Department of Neurology, Chinese People's Liberation Army General Hospital, Beijing 100853, China.
Chin Med J (Engl). 2018 Sep 20;131(18):2146-2151. doi: 10.4103/0366-6999.240798.
Nowadays, it is widely known that decremental responses in low-frequency repetitive nerve stimulation (LF-RNS) are frequently observed in patients with amyotrophic lateral sclerosis (ALS). The pathological mechanism of this phenomenon remains unknown. This study aimed to illuminate the features of RNS in Chinese patients with ALS.
Clinical and electrophysiological data of 146 probable and definite ALS patients who underwent RNS were retrospectively enrolled and analyzed. LF-RNS (3 Hz) was performed in trapezius, deltoid, abductor digiti minimi (ADM), quadriceps femoris, and tibialis anterior. High-frequency RNS (HF-RNS, 10 Hz) was performed only in ADM. The two-sample t-test and Chi-squared test were used for statistical analysis.
Decremental responses to LF-RNS (≥10%) in at least one muscle were detected in 83 (56.8%) of the cases and were most commonly seen in trapezius and deltoid. The incidence of decremental response was higher in patients with upper limb onset. Incremental responses to HF-RNS (≥60%) in ADM were observed in 6 (5.6%) of the cases. In 106 muscles with decremental response, 62 (57.4%) muscles had a continuous decremental pattern, more than a U-shape pattern (37 cases, 34.3%). Nineteen cases showed definite decrements in LF-RNS tests in trapezius, while no abnormalities were found in the electromyography and neurological examination of the sternocleidomastoid muscle, supplied by the accessory nerve as well.
Decremental responses in the RNS are commonly observed in ALS patients. The findings regarding the trapezius indicated that some ALS onsets could be initiated by a "dying back" process, with destruction of neuromuscular junctions (NMJs) before motor neurons. Incremental responses in the ADM implied damage of the NMJs involved both the post and presynaptic membranes.
如今，低频重复神经刺激（LF-RNS）的递减反应在肌萎缩侧索硬化症（ALS）患者中经常观察到，这一现象的病理机制尚不清楚。本研究旨在阐明中国 ALS 患者的 RNS 特征。
回顾性纳入并分析了 146 例接受 RNS 的疑似和确诊 ALS 患者的临床和电生理数据。在斜方肌、三角肌、小指展肌（ADM）、股四头肌和胫骨前肌进行 LF-RNS（3 Hz）。仅在 ADM 进行高频 RNS（HF-RNS，10 Hz）。采用两样本 t 检验和卡方检验进行统计学分析。
83 例（56.8%）至少有一块肌肉的 LF-RNS 出现递减反应（≥10%），最常见于斜方肌和三角肌。上肢起病患者的递减反应发生率较高。6 例（5.6%）ADM 出现 HF-RNS 递增反应（≥60%）。在 106 块有递减反应的肌肉中，62 块（57.4%）肌肉呈连续递减模式，超过 U 形模式（37 例，34.3%）。19 例斜方肌 LF-RNS 检查出现明显递减，而副神经支配的胸锁乳突肌肌电图和神经系统检查均未见异常。
RNS 中的递减反应在 ALS 患者中很常见。斜方肌的发现表明，一些 ALS 发作可能是由运动神经元前神经肌肉接头（NMJs）破坏的“退行性”过程引发的。ADM 的递增反应提示 NMJs 的破坏涉及突触前膜和突触后膜。
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