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.
ObjectiveTo explore the differences in the detection of vigabatrin-associated brain abnormalities on MRI by different MRI sequences, so as to further guide the clinical understanding of VABAM and improve the appropriate imaging sequences. MethodsA total of 353 patients with infantile spasm or epileptic spasm who were admitted to the Epilepsy Center of Yuquan Hospital of Tsinghua University from January 2020 to January 2023 were retrospectively included. MRI was performed in 131 cases, including 3D T1, T2, T1- fluid-attenuated inversion recovery sequence (FLAIR) images, DWI and ADC sequences, of which 65 cases taking VGB. We aim to evaluate the detection of vigabatrin-associated brain abnormalities on MRI by different MRI sequences in these children. Results Among the 65 patients, VABAM was detected in 23 cases, the detection rate was 35.4%. The average dosage of vigabatrin was 100.73±35.54 mg/(kg·d). The positive detection rates of VABAM were 95.7% in DWI sequence, 26.1% in ADC sequence, 21.3% in FLAIR sequence, 4.3% in T2 sequence and 0 in T1 sequence. The detection rate of ADC sequence was significantly different from DWI sequence and T1 sequence, but not from T2 sequence and FLAIR group. ConclusionDWI sequence has irreplaceable advantages in the detection rate of VABAM. Therefore, for patients with infantile spasm and epileptic spasm who take vigabatrin, we should try our best to add DWI sequence scanning to improve the positive detection rate and avoid clinical symptoms, so as to avoid further brain damage.
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 understand the prognosis of infantile spasm through long-term follow-up. MethodsChildren with infantile spasm diagnosed and treated in Children’s Hospital from January 2010 to December 2015 were retrospectively analyzed. Clinical data were collected and telephone follow-up was conducted. ResultsA total of 169 cases were collected, and only 59 cases were successfully followed up, of which 13 patients were allowed to attend school. 38 patients were not allowed to go to school, 8 patients were dead. In the group that did not go to school, 17 of them could not walk, accounting for 44.74% of the non-school attendance group, 27 of them could not run, accounting for 71.05% of the non-school attendance group. There are 33 caese who could only depend on family care instead of themselves, accounting for 86.84% of the non-school group.There were 36 children who could not speak simple sentences, accounting for about 94.74%. In addition, in terms of seizures, there were 15 patients seizure-free for more than 5 years, accounting for 29.41% of the survival group. ConclusionThe long-term prognosis of infantile spasm was poor, the mortality rate was 13.56%, the school attendance rate was 25.5% in the survival group, and the self-care ability of the non-school attendance group was poor. The normal development at the onset of the disease and the early control of epilepsy without seizures are the important prognostic factors of the disease.
ObjectiveThe purpose of this study was to explore the expression of Corticotropin releasing hormone (CRH), Corticotropin releasing hormone receptor 1 (CRHR1), Protein kinase C (PKC) in epileptogenic zone of Infantile spasm (IS).MethodsCollected 17 cases of tissues of IS patients from operation and 6 cases of normal brain tissues from clinical autopsy during June 2011 to June 2014. Westen blot was used to detected the protein expression of CRH, CRHR1, PKC. PCR was used to exam the mRNA expression of CRH, CRHR1, PKC. Immunohistochemistry and fluorescenceimmuno assay were used to detect the expression of CRH, CRHR1, PKC.ResultsThe mRNA expression of CRH and CRHR1 in IS group are higher than control group, and the protein expression of CRH and CRHR1 in IS group are higher than control group. CRH are slightly expressed in the controls, medium and strong expressed in IS, CRH and NF200 both expressed in IS; CRH is negative in GFAP positive astrocyte; CRH is negative in HLA positive microglial cell. CRHR1 are slightly and medium expressed in the controls, medium and strong expressed in IS, CRHR1 and NF200 both expressed in IS; CRHR1 and GFAP are both positive in astrocyte; CRHR1 and HLA are both positive in microglial cell. PKC are slightly and medium expressed in the controls, medium and strong expressed in IS, PKC and NF200 both expressed in IS; PKC and GFAP are both positive in astrocyte; PKC and HLA are both positive in microglial cell. Spearman analysis showed positive correlation between the expression of CRH, CRHR1, PKC with epileptic spasm in IS patients, as well as positive correlation between PKC with CRHR1.ConclusionsOver expression of CRH, CRHR1, PKC with epileptic spasm in IS patients were positive related with epileptic seizure in IS patients, indicated that CRH signal pathway is related with IS pathogenesis.