Research Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Shandong University, Jinan, 250012, China.
Brain Science Research Institute, Qilu Hospital, Shandong University, Jinan, 250012, China.
Neurol Sci. 2019 Dec;40(12):2609-2615. doi: 10.1007/s10072-019-04027-7. Epub 2019 Aug 3.
To study compound muscle action potential (CMAP) decrement by low-frequency repetitive nerve stimulation (RNS) in different hand muscles of amyotrophic lateral sclerosis (ALS) patients and the relationship with split hand phenomenon and clinical manifestation.
Clinical and decrement data of 51 ALS patients who had done RNS in different hand muscles were retrospectively reviewed from November 2016 to July 2017. Decrement data of 24 myasthenia gravis (MG) and 20 Lambert Eaton myasthenia syndrome (LEMS) patients was also reviewed to compare decrement pattern with hand muscles of ALS patients.
There was statistical significance between the decrement ratio of abductor digiti minimi (ADM) and abductor pollicis brevis (APB) as well as ADM and first dorsal interosseous (FDI). The decrements of the APB, ADM, and FDI were negatively correlated with their amplitude of CMAPs respectively. The difference between the decrement ratio of the APB and ADM was negatively correlated with the division ratio (CMAP/CMAP). The decrement ratio of APB and FDI was negatively correlated with their muscle strength. There was a mild correlation between decrement ratio of APB and disease course. There was no statistical significance in the decrement pattern of the three-hand muscles of ALS patients. There was statistical significance in decrement pattern between APB of ALS and LEMS patients.
Dysfunction of neuromuscular transmission was found in hand muscles of ALS patients, APB was involved most significantly. The dysfunction of neuromuscular transmission might be involved in the formation of the split hand phenomenon.
研究肌萎缩侧索硬化(ALS)患者不同手部肌肉低频重复神经刺激(RNS)时复合肌肉动作电位(CMAP)的递减情况及其与手部分裂现象和临床表现的关系。
回顾性分析 2016 年 11 月至 2017 年 7 月间 51 例接受不同手部肌肉 RNS 的 ALS 患者的临床和递减数据,并回顾 24 例重症肌无力(MG)和 20 例 Lambert Eaton 肌无力综合征(LEMS)患者的递减数据,以比较与 ALS 患者手部肌肉的递减模式。
示指展肌(ADM)和拇指短展肌(APB)以及 ADM 和第一背侧骨间肌(FDI)的递减率之间存在统计学差异。APB、ADM 和 FDI 的递减与它们的 CMAP 振幅呈负相关。APB 和 ADM 的递减率差值与分割比(CMAP/CMAP)呈负相关。APB 和 FDI 的递减率与肌肉力量呈负相关。APB 的递减率与病程之间存在轻度相关性。ALS 患者的三组手部肌肉的递减模式无统计学意义。ALS 患者的 APB 与 LEMS 患者的递减模式存在统计学意义。
ALS 患者手部肌肉存在神经肌肉传递功能障碍,以 APB 最明显。神经肌肉传递功能障碍可能参与了手部分裂现象的形成。