氟桂利嗪治疗儿童交替性偏瘫的临床疗效及影响因素

Clinical efficacy of Flunarizine in treating alternating hemiplegia in children and the influencing factors

目的 研究氟桂利嗪治疗儿童交替性偏瘫（AHC）的有效率,分析影响其疗效的相关因素.方法 收集2005年8月至2016年5月在北京大学第一医院儿科神经门诊就诊及病房住院的AHC患儿的临床资料及外周血DNA,筛查ATP1A3基因突变.随访AHC患儿口服氟桂利嗪对改善交替性偏瘫等发作性症状的临床效果.结果 共收集96例AHC患儿,随访到75例患儿口服氟桂利嗪治疗.其中男45例,女30例.随访时间1 ～11年,末次随访年龄1～21岁（中位年龄5岁）.有效者50例（66.7％,50/75例）,无效者25例（33.3％,25/75例）.50例有效者中,偏瘫发作频率降低者43例（86.0％）,发作时间缩短者28例（56.0％）,发作程度减轻者3例（6.0％）.在有效组与无效组之间进行单因素分析,结果显示起病年龄,开始治疗年龄,服药剂量,及是否携带ATP1A3基因D801N、E815K或G947R突变等因素,组间比较差异均无统计学意义（均P＞0.05）.多因素分析显示,起病年龄、治疗年龄,服药剂量,是否携带E815K、D801N或G947R突变,与氟桂利嗪治疗效果不具有相关性.结论 氟桂利嗪对于多数AHC患儿有效,可降低偏瘫发作频率,缩短偏瘫持续时间,减轻偏瘫严重程度.起病年龄,治疗年龄,服药剂量,是否携带E815K、D801N或G947R突变,均不是影响疗效的因素.

Objective To investigate the effective rate of Flunarizine in treating alternating hemiplegia during childhood （AHC） kids,and to analyze the related factors influencing efficacy.Methods The clinical data and peripheral blood DNA of AHC patients at the Outpatient and Inpatient Ward of Department of Pediatrics,Peking University First Hospital from August 2005 to May 2016 were collected,and the A TP1A3 gene mutations were screened.Clinical efficacy of oral administration of Flunarizine for improving paroxysmal symptoms such as alternating hemiplegia in AHC patients was followed up.Results A total of 96 AHC patients were collected,and among them,75 cases received oral administration of flunarizine were followed up for 1-11 years.The age of last follow-up was 1-21 years old （the median age was 5 years old）.Fifty of these 75 patients （66.7%） were improved,while 25 patients were not alleviated （33.3%）.In 50 improved patients,43 patients （86.0%） reduced the frequency of hemiplegia attacks,28 patients （56.0%） reduced the duration,and 3 patients （6.0%） alleviated the severity.Univariate analysis between the effective group and ineffective group showed that differences in age of onset,age of initial treatment,dose and carrying D801N,E815K or G947R mutation of ATP1A3 gene were not statistically significant（all P 〉0.05）.The findings by multivariate analysis indicated that age of onset,age of initial treatment,dose and carrying D801N,E815K or G947R mutation of ATP1A3 gene were not related to the efficacy of Flunarizine.Conclusion Flunarizine is effective for most AHC children,which can reduce the frequency of hemiplegia attacks,shorten the duration,and alleviate the severity of attacks.Age of onset,age of initial treatment,dose and carrying D801N,E815K or G947R mutation of gene A TP1A3 are not factors influencing efficacy.