Ultra-early amplitude decrement after repetitive nerve stimulation supports early neuromuscular junction injury in amyotrophic lateral sclerosis:a prospective cross-sectional study  

作　　者：Jing-Yue Ma Xiang-Yi Liu Shuo Zhang Dong-Sheng Fan 

机构地区：[1]Department of Neurology,Peking University Third Hospital,Beijing,China [2]Beijing Municipal Key Laboratory of Biomarker and Translational Research in Neurodegenerative Diseases,Beijing,China

出　　处：《Neural Regeneration Research》2022年第3期655-660,共6页中国神经再生研究（英文版）

摘　　要：The dying-back hypothesis holds that the damage to neuromuscular junctions and distal axons in amyotrophic lateral sclerosis occurs at the earliest stage of the disease.Previous basic studies have confirmed early damage to neuromuscular junctions,but it is difficult to obtain such evidence directly in clinical practice.In this prospective cross-sectional study,we recruited 22 patients with early amyotrophic lateral sclerosis with disease duration < 12 months and with clinical symptoms limited to the upper limbs.We also recruited 32 healthy controls.Repetitive nerve stimulation was performed,and patients were followed for 12 months.We found a significant change in the response to repetitive nerve stimulation in amyotrophic lateral sclerosis patients without spontaneous electromyographic activity.Patients that were prone to denervation had an increased decrement response of target muscles after repetitive nerve stimulation.These results suggest that changes in response to repetitive nerve stimulation may occur before denervation in amyotrophic lateral sclerosis patients.The damage to lower motor neurons is more obvious in patients with a higher percentage of repetitive never stimulation-related amplitude decrements.This study was approved by the Institutional Ethics Committee of Peking University Third Hospital(approval No.M2017198) on August 24,2017.

关 键 词：amplitude decremental response amyotrophic lateral sclerosis dying-back hypothesis motor neuron disease nerve electrophysiology PHYSIOPATHOLOGY prognosis repetitive nerve stimulation 

分 类 号：R444[医药卫生—诊断学] R741[医药卫生—临床医学]