神经肌肉接头动态改变在肌萎缩侧索硬化中的研究价值与进展

Research advances and value of neuromuscular junction dynamic change in amyotrophic lateral sclerosis

肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是罕见病中的常见病,以选择性累及上、下运动神经元为特征。近年来多项基础研究表明,神经肌肉接头(neuromuscular junction,NMJ)解体是ALS的早期病理损伤之一,且往往早于运动神经元的变性丢失。重复神经刺激是临床上评价NMJ功能的主要电生理手段。已有多项临床研究表明ALS中可见重复神经刺激低频递减现象,且可早于自发电位的出现。因此,本文以ALS中的NMJ为焦点,对其在疾病进展中的动态改变及重复神经刺激在ALS中的临床应用价值进行综述,旨在提高读者对ALS中NMJ损伤的认识,促进以NMJ为靶点的药物研究,并提高重复神经刺激技术在ALS中的应用。

Amyotrophic lateral sclerosis(ALS)is a progressive,paralytic disorder characterized by selective degeneration of upper and lower motor neurons.Recently,more and more basic studies have shown that neuromuscular junction(NMJ)is defective in the asymptomatic stage of ALS,even earlier than the degeneration of motor neurons.Repetitive nerve stimulation(RNS)is commonly used to examine the function of NMJ.Some clinical studies have shown early dec remental responses to RNS in ALS,which might be earlier than the emergence of spontaneous potential.Here,focusing on the NMJ in ALS,we review the dynamic changes of NMJ in the progression of ALS and highlight the clinical value of RNS,with the aim of improving the understanding of NMJ defect in ALS,promoting the drug exploit,and encouraging the application of RNS in ALS.