Objectives Retrospective analysis of the Tibetan convulsive status epilepticus (CSE) for the aetiology, prognosis and its influencing factors in Tibet area. Methods Through electronic patient record, making “epilepsy”, “status epilepticus ”, “epileptic seizure” as keywords, convulsive status epilepticus patients in the People’s Hospital of Tibet Autonomous Region hospitalized from January 2015 to December 2020 were retrospectively observed, gathering their clinical data and aided examinations furthermore, and the prognoses were returned by telephone, meanwhile the functional status of those patients was assessed by the modified rankin scale. and the causes differ in gender, age, out-of-hospital antiepileptic treatment, family history of epilepsy and history of epilepsy were analyzed. The prognostic factors were analyzed by logistic regression. Results A total of 2 254 hospitalized patients with epilepsy were retrieved, including 331 CSE patients aged 14~84 years, 219 males and 112 females. There were 36 lost calls, 62 CSE deaths (21.01%), and 4 adverse outcomes (non-death)(1.7%).There were statistically significant differences in etiology of CSE in different ages and history of epilepsy (P<0.05), but there were no statistically significant differences in gender, out-of-hospital antiepileptic treatment, progression of refractory status epilepticus and family history of epilepsy. Cerebrovascular disease was the main cause of CSE in people aged 45 and over (54 cases), while the main cause of CSE in people aged under 45 was unknown (104 cases).Among the patients with previous history of epilepsy, the highest proportion was unknown cause [117 cases (48.8%)]; Among patients without a history of epilepsy, cerebrovascular disease [34 cases (37.4)] was the most common cause of CSE. Multivariate logistic regression analysis of prognostic factors of CSE showed that gender, age, GCS and electrolyte disorder had statistically significant effects on the death of CSE patients (P<0.05), while altitude and their duration and other factors had no statistically significant effects on the death of CSE patients (P>0.05). ConclusionsCerebrovascular disease is the leading cause of CSE in people aged 45 and over. Male, advanced age, low GCS score at discharge, and electrolyte disorder were risk factors.
ObjectiveTo study the clinical features of children with seizures as core symptoms of neuronal surface antibody syndromes. MethodsThe clinical data of neuronal surface antibody syndromes between December 2015 and December 2016 were obtained and analyzed. All children presented to hospital with seizures as core symptoms. ResultsThere were 1 male and 9 females in this study. The ages ranged from 3 years to 13 years. The disease course was between 3 and 14 days. All children presented to hospital with seizures as core symptoms.Two children had tonic seizures. one had tonic-clonic seizure. Seven had partial seizures. Among them, six children had status epilepticus and cluster attack. The other symptoms in the course of the disease were psychiatric symptoms and extrapyramidal symptoms.The anti-NMDAR antibody were found in 9 patients' CSF and blood. The LGI1 antibody was found in one patients' CSF and blood.The EEG test of 7 patients showed slow wave and sharp slow wave. Two showed spike wave. One showed slow wave.The MRI test of one patient showed abnormal. Ten cases were treated with IVIG and methylprednisolone during acute stage. The patients had been followed up for 3 to 6 months. Eight of them recovered completely. Two cases had seizures. Two cases diagnosed with anti-NMDAR related epilepsy received sound effects after treated with cyclophosphamide. ConclusionsConvulsion may be the first common symptom of neuronal surface antibody syndromes in children. Immune factors should be screened when children with acute seizures and status epilepticus. Accompanying psychiatric symptoms, autoimmune epilepsy should be considered. The most common neuronal surface antibody in children with neuronal surface antibody syndromes is NMDAR antibody. EEG usually shows slow wave and sharp slow wave during seizures. Brain MRI is usually normal. Immunotherapy is effective in the majority of patients as the first line treatment. When the first-line treatment failed, second-line immunotherapy such as cyclophosphamide shock therapy on a regular basis is helpful.
Objective Using cortex convulsions threshold detector and electrical stimulation in rats cortex convulsions threshold model, compare the efficacy and aging of domestic lamotrigine (LTG) and imported LTG. Methods Electrical stimulation convulsions threshold model in rats after stability, 40 rats were randomly divided into A、B、C、D groups,AandBgroup were divided into three different dose groups: domestic LTG low dose (12.5 mg/kg/d), middle dose (25 mg/kg·d), high dose group (37.5 mg/kg·d); imported LTG low doses (12.5 mg/kg·d), middle dose (25 mg/kg·d), high dose group (37.5 mg/kg·d); Carbamazepine middle dose group (72 mg/kg·d); the control group (normal saline 2 ml/time). Recording electrical stimulation in rats cortex convulsions threshold model after administration, compare the differences before and after the administration. Results Three different dose groups of domestic LTG and imported LTG all hadahigher level of electrical stimulation cortex convulsions threshold, and showedadose-response relationship. Onset time of LTG after administration was 1 to 2 hours, peak time was 3 to 4 hours, maintaining time was 8 to 10 hours. Conclusion LTG can improve cortex convulsions threshold in the electrical stimulated rats, there was no significant difference with carbamazepine, and showedadose-response relationship; Repeat dosing for 4 days, both domestic and imported LIG can maintain effective anticonvulsive effect, the efficacy and the aging of two groups of LTG have no significant difference (P＞0.05).
