肌萎缩侧索硬化患者膈神经传导的电生理分析

Electrophysiological study of phrenic nerve conduction in patients with amyotrophic lateral sclerosis

目的通过分析肌萎缩侧索硬化(Amyotrophic Lateral Sclerosis,ALS)患者膈神经传导检测,并结合其它神经电生理资料,为该病提供更深入的认识,进一步指导临床诊疗。方法研究范围为武汉大学人民医院2014年1月-2021年12月就诊的ALS患者共88例,收集患者的一般资料、主要症状及体征、肌萎缩侧索硬化改良量表(ALSFRS-R)评分、运动神经传导检测中的复合肌肉动作电位(CMAP)波幅和远端运动潜伏期(DML)等指标。结果(1)运动神经传导检测中CMAP波幅降低192条(43.6%),膈神经波幅异常率为35.2%;远端潜伏期延长116条(26.4%),膈神经DML异常率为77.3%;(2)膈神经DML在性别方面存在明显差异(P<0.01);(3)ALSFRS-R评分与膈神经、尺神经、正中神经、腓总神经、胫神经的CMAP波幅呈正相关(r=0.393,P<0.01;r=0.375,P<0.01;r=0.413,P<0.01;r=0.251,P<0.05;r=0.442,P<0.01);(4)膈神经DML及CMAP波幅在起病部位方面存在明显差异(P<0.05;P<0.05);(5)膈神经DML在判断病情中度和轻度之间的最佳界点为9.095 ms,敏感性为85.7%,特异性为80.2%。结论ALS患者的运动神经传导可表现异常,CMAP波幅下降占比较大,但膈神经中潜伏期延长比CMAP波幅降低更多见。膈神经传导检测存在一定程度的性别差异。行运动神经传导检测时多条神经CMAP波幅变化可反映ALS患者病情严重程度。膈神经潜伏期变化可更敏感地反映ALS的病情严重程度,以期指导临床诊断与治疗。

Objective To provide a deeper understanding of amyotrophic lateral sclerosis(ALS)and further guide the clinical diagnosis and treatment by analyzing the phrenic nerve conduction and other neuroelectrophysiological data.Methods A total of 88 patients with ALS treated at Renmin Hospital of Wuhan University were included in the study from January 2014 to December 2021.The clinic data,main symptoms and signs,Amyotrophic Lateral Sclerosis Function Rating Scale(ALSFRS-R)score,the compound muscle action potential(CMAP)amplitude and distal motor latency(DML)in motor nerve conduction test were collected.Results①The CMAP amplitude decreased by 192(43.6%)in motor nerve conduction test,and the abnormal amplitude rate of phrenic nerve conduction was 35.2%.The distal latency was prolonged by 116(26.4%),and the abnormal rate of DML in phrenic nerve was 77.3%.②There was significant difference in the distribution of phrenic nerve latency in the analysis of gender differences(P<0.01).③It had positive correlation between ALSFRS-R score and the CMAP amplitude of phrenic nerve,ulnar nerve,median nerve,tibial nerve and peroneal nerve respectively(r=0.393,P<0.01;r=0.375,P<0.01;r=0.413,P<0.01;r=0.442,P<0.01;r=0.251,P<0.05).④There were significant differences between phrenic nerve latency and CMAP amplitude distribution and the onset site(P<0.05,P<0.05).⑤The best cut-off point of DML in phrenic nerve was 9.095 ms to judge the condition between moderate and mild,with sensitivity of 85.7%and specificity of 80.2%.Conclusions The motor nerve conduction of ALS patients may be abnormal.The decreased CMAP amplitude is more common,but prolonged DML in phrenic nerve is more common than decreased CMAP amplitude.There is a gender difference in phrenic nerve conduction in ALS patients.When the motor nerve conduction was detected,the changes of CMAP amplitude of multiple nerves could reflect the severity of ALS.The change of DML in the phrenic nerve can more sensitively reflect the severity of ALS in order to guide clinical diagnosis and treatment.