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).
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.
ObjectiveTo recognize the convulsion caused by hypoglycemia, and to analyze its genotype and clinical phenotype, so as to deepen the understanding of hyperinsulinemia.MethodFull exon detection were performed on 2 children with hypoglycemia and convulsions, who had been treated with antiepileptic drugs for 1 year in pediatric neurology department, Henan Provincial People’s Hospital in 2012 and 2014 respectively, but with poor curative effect.ResultABCC8 gene mutations were found in a child. The mutations located in Chromosome 11, with the nucleic acid changes of c.4607C>T (exon38) and the amino acid change of p.A1536V, rs745918247. The inheritancemode of ABCC8 gene could be autosomal dominant or autosomal recessive inheritance. Both of the parents were wild type on this genelocus. The gene mutation is associated with type 1 familial hyperinsulinemic hypoglycemia/nesidioblastosis. The other child was carrying GLUD1 gene mutation, witch is located in chromosome 10, with the nucleic acid changes of c.1498G>A (exon12) and the amino acid change of p.A500T. The inheritance mode of GLUD1 gene is autosomal dominant andthe child’s parents were both wild type. This gene mutationis associated with type 6 familial hyperinsulinemic hypoglycemia/nesidioblastosis. The 2 mutations have not been reported, which are new mutations.ConclusionMutations in these 2 gene loci may be the underlying cause of hypoglycemic convulsions, and are the best explanation for the poor convulsionscontrol of antiepileptic drugs.