神经肌肉接头电生理功能异常早于运动神经元损害的肌萎缩侧索硬化患者特点分析  

Analysis of the characteristics of patients with amyotrophic lateral sclerosis with neuromuscular junction dysfunction prior to motor neuron degeneration

作　　者：章婧虹 陈朝晖[1] 凌丽[1] 程宏梅 张莹[1] 赵佳瑞 黄旭升[1] Zhang Jinghong;Chen Zhaohui;Ling Li;Cheng Hongmei;Zhang Ying;Zhao Jiarui;Huang Xusheng(Department of Neurology,Chinese PLA General Hospital,Beijing 100853,China)

机构地区：[1]解放军总医院第一医学中心神经内科,北京100853

出　　处：《中华内科杂志》2024年第7期660-665,共6页Chinese Journal of Internal Medicine

摘　　要：目的:探讨肌萎缩侧索硬化(ALS)患者副神经低频重复神经刺激(RNS)阳性、但同侧胸锁乳突肌针极肌电图(EMG)结果正常病例的临床及电生理特点,提高对这类ALS患者疾病的认识。方法:病例系列研究。收集解放军总医院第一医学中心神经内科2016年6月至2022年8月诊治的同时进行副神经3 Hz RNS和同侧胸锁乳突肌EMG检测的612例ALS患者病例资料,以副神经3 Hz RNS(+)且具有同侧胸锁乳突肌EMG检测的267例病例作为研究对象,比较RNS(+)/EMG(-)病例组和RNS(+)/EMG(+)病例组的临床指标差异,通过构建二项分布多维统计模型定量分析主要影响因素及其影响强度。结果:初次诊断时,副神经3 Hz RNS(+)/同侧胸锁乳突肌EMG(-)97例占ALS患者的15.8%,占RNS(+)病例的36.3%。这类病例的副神经3 Hz RNS波幅递减幅度低于RNS(+)/EMG(+)病例组[-14%(-19%,-12%)比-17%(-23%,-13%),P<0.05],上肢起病比例(64.9%)和非确诊比例(28.9%)均高于RNS(+)/EMG(+)病例组(54.7%和13.5%,均P<0.05)。ALS功能量表修订版(ALSFRS-R)评分[40(37,42)分]、体重指数(BMI)[23.8(22.0,25.4)kg/m 2]和用力肺活量(FVC)[92.8%(76.6%,103.8%)]均高于RNS(+)/EMG(+)病例组(P<0.05)。多维统计模型揭示,在RNS(+)/EMG(-)病例组中,上肢起病比例与下肢起病比例的比值为1.04,上肢起病比例与球部起病比例的比值为2.02,下肢起病比例与球部起病比例的比值为1.94,非确诊比例与确诊比例的比值为1.13。较高的ALSFRS-R评分、BMI和FVC(%)对运动神经元电生理功能具有保护效应,ALSFRS-R评分和BMI指数与FVC(%)的影响强度比值分别是3.37和1.14。结论:副神经3 Hz RNS(+)/同侧胸锁乳突肌EMG(-)病例组具有较小的RNS波幅递减幅度,较高的上肢起病比例和非确诊比例。较高的ALSFRS-R评分、BMI指数和FVC(%)对运动神经元电生理功能具有保护效应,影响强度是ALSFRS-R评分>BMI指数>FVC(%)。Objective To investigate the clinical and electrophysiological characteristics of patients with amyotrophic lateral sclerosis(ALS)with positive repetitive nerve stimulation(RNS)test results on the accessory nerve and negative needle electromyography(EMG)test results on the sternocleidomastoid with the goal to enrich the knowledge of disease progression in patients with ALS.Methods The clinical data of 612 patients diagnosed with ALS at the Neurology Department of the First Medical Center,Chinese PLA General Hospital from June 2016 to August 2022 were collected.In total,267 cases had undergone EMG tests on the sternocleidomastoid following a positive 3 Hz RNS test result on the accessory nerve,who were selected as the study subjects.The differences in clinical indicators were compared between RNS(+)/EMG(-)group and RNS(+)/EMG(+)group.A binomial distribution model with multiple variables was built to quantitatively analyze the major factors and their effects.Results At the initial visit,15.8%of patients with ALS were 3 Hz RNS(+)on the accessory nerve and EMG(-)on the ipsilateral sternocleidomastoid,accounting for 36.3%of RNS(+)patients.The decremental range of the 3 Hz RNS test delivered to the accessory nerve in these patients[-14%(-19%,-12%)]was lower than that in patients with RNS(+)/EMG(+)[-17%(-23%,-13%)](P<0.05),while the ratio of upper limb onset(64.9%)and non-definite diagnosis(28.9%)were higher[54.7%and 13.5%for patients with RNS(+)/EMG(+),P<0.05].Furthermore,the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale(ALSFRS-R)score[40(37,42)],body mass index(BMI)[23.8(22.0,25.4)kg/m2]and forced vital capacity(FVC)[92.8%(76.6%,103.8%)]were higher in patients with RNS(+)/EMG(+)(P<0.05).The multivariate model suggested that,in patients with RNS(+)/EMG(-),the ratio of upper limb onset to lower limb onset was 1.04,while that of upper limb onset to bulbar onset was 2.02,and that of lower limb onset to bulbar onset was 1.94.The ratio of non-definite ALS to definite ALS was 1.13.The ALSFRS-R score,BMI,and F

关 键 词：肌萎缩侧索硬化 副神经 肌电图 

分 类 号：R744.8[医药卫生—神经病学与精神病学]