Clinical Analysis Of 109 Patiens Of West Syndrome Treated With ACTH

Objective: By comparing the clinical features of 109 patients with West syndrome treated with long-course(4 weeks)and short-course(2 weeks)ACTH treatment,to evaluate the effectiveness and safety of two different treatments duration and to assess factors that affect the therapeutic efficacy and prognosis.Methods: The data of 109 patients with West syndrome in the first affiliated hospital of Guangxi Medical University from January 2012 to September 2018 was collected for retrospective analysis.According to the ACTH treatment course,the patients were divided into 4 weeks of long-course group(n=45)and 2 weeks of short-course group(n=64),and the clinical characteristics of each group were collected.All children were followed up for more than half a year.Statistical methods were used to compare the rate of cessation of spasms,rate of complete resolution of hypsarrhythmia,rate of electroclinical remission,the incidence of adverse effects,and improvement in psychomotor development at the end of treatment.Comparing the short-term relapse rate(1 month,6 months after treatment),long-term relapse rate(more than half a year after treatment).And to assess factors that affect the therapeutic efficacy and prognosis.Result:(1)There was no significant difference in the rate of cessation of spasms,rate of complete resolution of hypsarrhythmia,rate of electroclinical remission,and the improvement in psychomotor development between the two groups at the end of the treatment.The incidence of adverse effects in the 4 weeks group was higher than that in the 2 weeks group(P=0.033).(2)The course of disease is longer than 2 months.,the age of onset was shorter than 3 months,the initial treatment was not ACTH,the symmetry attack,and the symptomatic WS patients had poor short-term effect after ACTH treatment(P<0.05).(3)There was a positive correlation between the control of seizures and the remission of hypsarrhythmia(P=0.03,r=0.287).(4)Sixty-six patients who underwent seizure control after ACTH treatment were followed up.There was no significant difference in recurrence rates at 1month and 6 months after treatment between the two groups.Early-onset and symptomatic WS were more likely to have recurrence in the short term(P<0.05).(5)There was no significant difference in the cumulative relapse rate within 24 months after treatment and the time of seizure control in the relapsed patients between the two groups.Symptomatic WS patients were more likely to relapse in the long-term(P=0.008).(6)There was a significant positive correlation between the persistence of hypsarrhythmia and the recurrence of seizures at 6 months after treatment and at the last follow-up(r>0.5,P < 0.001).(7)The WS patients who had long course of disease,recurrent spasms,symptoms,persistent high EEG hypsarrhythmia had poor long-termimprovement in psychomotor developmen(P<0.05).Conclusions:(1)There was no significant difference in the efficacy and recurrence rate between the two groups at the end of treatment.The incidence of adverse reactions was higher in the four weeks group than the two weeks group.(2)Young age of onset,long course of disease,symptomatic WS have poor effect after ACTH treatment.(3)There was a significant correlation between the recurrence of seizures and the remission of hypsarrhythmia after ACTH treatment.