Baslo Sezin Alpaydın, Şirin Nermin Görkem, Orhan Elif Kocasoy, Baslo Mehmet Barış, Öge Ali Emre
Istanbul University, Graduate School of Health Sciences, Aziz Sancar Institute of Experimental Medicine, Deparment of Neuroscience, Electroneurophysiology Master Programme, Istanbul, Türkiye.
University of Health Sciences, Bakirkoy Prof. Dr. Mazhar Osman Training and Research Hospital for Psychiatric, Neurologic, and Neurosurgical Diseases, Department of Neurology, Istanbul, Türkiye.
Noro Psikiyatr Ars. 2024 Nov 30;61(4):296-305. doi: 10.29399/npa.28864. eCollection 2024.
The aim of the study is to determine the role of upper motor neuron (UMN) or lower motor neuron (LMN) dysfunction as the primary initiator of distal-proximal and lateral-medial gradients of muscle involvement in amyotrophic lateral sclerosis (ALS).
Concentric needle electromyography recordings were performed in deltoid, abductor digiti minimi, and first dorsal interosseous (FDI) muscles in patients with ALS and controls during slight voluntary contraction needed to activate two motor units (MU). Five motor unit potential (MUP) pairs were recorded from each muscle. Motor unit potential analyses were performed offline using Multi-MUP analysis program. Quantitative MUP parameters, MU firing rate (FR), FR variability (FRV), and mean consecutive difference (MCD) were calculated. Motor-evoked potentials and the triple stimulation technique (TST) were performed to evaluate UMN involvement.
Twenty patients with ALS along with 20 age and sex-matched healthy volunteers were enrolled. Quantitative MUP parameters compatible with denervation and reinnervation were found in patients with ALS, who also showed higher FR, FRV, and MCD values, most prominently in FDI. First dorsal interosseous FRV was lower in patients with abnormal central motor conduction time (CMCT). Firing rate and FRV were negatively correlated with CMCT, but not with TST.
Distal limb muscles, particularly FDI, revealed more prominent FR abnormalities in patients with ALS in parallel with the distal-proximal and lateral-medial gradients of the selective muscle involvement pattern which seems predominantly to be correlated with LMN dysfunction. Reduced FRV may be associated with the presence of UMN dysfunction in ALS.
本研究的目的是确定上运动神经元（UMN）或下运动神经元（LMN）功能障碍作为肌萎缩侧索硬化症（ALS）中肌肉受累的远端 - 近端和外侧 - 内侧梯度的主要启动因素所起的作用。
在ALS患者和对照组的三角肌、小指展肌和第一背侧骨间肌（FDI）中进行同心针肌电图记录，记录过程中需要轻微自主收缩以激活两个运动单位（MU）。从每块肌肉记录五对运动单位电位（MUP）。使用多MUP分析程序离线进行运动单位电位分析。计算定量MUP参数、运动单位放电频率（FR）、FR变异性（FRV）和平均连续差值（MCD）。进行运动诱发电位和三重刺激技术（TST）以评估UMN受累情况。
纳入了20例ALS患者以及20名年龄和性别匹配的健康志愿者。在ALS患者中发现了与去神经支配和再支配相符的定量MUP参数，这些患者还表现出较高的FR、FRV和MCD值，在FDI中最为明显。中央运动传导时间（CMCT）异常的患者，其第一背侧骨间肌FRV较低。放电频率和FRV与CMCT呈负相关，但与TST无关。
在ALS患者中，肢体远端肌肉，特别是FDI，显示出更明显的FR异常，这与选择性肌肉受累模式的远端 - 近端和外侧 - 内侧梯度平行，而这种模式似乎主要与LMN功能障碍相关。FRV降低可能与ALS中UMN功能障碍的存在有关。
