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.
ObjectiveTo investigate the efficacy and safety of the phase Ⅰ corpus callosotomy in the treatment of adult refractory epilepsy. MethodsWe conducted a retrospective analysis of 56 adults with intractable epilepsy in Tangdu Hospital from January 2011 to July 2016.All patients were treated for the phase Ⅰ total corpus callosotomy, followed up 1~5 years after surgery. Results14 cases (25.0%) patients achieved complete seizure free after surgery, 19 cases (33.9%) whose seizures reduced more than 90%, 10 cases (17.9%) reduced between 50%~90%, 7 cases (12.5%) between 30%~50%, 6 cases (10.7%) decreased below 30%; Drop attacks of 47 cases (83.9%) patients disappeared. Postoperative complications occurred in 13 cases(23.2%), and most of them recovered well. 5 cases(8.9%) had long-term sensory disassociation, no serious complications and death. The percentage of patients reporting improvement in quality of life was 67.9%. ConclusionsFor patients with intractable epilepsy who can not undergo focal resection, Ⅰ phase total corpus callosotomy has a certain effect on reducing seizure frequency, eliminating drop attacks, and improving the quality of life.
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.
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.
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.
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 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.
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.
ObjectiveTo explore and clarify the relationship between epileptic seizure and inducing factors. Avoid inducing factors and reduce epileptic seizure, so as to improve the quality of life in patients with epilepsy.MethodsClinical data of 604 patients diagnosed with epilepsy in Xijing Hospital of Air Force Military Medical University from January 2018 to January 2019 were collected. The clinical data of patients with epilepsy were followed up 6 months.ResultsAmong the 604 patients, 318 (52.6%) were seizure-free in the last 6 months, 286 (47.4%) had seizures. 169 (59.1%) had seizures with at least one inducing factor. Common inducing factors: 123 cases of sleep disorder (72.8%), 114 cases of emotion changes (67.5%), 87 cases of irregular medication (51.5%), 97 cases of diet related (57.4%), 33 cases of menstruation and pregnancy (19.5%), etc. Using the χ2 test, seizures with age, gender differences had no statistical significance (P > 0.05), but seizure type was statistically different between inducing factors. In generalized seizures, tonic-clonic seizures associated with sleep deprivation (χ2= 0.189), absence seizures and anger (χ2= 0.237), pressure (χ2= 0.203), irregular life (χ2= 0.214). In the focal seizures, focal motor seizures was correlated with coffee consumption (χ2=0.145), focal sensory seizures with cold (χ2=0.235), electronic equipment use (χ2 =0.153), satiety (χ2 =0.257). Complex partial seizures was correlated with anger (χ2 =0.229), stress (χ2 =0.187), and cold (χ2 =0.198). The secondarily generalized seizures was correlated with drug missing (χ2 =0.231), sleep deprivation (χ2 =0.158), stress (χ2 =0.161), cold (χ2 =0.263), satiety (χ2 =0.182). Among the inducing factors, sleep deprivation was correlated with anger (χ2 =0.167), fatigue (χ2 =0.283), and stress (χ2 =0.230).ConclusionsEpileptic seizure were usually induced by a variety of factors. Generalized seizures were associated with sleep disorders, emotional changes, stress, irregular life, etc. While focal seizures were associated with stress, emotional changes, sleep disorders, cold, satiety, etc. An analysis of the triggers found that sleep deprivation was associated with anger, fatigue, and stress. Therefore, to clarify the inducing factors of epileptic seizure, avoid the inducing factors as much as possible, reduce the harm caused by seizures, and improve the quality of life of patients.
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.