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 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.