| Objective:To summarize and analyze possible influencing factors of poor prognosis of infantile spasms,select out those clinical features related to poor prognosis and extract predictors of neuropsychiatric developmental disorders in infantile spasms.Methods:The clinical data(including gender,age,family history,personal history,seizure pattern,neuropsychiatric development and treatment),electroencephalogram(EEG)and imaging examination of 141 patients diagnosed infant spasms in Shenzhen Children’hospital during Sep 2018 to Dec 2020 were collected and analyzed.The long-term epileptic seizure status and EEG were followed up.Chi-square test was used to analyze the difference of control rate between groups.Results:The ratio of male to female was 1.6:1 in 141 patients.The peak age of onset was 3 to 7 months(47.5%),and30 cases(21.3%)showed mental retardation before onset.The etiology was determined in 103 cases(73.0%),among which 21 cases(14.9%)were genetic,82 cases(58.2%)were structure-metabolic and 38 cases(38.0%)were unknown.Focal cortical dysplasia(FCD)was the most common etiology.89 patients received ACTH treatment,the overall short-term remission rate after ACTH was 33%.It showed that the short-term remission rate after ACTH(41.1%)was higher in the group whose onset age was≥3months(x~2=6.138,p=0.023).The overall long-term remission rate was 42.4%,and statistical analysis showed that patients with unknown etiology(x~2=5.441,p=0.034),lead time to treatment<2 weeks(x~2=5.344,p=0.021),and normal mental development before onset(x~2=4.465,p=0.030)had higher long-term remission rate.Conclusion:Patients with unknown etiology and normal development before onset may have better long-term prognosis.Early treatment is of great significance to improve the prognosis.Objective: To analyze the changes of microstructure in gray matter and function in white matter fiber and explore the relation between brain microstructure-functional changes and neurodevelopmental impairment in Infantile Spasms(IS),also to provide imaging basis for the diagnosis,treatment and prognosis of IS.Methods: 3DMPRAGE and DTI data of all the subjects were collected.The Bailey Scales of Infant Development was performed to assess neuropsychiatric development.Based on voxelbased morphometry(VBM)and Surface-based morphometry(SBM),the whole brain gray matter volume and cortical structure of 34 IS patients and 32 healthy controls(race,gender and age were matched)were analyzed.Based on voxel-based-analysis,difference of white matter fiber function between two groups was analyzed.Correlation between the volume of gray matter in brain regions that differ between groups and clinical cognitive behavioral scores(MDI and PDI)was analyzed.Results: The scores of MDI and PDI in group IS were significantly lower than HC group(p<0.001).VBM results showed reduced gray matter volume of bilateral inferior temporal gyrus,left middle occipital gyrus,left posterior cingulate gyrus,bilateral anterior central gyrus,left putamen,right superior marginal gyrus,right middle temporal gyrus,left inferior parietal lobule,right Para hippocampal gyrus,and right amygdala in group IS(p<0.001).SBM results showed cortical thinning in the right Para hippocampal gyrus,right superior marginal gyrus,right middle occipital gyrus,left precuneus,and left middle temporal gyrus(p<0.001).DTI results showed decreased FA in bilateral corticospinal tract,left posterior cingulate(hippocampal junction),and occipital forceps(p<0.001).Conclusion: The infantile spasms patients suffered from serious impairment of cognition,motor function and overall underdevelopment.Abnormalities in the visual-auditing-language association cortex such as the middle occipital gyrus,middle temporal gyrus and inferior temporal gyrus,may be the basis of impaired cognitive function,and the abnormality of anterior central gyrus and corticospinal tract may be the basis of impaired motor function in IS patients. |
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