NEUROMUSCULAR-TRANSMISSION IN AMYOTROPHIC-LATERAL-SCLEROSIS
NEUROMUSCULAR-TRANSMISSION IN AMYOTROPHIC-LATERAL-SCLEROSIS
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DOI：
10.1002/mus.880161109
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发表时间：
1993-11-01
期刊：
影响因子：
3.4
通讯作者：
ROOS, RP
中科院分区：
文献类型：
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作者：
MASELLI, RA;WOLLMAN, RL;ROOS, RP
对10例肌萎缩侧索硬化（ALS）患者肘肌活检标本进行神经肌肉接头功能和结构特征研究。细胞内记录显示微终板电位（MEPPs）的幅度下降。肌萎缩侧索硬化患者的MEPP频率变化很大，但平均MEPP频率与对照组无差异。终板电位的平均量子含量（m）、可立即释放的平均量子（n）和平均量子储存（N）均降低。相反，在大多数ALS终板中，量子释放的平均概率（p）是正常的，量子储存释放的平均概率（P）令人惊讶地高。在所有ALS患者中观察到去神经支配和小或缺失神经末梢的组织学证据。这些神经肌肉接头的功能和结构异常可以解释肌萎缩侧索硬化症患者的疲劳性和神经肌肉传递受损的肌电图证据。(C)John Wiley & Sons，Inc.
The functional and structural characteristics of the neuromuscular junction were studied in anconeus muscle biopsies of 10 patients with amyotrophic lateral sclerosis (ALS). Intracellular recordings revealed decreased amplitudes of miniature endplate potentials (MEPPs). The MEPP frequencies were highly variable in ALS patients but the average MEPP frequency was not different from that of control patients. The mean quantal content of endplate potentials (m), the mean quanta available for immediate release (n), and the mean quantal stores (N) were all decreased. In contrast, the mean probability of quantal release (p) was normal and the mean probability of quantal store release (P) was surprisingly high at the majority of ALS endplates. Histologic evidence of denervation and small or absent nerve terminals were observed in all ALS patients. These functional and structural abnormalities of the neuromuscular junction may explain the fatigability and the electromyographic evidence of impaired neuromuscular transmission often encountered in ALS patients. (C) 1993 John Wiley & Sons, Inc.