parents.
In recent years, epileptic seizure detection based on electroencephalogram (EEG) has attracted the widespread attention of the academic. However, it is difficult to collect data from epileptic seizure, and it is easy to cause over fitting phenomenon under the condition of few training data. In order to solve this problem, this paper took the CHB-MIT epilepsy EEG dataset from Boston Children's Hospital as the research object, and applied wavelet transform for data augmentation by setting different wavelet transform scale factors. In addition, by combining deep learning, ensemble learning, transfer learning and other methods, an epilepsy detection method with high accuracy for specific epilepsy patients was proposed under the condition of insufficient learning samples. In test, the wavelet transform scale factors 2, 4 and 8 were set for experimental comparison and verification. When the wavelet scale factor was 8, the average accuracy, average sensitivity and average specificity was 95.47%, 93.89% and 96.48%, respectively. Through comparative experiments with recent relevant literatures, the advantages of the proposed method were verified. Our results might provide reference for the clinical application of epilepsy detection.
ObjectiveTo systematically evaluate the efficacy of steroids combined with antiepileptic drugs compared with alone antiepileptic drugs in the treatment of children with epileptic electrical status during sleep to provide evidence-based medical basis for its treatment.MethodsElectronic searches were maded in PubMed, Embase database, Cochrane Library, CNKI, Wanfang and the Chinese biomedical literature database for the literature about steroids combined with antiepileptic drugs compared with alone antiepileptic drugs in the treatment, and retrieval time is from January 1990 to October 2020. Two evaluators independently screened literature, extracted data, evaluated literature quality and risk of bias and checked each other. Meta analysis was performed by stata16.0 software.ResultsA total of 679 children with ESES were included in 10 studies, including 9 randomized controlled trials and 1 retrospective cohort study. Meta analysis results showed that there were statistically significant differences in clinical improvement rate [RR=1.31, 95%CI (1.21, 1.42), P<0.01], electroencephalogram discharge improvement rate [RR=1.35, 95%CI (1.25, 1.46), P<0.01] and cognitive intelligence score [SMD=1.19, 95%CI (0.80, 1.57), P<0.01] between steroids combined with antiepileptic drugs group and alone antiepileptic drugs group after 6 months follow-up. The incidence of adverse reactions in steroids combined with antiepileptic drugs group was higher than that in alone antiepileptic drugs group, and the difference was statistically significant [RR=4.13, 95%CI (1.06, 16.13), P<0.01]. All adverse reactions improved or disappeared after drug withdrawal.ConclusionCompared with alone antiepileptic drugs, steroids combined with antiepileptic drugs group has advantages in controlling epileptic seizures, improving electroencephalogram abnormalities and improving cognitive ability, and it is relatively safe.
ObjectiveTo explore the clinical, genetic and prognostic features of early infantile epileptic encephalopathy caused by DNM1 gene pathogenic variations.MethodsClinical phenotype, genotype and prognosis of 3 individuals with de novo variants in DNM1 gene were analyzed retrospectively. Through using “Dynamin-1” or “DNM1” as key words to search literature at database of China National Knowledge Infrastructure, Wanfang, PubMed and OMIM. Genotype-phenotype correlations were analyzed by analysis of variance (ANOVA).ResultAmong the 3 patients, 1 female and 2 males. 2 cases with epileptic spasm and 1 case with focal clonic seizure or secondary generalized tonic-clonic seizure were manifested with onset age from 2 to 17 months. De novo variants at NM_004408.4: c.415 G>A(P. Gly 139Arg) in 2 inviduals and NM_004408.4: c.545 C>A(P. Ala 182Asp)in 1 invidual of DNM1 gene were identified by gene testing. After follow-up at age of 2~3 years, all patients were presented with hypotonia, severe intellectual disability, non-verbal, non-ambulatory, drug-resistant epilepsy and feeding difficulties. 36 cases with pathogenic DNM1 variants were reported by far, totally 39 cases were included. Of the 39 patients, hypotonia were found to be independent of the locus of genetic variants, while those inviduals with variants in the GTPase and middle domains almost presented severe or profound intellectual disability and epilepsy. 31 patients diagnosed with epilepsy and complete clinical data were further analyzed, epileptic spasm was the most common types of seizure. Absent seizure was significantly more common in those patients with variants in the GTPase domains (P=0.02), compared to those patients with variants in the middle domains. No statistical differences were found in gender, onset age, other types of seizure and drug treatment response between variants in the GTPase and middle domains.ConclusionHypotonia, early onset epilepsy, severe intellectual and movement disability were the common features in patients with DMN1 related encephalopathy. Epileptic spasm was the most common types of seizure, no significant differences were found in the phenotype between the GTPase and middle domains expect for absent seizure. Our patients also presented with feeding difficulties.
