Research On The Recent And Long-term Outcome Of ACTH Therapy For Infantile Spasms And Their Influencing Factors

Objective:By analyzing the recent and long-term outcome of ACTH treatment ofinfantile spasms,we tried to provide evidence for improving the efficacy of the treatment ofinfantile spasms.Methods:This was a prospective,cohort study design in a single center.According to thediagnostic criteria,inclusion criteria,exclusion criteria,we established a database,collectedcases,follow-up,analyzed the cases of patients with infantile spasms from1999to2010atShenzhen Children’s Hospital,categorized patients as the ACTH first line and non-first linedrug group.We compared the recent (within8weeks) spasm-free rate and the influence toit between two groups.Analyzed the correlation between ACTH recent effects and VEEGresults;Analyzed the factors influencing ACTH recent effects in non-first line drug group,as well as long-term outcome and its related factors.Results:The final follow-up time was December,2013,we got113cases.1.33cases werein ACTH first line drug group and80cases were in ACTH non-first line group.21cases inACTH first line drug group had recent spasm-free efficacy,accounting for63.3%,33casesin ACTH non-first line drug group had the recent spasm-free efficacy,accounting for41.3%.The difference between two groups was statistically significant(chi-square=4.69,P<0.05).2.A total of113cases had hypsarrhythmia in interictal EEG before ACTHtreatment.After treatment,54cases became spasm-free,38of them had the hypsarrhythmiadisappeared,accounting for70.4%;59cases without seizure free but23of them hadhypsarrhythmia disappeared,accounting for39%.The difference between two groups wasstatistically significant(chi-square=11.18,P<0.01).3.Those80cases in group that chosedACTH as non first line drug,33cases had recent spasm-free,which accounted for41.3%.The efficacy was correlated with the age of onset and age at newly diagnosed (patientswith age of onset>6months,age at newly diagnosed>6months,would get better resultwith ACTH treatment after the failure of antiepileptic drugs),regardless of gender,pathogenesis,etiology;4.At the final point follow-up the recurrence profile: in ACTH asfirst line drug group there were21cases had spasm-free,7cases had recurrence,with therecurrence rate33.3%;In ACTH as not-first line drug group,there were33cases hadspasm-free,14cases had recurrence,with the recurrence rate42.4%.The difference between two groups was not statistically significant(chi-square=0.78,P>0.05).Long-term outcomeof seizures:33of54spasm-free cases showed no recurrence,which were spasm-free afterACTH treatment,a total of55cases showed recurrence and completely uncontrolled(7cases died,12cases of that had seizure types changed, including myoclonic seizures,tonicseizures,typical or atypical absence seizures,focal seizure and so on),15cases werecontrolled by other antiepileptic drugs after ACTH failure.5.There were32cases gainedlong-term mental development improvement,17cases returned to normal,57cases had noimprovement or regression.Psychomotor development was correlated with the age ofonset,age at newly diagnosed.Patients with age of onset>6months,age at newly diagnosed>6months,cryptogenic etiology would acquire higher recent spasm-free rate after ACTHtreatment as well as better long-term intelligence development(P<0.05), regardless ofgender and recent hypsarrhythmia disappearance after ACTH treatment(P>0.05).Conclusion:1.The infantile spasms patients treated with ACTH as first line drug couldget a higher spasm-free rate (63.6%),while others treated with ACTH after the failure of atleast one other antiepileptic drugs have a lower spasm-free rate (41.3%),which suggests itis still worth trying even if other antiepileptic drugs have failed;2.VEEG is an importanttest to evaluate the recent efficacy,“spasm-free” may not fully reflect the situation ofVEEG hypsarrhythmia disappearance circumstances;3.Patients with age of onset>6months,age at newly diagnosed>6months,would get higher spasm-free rate by ACTHtreatment after other antiepileptic drug failure;4.ACTH as the first line choice or not is notrelated with the long-term recurrence rate;5.Patients with the age of onset>6months,ageat newly diagnosed>6months,recent and long-term seizure free,cryptogenic etiologywould get better movement and intellectual development.