Genetic epilepsy with febrile seizures plus (GEFS+) is a new type of genetic epilepsy syndrome with a marked hereditary tendency. Febrile seizure is the most common clinical symptom, followed by febrile seizure plus, and with/without absence seizures, focal seizures, and generalized tonic-clonic seizures. Results of the polymerase chain reaction (PCR), exon sequencing and single nucleotide polymorphism (SNP) analysis showed that the occurrence of GEFS+ is mainly related to the mutation of gamma aminobutyric acid type A receptor gamma 2 subunit (GABRG2), but its pathogenesis was still unclear. The main types of GABRG2 mutations include missense mutation, nonsense mutation, frameshift mutation, point mutation and splice site mutation. All these types of mutations can reduce the function of ion channels on cell membrane, but the degree and mechanism of dysfunction are different, which may be the main mechanism of epilepsy. This article will focus on the relationship between GEFS+ and the mutation types of GABRG2 in recent years, which is of great significance for clinical accurate diagnosis, anti-epileptic treatment strategy and new drug development.
Febrile seizures (FS) are one of the most common neurological disorders in pediatrics, commonly seen in children from three months to five years of age. Most children with FS have a good prognosis, but some febrile convulsions progress to refractory epilepsy (RE). Epilepsy is a common chronic neurological disorder , and refractory epilepsy accounts for approximately one-third of epilepsies. The etiology of refractory epilepsy is currently complex and diverse, and its mechanisms are not fully understood. There are many pathophysiological changes that occur after febrile convulsions, such as inflammatory responses, changes in the blood-brain barrier, and oxidative stress, which can subsequently potentially lead to refractory epilepsy, and inflammation is always in tandem with all physiological changes as the main response. This article focuses on the pathogenesis of refractory epilepsy resulting from post-febrile convulsions.
ObjectiveUsing Quality in prognosis studies (QUIPS) analysis, this paper systematically reviewed the factors influencing the poor outcome of children with convulsive status epilepticus (CSE).MethodsTo longitudinal cohort studies on the prognostic evaluation of CSE mortality and mobidity in children.The retrieval time was from January 2008 to November 2019, and three system reviewers PUBMED, EMBASE, COCHRANE and other databases were used to search for literatures related carried out literature extraction and quality evaluation. According to the QUIPS analysis method, the included literatures were scored, the quality grade was divided, and the analysis variables of medium/high quality literatures with statistical significance were selected to draw a conclusion.ResultsQUIPS analysis was used to assess the literature quality, 17 medium/high quality literatures were included, and the factors with statistical significance (P<0.05) mentioned at least twice or more in≥2 medium/high quality literatures were selected, which were considered as important risk factors affecting prognosis.These factors include: etiology, age, duration of convulsion, refractory CSE, neuroimaging abnormalities.ConclusionFive risk factors indicating poor outcome of CSE in children were summarized. Due to the heterogeneity of various literature studies, Meta-analysis has not been completed, so it has certain limitations.
Objective To investigate the diagnosis and treatment of status epilepticus in hospitals of different levels and the knowledge of status epilepticus in clinical physicians, in order to better guide clinical education in the future. Methods From August 2014 to August 2015, a questionnaire was designed and used to investigate the general situation of the hospital, the diagnosis of status epilepticus and the clinical practice among trainee doctors and students in the epilepsy training class in the Neurological Intensive Care Unit and the Department of Neurology of West China Hospital, Sichuan University. The results of the investigation were statistically analyzed. Results Ninety questionnaires were distributed, and all the questionnaires were retrieved with validity. The number of investigated physicians was 42 (46.7%) from the Department of Neurology, 6 (6.7%) from the Department of Neurosurgery, 30 (33.3%) from the Intensive Care Unit and 12 (13.3%) from other departments. Twenty-seven (30.0%) physicians were from class Ⅲ grade A hospitals, 31 (34.4%) from class Ⅲ grade B hospitals, and 32 (35.6%) from class Ⅱ grade A hospitals. All the class Ⅲ hospitals and 53.1% of class Ⅱ hospitals had electroencephalograph monitoring facilities. The proportion of status epilepticus patients ranged from 0.5% to 10.0% in different hospitals. There were great differences in the identification and treatment of convulsive status epilepticus among different hospitals. Conclusions Status epilepticus is a common emergency. Questionnaire survey is an effective means to reflect the difference in identifying and treating the emergency among different departments and hospitals. It can guide clinical education and promote the identification and treatment of the emergency more accurately in doctors of all levels.
ObjectiveTo study the clinical and EEG features, therapeutic response and prognosis of eyelid myoclonia-nonconvulsive status epilepticus (EM-NCSE) in children.MethodsCollected the clinical and EEG data of 3 children with EM-NCSE that were diagnosed in department of neurology in Qilu Children Hospital of Shandong university during the January in 2015 to August in 2016.Analysed the therapeutic response to antiepletic drugs(AEDs).ResultsAmong the three children, there were 2 girls and 1 boy.The age at the onset of the disease was from 6 to 10 years old.The average age of them is 8.67 years old.The clinical manifestations include mental confusion, dysphoria, winking and scrolling up the eyes.The typical vedio electroencephalography (VEEG) in the patients showed 3~6 Hz generalized spike and waves and polyspikes burst, especially in the frontal and the anterior temporal region.In addition, the eye closure and intermittent photic stimulation helped to induce discharges and clinical events as eyelid myoclonia (EM).ConclusionsEM-NCSE is one of the idiopathic and generalized epileptic disease and characterized by EM.Video EEG monitoring plays an important role in the diagnosis of this disease.The drugs of choice for treatment was diazepam.When the event was controlled, AEDs were effective for the following therapy.