上肢起病的经典型肌萎缩侧索硬化症与连枷臂综合征的对比分析  

Comparison between upper limb onset classic amyotrophic lateral sclerosis and flail-arm syndrome

作　　者：康丽[1] 靳娇婷[1] 胡芳芳[1] 秦星[1] 贾蕊[1] 刘潇 张荣华 党静霞[1] KANG Li;JIN Jiaoting;HU Fangfang;QIN Xing;JIA Rui;LIU Xiao;ZHANG Ronghua;DANG Jingxia(Department of Neurology,The First Affiliated Hospital of Xi'an Jiaotong University,Xi’an 710061,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

出　　处：《西安交通大学学报（医学版）》2020年第6期848-853,共6页Journal of Xi’an Jiaotong University（Medical Sciences）

摘　　要：目的探讨上肢起病的经典型肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)与ALS变异型连枷臂综合征(flail-arm syndrome,FAS)的临床及电生理特点,为鉴别诊断及判断预后提供参考。方法收集2013-2018年就诊于西安交通大学第一附属医院神经内科的120例上肢起病的经典型ALS和18例FAS患者,比较2组患者的临床、电生理特点及生存情况。结果与上肢起病的经典型ALS相比,FAS患者诊断级别低(P<0.001),诊断延迟时间长(15月vs.11月;Z=-2.749,P=0.005),进展率低(0.33 vs.0.64;Z=-3.055,P=0.002),进展到第二体区时间长(33月vs.8月;Z=-4.852,P<0.001)。FAS患者虽然与上肢起病的经典型ALS患者同样多为单上肢起病,但FAS组中双上肢起病者比例高于上肢起病的经典型ALS组(33.3%vs.8.3%;χ^2=7.261,P=0.007),FAS患者中近端症状重的比例更高(55.6%vs.18.3%;χ^2=8.856,P=0.003)。FAS患者中存在上肢反射高或病理阳性的比例低(11.1%vs.72.5%;χ^2=25.759,P<0.001)。FAS患者在拇短展肌和小指展肌上记录的复合肌肉动作电位(compound muscle action potential,CMAP)比较显示,CMAP拇短展肌/CMAP小指展肌波幅比值较上肢起病的经典型ALS稍高,但两组间差异无统计学意义;且FAS患者其他体区针电极肌电图异常与上肢起病的经典型ALS间差异无统计学意义。FAS患者的生存时间较上肢起病的经典型ALS长(53月vs.22月;Z=-4.421,P<0.001);与诊断为上肢起病的经典型ALS相比,诊断为FAS是死亡风险较低的独立预测因子(HR=0.174,95%CI:0.061~0.496,P=0.001)。结论FAS和上肢起病的经典型ALS的自然病程不同,但肌电图表现类似;早期以双上肢起病、近端症状重于远端、上肢以下运动神经元损害体征为著者,对FAS诊断有一定的提示作用。Objective To investigate the clinical and electrophysiological characteristics of upper limb onset classic amyotrophic lateral sclerosis(ALS)and ALS variant flail-arm syndrome(FAS)so as to provide reference for differential diagnosis and prognosis.Methods We recruited 120 upper limb onset classic ALS and 18 FAS patients from Neurology Department of The First Affiliated Hospital of Xi'an Jiaotong University between January 2013 and December 2018.The clinical and electrophysiological characteristics and survival were compared between the two groups.Results Compared with those in upper limb onset classic ALS,FAS patients diagnostic level was lower(P<0.001),diagnostic delay time was longer(15 mon vs.11 mon;Z=-2.749,P=0.005),progression rate was slower(0.33 vs.0.64;Z=-3.055,P=0.002),and time from the first region to the second was longer(33 mon vs.8 mon;Z=-4.852,P<0.001).FAS patients were as likely to have single lateral upper limb onset as those with upper limb onset classic ALS,but the proportion of patients with bilateral upper limbs onset in FAS group was higher than that in the upper limb onset classic ALS group(33.3%vs.8.3%;χ^2=7.261,P=0.007),the proportion of proximal upper limb being more severely affected was higher in FAS group(55.6%vs.18.3%;χ^2=8.856,P=0.003).The proportion of FAS patients with high upper limb reflex or pathologic sign was lower(11.1%vs.72.5%;χ^2=25.759,P<0.001).Compared with upper limb onset classic ALS,FAS group s ratio of compound muscle action potential(CMAP)abductor pollicis brevis/CMAP abductor digiti minimi was higher,but without statistical significance;needle electrode electromyographic results were similar between the two groups.Survival time of FAS patients was longer than that of patients with upper limb onset classic ALS(53 mon vs.22 mon;Z=-4.421,P<0.001),and FAS diagnosis itself was an independent prognostic factor of lower death risk compared with the diagnosis of upper limb onset classic ALS(HR=0.174,95%CI:0.061-0.496,P=0.001).Conclusion Although natural histories of FAS

关 键 词：肌萎缩侧索硬化症 连枷臂综合征 临床特点 电生理特点 生存分析 

分 类 号：R741[医药卫生—神经病学与精神病学]