Progression of cortical and spinal dysfunctions over time in amyotrophic lateral sclerosis
Progression of cortical and spinal dysfunctions over time in amyotrophic lateral sclerosis
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DOI：
10.1002/mus.20942
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发表时间：
2008-03-01
期刊：
影响因子：
3.4
通讯作者：
Schmied, Annie
中科院分区：
文献类型：
--
作者：
Attarian, Shahram;Vedel, Jean-Pierre;Schmied, Annie
鉴于肌萎缩侧索硬化症（ALS）中下运动神经元和上运动神经元（LMN，UMN）功能障碍之间的联系存在相互矛盾的结果，我们进行了这项研究，以关联它们随时间的变化。单运动单位（MU）的特点是他们的宏MU电位，抽搐幅度，和兴奋性反应，经颅磁刺激（TMS）。对10名ALS患者进行了2至4次研究，并将其数据细分为对应于平均疾病持续时间12（58 MU）、20（60 MU）、32（50 MU）、43（40 MU）和168个月（55 MU）的时期。随着时间的延长，MU的大小增加，收缩效率和兴奋性反应率显著降低。产生正常兴奋性反应的MU的收缩有效性随时间的推移而下降，而兴奋性反应的逐渐丧失与正常的机电性能的MU之间观察到，由于没有相关性之间发现UMN和LMN功能障碍，我们得出结论，UMN和LMN可能退化独立的ALS。
In view of the conflicting results about the links between lower and upper motor neuron (LMN, UMN) dysfunction in amyotrophic lateral sclerosis (ALS), we undertook this study to correlate their changes over time. Single motor units (MUs) were characterized by their macro-MU potentials, twitch amplitude, and excitatory responses to transcranial magnetic stimulation (TMS). Ten ALS patients were studied 2 to 4 times and their data were subdivided into epochs corresponding to mean disease duration of 12 (58 MUs), 20 (60 MUs), 32 (50 MUs), 43 (40 MUs), and 168 months (55 MUs). The MU size increased and the contractile effectiveness and the excitatory response rates decreased significantly with time. The contractile effectiveness of MUs producing normal excitatory responses decreased with time, whereas a gradual loss of excitatory responses was observed among MUs with normal electromechanical properties, Since no correlation was found between UMN and LMN dysfunction, we conclude that UMN and LMN probably degenerate independently in ALS.