延髓受累时间对脊髓起病型肌萎缩侧索硬化症患者生存的影响  

Bulbar involvement time in spinal-onset amyotrophic lateral sclerosis:Effect on survival

作　　者：胡芳芳[1] 靳娇婷[1] 陈乔依 康丽[1] 贾蕊[1] 秦星[1] 刘潇 刘晨昱 张荣华 王亮[1] 王春娟 党静霞[1] HU Fangfang;JIN Jiaoting;CHEN Qiaoyi;KANG Li;JIA Rui;QIN Xing;LIU Xiao;LIU Chenyu;ZHANG Ronghua;WANG Liang;WANG Chunjuan;DANG Jingxia(Department of Neurology,The First Affiliated Hospital of Xi'an Jiaotong University,Xi'an 710061;Department of Cell Biology and Genetics,School of Basic Medical Sciences,Xi'an Jiaotong University Health Science Center,Xi'an 710061;East Hospital of The First Affiliated Hospital of Xi'an Jiaotong University,Xi'an 710087,China)

机构地区：[1]西安交通大学第一附属医院神经内科,陕西西安710061 [2]西安交通大学医学部细胞生物学与遗传学系,陕西西安710061 [3]西安交通大学第一附属医院东院,陕西西安710087

出　　处：《西安交通大学学报（医学版）》2020年第6期842-847,共6页Journal of Xi’an Jiaotong University（Medical Sciences）

基　　金：陕西省2020年重点研发计划项目(No.2020SF-098)。

摘　　要：目的探讨延髓受累时间对脊髓起病型肌萎缩侧索硬化症(amyotrophic lateral sclerosis,ALS)患者生存时间的影响。方法对2011年1月2日至2017年12月31日在我院就诊的168例脊髓起病型ALS患者随访至2018年12月31日,通过COX单因素和多因素分析ALS患者生存时间的影响因素,并通过Kaplan-Meier分析延髓受累时间对生存时间的影响。结果COX多因素分析表明,起病年龄<55岁ALS患者的死亡风险是起病年龄≥55岁ALS患者的0.72倍(P=0.09),诊断延迟时间<10.98个月的ALS患者死亡风险是诊断延迟时间≥10.98个月ALS患者的2.64倍(P<0.001),延髓受累时间≥11.5个月和延髓未受累的ALS患者死亡风险分别是延髓受累时间<11.5个月ALS患者的0.30倍和0.32倍(P<0.001)。Kaplan-Meier分析表明,ALS患者中位生存时间在延髓受累时间<11.5个月、≥11.5个月和延髓未受累3组间差异有统计学意义(中位生存时间20.37个月vs.40.6个月vs.39.60个月,统计量=39.96,P<0.001)。延髓受累时间<11.5个月的脊髓起病型ALS患者2年、3年和5年生存率分别为32.17%、10.80%和0%,延髓受累时间≥11.5个月的患者2年、3年和5年生存率分别为89.20%、57.24%和10.53%,延髓未受累的患者2年、3年和5年生存率分别为62.16%、38.39%和10.53%。结论同诊断延迟时间和是否服用利鲁唑一样,脊髓起病型ALS患者在发病后11.5个月是否出现延髓受累也是影响其生存期的独立危险因素,且延髓受累时间<11.5个月的患者中位生存时间明显短于延髓受累时间≥11.5个月患者和延髓未受累的患者。Objective To explore the effect of bulbar involvement time on survival time of patients with spinal-onset amyotrophic lateral sclerosis(ALS).Methods We followed up 168 patients with spinal-onset ALS admitted to our hospital from January 2,2011 to December 31,2017 until December 31,2018.Univariate and multivariate analyses were performed to evaluate the affecting factors of the ALS patients survival time.Kaplan-Meier analysis was made to evaluate the effects of bulbar involvement time on survival time.Results COX multivariate analysis showed that the risk of death in age-onset<55 y patients was 0.72 times that in age-onset≥55 y(P=0.09),the risk of death in diagnosis delay time<10.98 m patients was 2.64 times that≥10.98 m(P<0.001);the risk of death in bulbar involvement time≥11.5 m and bulbar uninvolvement was 0.30 and 0.32 times respectively that bulbar involvement time<11.5 m(P<0.001).Kaplan-Meier analysis showed differences among bulbar involvement time<11.5 m,≥11.5 m and bulbar uninvolvement groups(median survival time 20.37 m vs.40.6 m vs.39.60 m,Test statistic=39.96,P<0.001).The 2-year,3-year and 5-year survival rates were 32.17%,10.80%and 0%,respectively,in bulbar involvement time<11.5 m patients;89.20%,57.24%and 10.53%in bulbar involvement time≥11.5 months patients;and 62.16%,38.39%and 10.53%in bulbar uninvolvement patients.Conclusion Similar to the diagnosis delay time and whether to have taken riluzole,the occurrence of bulbar involvement at 11.5 month after onset was an independent risk factor affecting survival time in spinal-onset ALS.The median survival time in patients with bulbar involvement time<11.5 months was significantly shorter than that in patients with bulbar involvement time≥11.5 months and bulbar uninvolvement.

关 键 词：肌萎缩侧索硬化症 延髓受累 延髓受累时间 生存分析 

分 类 号：R741[医药卫生—神经病学与精神病学]