| Objective:To summarize the clinical characteristics,EEG,neuroi maging examination and therapeutic regimen of infantile spasms,and analyze the influence factors of outcomes,guiding clinical treatment a nd providing some help and guidance for clinicians.Methods:The clinical data including sex,age of onset,course o f disease,personal history,family history,pattern of seizures,psycho motor development,EEG,neuroimaging examination,etiology and tre atment of 121 children diagnosed with infantile spasms in Children's Hospital of Chongqing Medical University from January 2015 to Dec ember 2016 were analyzed retrospectively.Analyzed the influence fact ors of long-term seizure control and neuropsychological development of a part of patients followed-up through specialist clinic and telepho ne.Results:1.A total of 121 cases with infantile spasms.The sex ratio of male:female was 1.42:1.The age of onset of infantile spas ms varied from 2 days to 1 year 9 months,with a peak age ranged from 3 to 7 months(58.68%),and the median age was 4 months.Th e course of spasms lasted from 1 day to 2 year,and the median cou rse was 1.7 months.10.74%cases had family history of seizure or e pilepsy.60 cases(49.59%)had neuropsychological development retard ation at early stage of onset,and 96 cases(79.34%)had neuropsycho logical development retardation at presentation.2.The clinical seizure patterns of infantile spasms including mainly flexor(70.25%),extens or(8.26%)and mixed extensor-flexor spasm(13.22%).Another 8.26%c ouldn't fit into these three categories.3.EEGs of all cases were abn ormal,including hypsarrhythmia(56.20%),modified hyssarrhythmia(27.27%)and other interictal patterns(16.53%).4.51 cases had abnormal personal history,including intrapartum asphyxia/hypoxia,etc.5.All accepted MRI,and abnormal findings were found in 85(70.25%)case s,including callosal agenesis(5),gray matter heterotopia(6),etc.6.A ccording to related guideline,the underlying causes were determined i n 89(73.55%)patients,and 32(26.45%)cases were cryptogenic.In th e symptomatic group,the major underlying causes were prenatal facto rs,and congenital encephalodysplasia came first,and intrapartum asph yxia/hypoxia second.7.Efficacy and prognosis:7.1.Recent efficacy:Among 47 patients newly diagnosed and treated with corticosteroids s tandardly,34(74.47%)became spasm-free.In the cryptogenic group,88.89%(8/9)had recent spasm-free,and in the symptomatic group,71.05%(27/38)became spasm-free.But the difference was not statisticall y significant(?~2=0.460,P=0.498).The recent efficacy in this sample only was correlated with age of onset(those with age of onset>3months would get better efficacy)(?~2=7.411,P=0.006).7.2.Long-ter m prognosis:78 cases were followed up through specialist clinic and telephone,in which 6(7.69%)died.Among 72 survived,30(41.67%)free of seizure,and 42(58.33%)still suffered from spasm or other f orms of seizure.In the cryptogenic group,58.33%(14/24)had seizure-free,and in the symptomatic group,33.33%(16/48)had seizure-free.The difference was statistically significant(?~2=4.114,P=0.043).Besid es,in the group with normal neuropsychological development at the early stage of onset,60.71%(15/26)had seizure-free,and in the abno rmal group,36.36%(15/46)had seizure-free.The difference was statist ically significant(?~2=4.300,P=0.038),suggesting that cases with und erlying causes had a poor prognosis.8.Long-term neuropsychological development:Retardation was present in all the 72 survived cases f ollowed up:mild in 22(30.56%),moderate-severe in 50(69.44%).In the cryptogenic group moderate-severe in 45.84%(11/24),and in the symptomatic group moderate-severe in 81.25%(39/48).The difference was statistically significant(?~2=9.458,P=0.002),suggesting that the cryptogenic patients would acquire better prognosis of neuropsycholo gical development.Besides,in the long-term seizure-free group,mild in 56.67%(17/30),and in the seizure-uncontrol group,mild in 11.90%(5/42).The difference was statistically significant(?~2=16.524,P=0.000).Conclusion:1.IS mostly onset during the first year of life,wit h a peak age of 3 to 7 months.Seizure forms based mainly on flex or type.The percentage of abnormal EEG was high,based mainly on hypsarrhythmia.2.The earlier age of onset was,the more atypical t he clinical manifestation was.For these infants,VEEG should be per fected as soon as possible.MRI imaging examination was help for et iological diagnosis with a positive rate up to 70.25%.So MRI was r ecommended to all children with IS.3.Symptomatic cases were mor e than cryptogenic cases.Perinatal factors were the main causes for s ymptomatic IS.The commonest cause was abnormal brain developme nt,and intrapartum asphyxia/hypoxia second.4.The recent efficacy o f corticosteroids was curative,with few side effects.5.The seizure c ontrol and neuropsychological development of infantile spasms of cry ptogenic group were all better than those of symptomatic group,sugg esting that cases with underlying causes had a poor prognosis. |
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