Altered activation of the tibialis anterior in individuals with Pompe disease: Implications for motor unit dysfunction
Altered activation of the tibialis anterior in individuals with Pompe disease: Implications for motor unit dysfunction
复制标题
DOI：
10.1002/mus.24444
复制
发表时间：
2015-06-01
期刊：
影响因子：
3.4
通讯作者：
Christou, Evangelos A.
中科院分区：
文献类型：
--
作者：
Corti, Manuela;Smith, Barbara K.;Christou, Evangelos A.
庞贝氏症是一种进行性的骨骼肌疾病，可导致肌肉萎缩。我们探讨了激活的胫骨前肌（TA）在晚发性庞贝氏症（LOPD）的个人在最大自主收缩（MVC）和诱发的不自主respons.MethodsFour LOPD患者和匹配的对照组进行MVC的TA背屈和TA诱发的反应。激活的TA记录与表面electromyography.ResultsThe庞贝氏症患者表现出更大的功率在频率低于60 Hz和降低功率高于100 Hz。他们还表现出减少增加M-波和延长M-波的潜伏期和持续时间在响应stimulation.ConclusionsThese结果提供的证据表明，LOPD个人有一个改变激活模式的TA在最大收缩。所观察到的激活模式可能反映了自主命令、神经肌肉接头病理或由于功能性运动神经元数量减少而引起的代偿驱动的损伤。肌肉神经51：877-883，2015
IntroductionPompe disease is a progressive disease that affects skeletal muscles and leads to loss of ambulation. We investigated the activation of the tibialis anterior (TA) in late-onset Pompe disease (LOPD) individuals during maximal voluntary contraction (MVC) and evoked involuntary responses.MethodsFour LOPD patients and matched control subjects performed MVC of the TA using dorsiflexion and TA evoked responses. Activation of the TA was recorded with surface electromyography.ResultsThe Pompe patients exhibited greater power at frequencies below 60 Hz and reduced power above 100 Hz. They also exhibited a reduced increase in M-wave and prolonged M-wave latency and duration in response to stimulation.ConclusionsThese results provide evidence that LOPD individuals have an altered activation pattern of the TA during maximal contractions. The observed activation pattern may reflect impairments in voluntary command, neuromuscular junction pathology, or compensatory drive due to a reduced number of functional motoneurons. Muscle Nerve 51: 877-883, 2015