Kern Helmut, Hofer Cristian, Loefler Stefan, Zampieri Sandra, Gargiulo Paolo, Baba Alfonc, Marcante Andrea, Piccione Francesco, Pond Amber, Carraro Ugo
a Physiko- und Rheumatherapie , St. Poelten , Austria.
b Ludwig Boltzmann Institute of Electrical Stimulation and Physical Rehabilitation , Vienna , Austria.
Neurol Res. 2017 Jul;39(7):660-666. doi: 10.1080/01616412.2017.1314906. Epub 2017 Apr 13.
Long-term lower motor neuron denervation of skeletal muscle is known to result in degeneration of muscle with replacement by adipose and fibrotic tissues. However, long-term survival of a subset of skeletal myofibers also occurs.
We performed transverse and longitudinal studies of patients with spinal cord injury (SCI), patients specifically complete Conus and Cauda Equina Syndrome and also of active and sedentary seniors which included analyses of muscle biopsies from the quadriceps m.
Surprisingly, we discovered that human denervated myofibers survive years of denervation after full and irreversible disconnection from their motor neurons. We found that atrophic myofibers could be rescued by home-based Functional Electrical Stimulation (h-bFES), using purpose developed stimulators and electrodes. Although denervated myofibers quickly lose the ability to sustain high-frequency contractions, they respond to very long impulses that are able to allow for re-emergence of tetanic contractions. A description of the early muscle changes in humans are hampered by a paucity of patients suffering complete Conus and Cauda Equina Syndrome, but the cohort enrolled in the EU RISE Project has shown that even five years after SCI, severe atrophic myofibers with a peculiar cluster reorganization of myonuclei are present in human muscles and respond to h-bFES.
Human myofibers survive permanent denervation longer than generally accepted and they respond to h-bFES beyond the stage of simple atrophy. Furthermore, long-term denervation/reinnervation events occur in elderly people and are part of the mechanisms responsible for muscle aging and again h-bFES was beneficial in delaying aging decay.
已知骨骼肌的长期下运动神经元去神经支配会导致肌肉退化,被脂肪和纤维化组织取代。然而,也有一部分骨骼肌肌纤维能长期存活。
我们对脊髓损伤(SCI)患者、特别是完全性圆锥和马尾综合征患者以及活跃和久坐的老年人进行了横向和纵向研究,其中包括对股四头肌肌肉活检的分析。
令人惊讶的是,我们发现人类失神经支配的肌纤维在与运动神经元完全且不可逆地断开连接后,能在去神经状态下存活数年。我们发现,使用专门开发的刺激器和电极进行家庭功能性电刺激(h-bFES)可以挽救萎缩的肌纤维。尽管失神经支配的肌纤维很快就会失去维持高频收缩的能力,但它们对非常长的脉冲有反应,这些脉冲能够使强直收缩重新出现。由于患有完全性圆锥和马尾综合征的患者数量稀少,对人类早期肌肉变化的描述受到了阻碍,但参与欧盟RISE项目的队列研究表明,即使在脊髓损伤五年后,人类肌肉中仍存在严重萎缩的肌纤维,其肌核有特殊的簇状重组,并且对h-bFES有反应。
人类肌纤维在永久性去神经状态下存活的时间比普遍认为的要长,并且在简单萎缩阶段之后它们对h-bFES仍有反应。此外,老年人会发生长期的去神经/再神经支配事件,这是导致肌肉衰老的机制之一,h-bFES同样有助于延缓衰老衰退。