According to their seizure patterns and EEG findings, status epilepticus can be divided into convulsive status epilepticus (CSE) and nonconvulsive status epilepticus (NCSE). Patients with NCSE have well-established EEG abnormalities without typical convulsive convulsions and only altered mental status or mild motor symptoms. Due to its atypical clinical symptoms, NCSE is prone to delayed diagnosis, misdiagnosis, or missed diagnosis, resulting in irreversible brain tissue damage, severe impairment of consciousness, function, and behavior, and even death in NCSE patients. It is of great significance to actively prevent seizures, identify symptoms early, and standardize treatment to improve the prognosis of NCSE patients. At present, there is no relevant standard and consensus on NCSE diagnosis and care. Here, we reported a patient with NCSE who admitted to the Epilepsy Center of Beijing Tiantan Hospital on June 21, 2024. After precise treatment and nursing, the patient's symptoms were well controlled, his condition was stable, and he was followed up for 1 month after discharge, and the prognosis was good. This case report aimed to provide some clinical suggestions to related disease.
In order to improve the epilepsy management and treatment of Hebei province, improve the life quality of epilepsy patients. Hebei Association Against Epilepsy start a multicenter-clinical trial about the diagnosis, medicine treatment and effect of epilepsy through mobile phone APP. The data collected by health management APP shows that the diagnosis rate of epilepsy syndrome has enhanced from 21% to 39% within six months; also 80.4% of patients have got seizure-free within the first month of treatment. Therefore, the diagnosis and treatment of epilepsy in Hebei province has been improved. However, only 46% of adult patients have been hospitalized within the first 2 years of seizure onset. Therefore, patients need further education about epilepsy in the future. In this trial, the daily management of patients by doctors has come true through the use of mobile phone APP. Through the mobile phone APP, doctors achieved the real-time supervision of disease progress and adjustment of the treatment. This trial provide evidence for future treatment and daily management of epilepsy patients.
ObjectiveTo observe the effects of Tianjiang Niuhuang Powder on cognition, seizure and hippocampal formation in epileptic model of pilocarpine rats.MethodsFouty SD rats were selected in this study. They were randomly divided into four groups (each group n=10), including a blank control group (group A) and three pilocarpine epilepsy model groups. The model groups were divided into Tianjiang Niuhuang Powder treatment Group (group B), Sodium valproate (group C) and model control group (group D) according to the different processing methods. The seizures number of discharges in the EEG monitor, cognitive ability were observed on the 7th, 14th and 21st day, The pathological sections of the brain and the neuronal damage in the hippocampal CA1 area were observed after the rats were sacrificed.ResultsThere was no significant difference in the number of spontaneous episodes, duration, and average number of discharges per minute between group B and group C (P>0.05). Compared with groups B and C, group D increased the number of episodes and increased duration. The number of discharges increased, the difference was statistically significant (P<0.05). Group A had no seizures. There were no significant differences in cognitive ability between groups A, B and C (P>0.05). Compared with group A, B and C, group D had poor cognitive ability (P<0.05). The number of surviving cells in the hippocampus CA1 region was significantly improved compared with the D group (P<0.05). Compared with the A group, the viable cells in the hippocampal CA1 region of the three experimental groups were significantly lower (P<0.05).ConclusionsTianjiang Niuhuang Powder can reduce seizures, improve cognition and effectively alleviate neuronal damage in hippocampal CA1 neurons in rats with epilepsy.
ObjectiveThe aim of this study was to understand the relationship between IQ and glucose metabolism in brain cells in a wide variety of epilepsy subjects. MethodsThe study participants were 78 children with epilepsy and 15 healthy children for comparison. All participants were administered the Chinese Wechsler Intelligence Scale for Children (C-WISC). The verbal intelligence quotient (VIQ), performance intelligence quotient (PIQ) and full scale intelligence quotient (FIQ) were compared between epileptic children and typically developing children. 78 patients underwent interictal positron emission computed tomography (PET) using 2-deoxy-2[18F]fluoro-D-glucose (FDG) as the tracer for evaluating brain glucose metabolism. ResultsVIQ, PIQ and FIQ based on the C-WISC were significantly lower in epileptic children than those in the healthy comparison group (P < 0.001, P=0.001 and P < 0.001, respectively). The IQ of patients with normal metabolism, unifocal abnormal hypometabolism and multifocal abnormal hypometabolism determined by PET differed significantly. The extent of the abnormal hypometabolism was negatively correlated with the FIQ (rs=-0.549, P < 0.001). In patients with lateralized hypometabolism based on PET, the VIQ/PIQ discrepancy (︱VIQ-PIQ︱≥15 points)scores differed significantly between the left hemisphere abnormal hypometabolism and right hemisphere abnormal hypometabolism subgroups, being negative values in the left and positive values in the right subgroups(P=0.004). ConclusionsBrain metabolic abnormalities are correlated with IQ, and perfoming interictal PET along with C-WISC can better assess the extent of severity of cognitive impairment and VIQ/PIQ discrepancy.