ObjectiveTo optimize the therapy protocols of high dose prednisone combined with topiramate (TPM) in children with infantile spasms (IS). MethodsSixty cases were collected in our hospital from September 2012 to September 2013 and randomly divided into two groups(n=30) and followed-up for more than 6 months.The spasms were assesses by video-electroencephalogram (VEEG) monitoring including awake and asleep states before treatment, after two weeks of therapy and the end of the courses respectively.And the Gessel developmental quotient (DQ) scores were performed before treatment and after six months of therapy. ResultsFor the unresponders to high dose prednisone in one week of therapy, there were 46.67%and 60.00% in test group higher than 31.25% and 37.50% in control group respectively in 2 week and in the end of treatment.And the rate of complete resolution of hypsarrhythmia in the test group was 46.67% and 60.00% higher than 25.00% and 37.50% in control group respectively in 2 week and in the end of treatment.But there were no statistical significances between two groups(P >0.05).The incidence of side effects(83.33% vs. 80.00%) and the relapse rate(39.14% vs. 40.00%), were not statistically significant between two groups(P >0.05).The responsive rates for the cases with the lead time within 2 months higher than beyond 2 months in two groups respectively in 2 weeks and in the end of treatment. ConclusionsThe protocol of the test group was superior to that of the control group.The responsive rates of children within 2 months of lead time were higher than beyond 2 months, which indicates that early diagnosis and early treatment would improve efficacy and have an important influence on the prognosis of IS.
ObjectiveTo analyze the clinical data of 5 cases of congenital hyperinsulinemia with sizures as the initial symptoms. MethodsRisk factors in perinatal period, clinical manifestation, laboratory examination, treatment and follow-up visits of 5 cases of congenital hyperinsulinemia with sizures as the initial symptoms were analyzed retrospectively, who were admitted to Department of Neurology of Jiangxi Children's Hospital from July 2012 to August 2016. Results5 children were all male. The onset time varied from 3 to 9 mouths old. All the cases presented seizures as the main clinical manifestations, persistent hypoglycemia, hyperinsulinemia, low free fatty acid and hypoketonemia. During follow-up, treatment with diazoxide and dietary therapy was effective in 3 cases, pure dietary therapy ineffective in 2 cases. 3 cases manifested as acute symptomic seizures, 2 cases as remote symptomic epilepsy. During follow-up, 1 cases showed normal intelligence, 3 cases developmental delay, and 1 case dropout. ConclusionMost of the infants with congenital hyperinsulinemia presented seizures as initial symptoms. Severe hypoglycemia and long duration would damage brain, and early management may significantly improve the prognosis. The early diagnosis can be made by measuring fasting blood glucose, insulin, free fatty acid and, plasma β-hydroxybutyric acid, C peptide and so on. The majority of children were effective by diazoxide.
ObjectiveTo explore the impact on the retention and effective rate of the mode of continuous guidance with wechat group in children with infantile spasms (IS) treated by ketogenic diet (KD).MethodsThe clinical data of 40 children who received KD treatment in Jiangxi Children’s Hospital from January 2017 to June 2019 were retrospectively analyzed, including 23 males and 17 females, the average age was (19.90±13.10) months and the average course was (9.95±7.61) months. They were randomly divided into control group (20 cases) and observation group (wechat group continuity Guidance Group, 20 cases), and followed up one year to compare the retention rate and efficacy.ResultsThere was no statistical difference between the general data of the observation group and the control group (P>0.05). After 9 and 12 months of KD treatment, the retention and effective rate of the observation group was significantly higher than the control group (P<0.05). And the seizure-free rate was higher than the control group (35% vs. 10%, 35% vs. 15%).ConclusionThis model of continuous guidance with wechat group can increase the KD retention rate of IS children, And increase the effective rate.