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.
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 etiology of patients with convulsive status epilepticus (CSE).MethodsBy taking epilepsy, seizure, status epilepticus, and epileptic seizure as keywords, the clinical data of epilepsy patients hospitalized in the First People’s Hospital of Longquanyi District of Chengdu and the People’s Hospital of Leshan from January, 2012 to December, 2017 were retrospectively collected from a retrieval system for electronic patient records. The collected CSE cases were screened by trained epilepsy specialists in strict accordance with inclusion criteria and exclusion criteria. The etiology of CSE, and the pathogenetic distinctions among patients with different ages, sexes, educational levels, places of residence, and histories of epilepsy were analyzed based on medical histories and accessory examinations. The prognostic factors for epilepsy were determined using logistic regression analysis.ResultsIn this study, a total of 852 hospitalized epilepsy cases were retrieved, among which 104 cases were CSE cases aged between 18 and 86, including 75 males and 29 females. There were 13 CSE deaths (12.5%). There were significant differences in the pathogeneses among CSE patients with different ages and histories of epilepsy (χ2=52.396, 18.354; P<0.05). However, no significant difference in CSE pathogeneses was observed among patients with different sexes, educational levels, or places of residence (P>0.05). Drug withdrawal or dose reduction was the leading cause of CSE in patients with a history of epilepsy (n=28, 57.1%), while cerebrovascular diseases (n=19, 34.5%) were common causes among those without a history. Among patients aged over 65, cerebrovascular diseases (n=17, 43.6%) were determined as the major causes of CSE, while for those aged under 65, drug withdrawal or dose reduction was the main pathogeny (n=20, 30.8%). Results obtained from multivariate logistic regression analysis on the prognostic factors for epilepsy showed that the duration of epileptic seizure significantly influenced the prognosis of patients [odds ratio=1.299, 95% confidence interval (1.074, 1.571), P=0.007], while there were no significant correlations between other factors and epilepsy prognosis (P>0.05).ConclusionsCerebrovascular diseases are the leading causes of geriatric CSE. Irregular medication of epilepsy patients is a prominent avoidable trigger for CSE.
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.
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 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.
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.
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.
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 clinical symptom and risk factors of diabetic seizures. MethodsThe clinical data of 44 patients with diabetes related seizures were analyzed with the clinical classification, blood glucose, Na+, Plasma Osmotic Pressure, HbA1c, EEG, brain MR, and the antiepileptic drugs. Results① Diabetic hyperglycemia (DH) related seizures: among the 28 patients, 17 cases were male patients, 11 cases were female patients. The mean age was 51.3 years old. Simple partial seizure without secondary generalized seizures (12/28, 42.8%) was the most common, 8 patients (8/28, 28.6%) showed complex partial seizure, 8 patients (8/28, 28.6%) showed no obvious focal origin generalized tonic-closure seizures. Patients with poor glycemic control (HbA1c > 9%) had significantly higher risk of generalized seizures (46.7% vs. 7.7 %, P < 0.05) (P < 0.05). ② Diabetic ketoa-cidosis or hypertonic state associated seizures: among the 7 patients, 6 cases were male patients, 1case was female patients. The mean age was 45.7 years old, 2 patients (2/7, 28.6%) had generalized tonic-clonic seizure, 2 patients (2/7, 28.6%) showed status epilepticus, 2 patients (2/7, 28.6%) showed local motor seizure, 1 patient (1/7, 14.2%) showed Jackson seizure. ③ Diabetic hypoglycemia related seizures: among the 9 patients, 7 cases were male patients, 2 cases were female patients. The mean age was 45.3 years old.5 patients showed generalized tonic-clonic seizure (5/9, 55.6%), 3 patients had complex partial seizure (3/9, 33.3%), 1 patients had generalized tonic-closure seizures (1/9, 11.1%). ConclusionSimple partial seizure is the most common in patients with diabetic hyperglycemia related seizures; so as to diabetic hypoglycemia and keto-acidosis, generalized seizures are relatively common. HbA1c can be an important risk factor of seizures for patients with hyperglycemia.