Department of Neurosurgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
Department of Neurological Surgery, University Hospitals-Cleveland Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA.
Cells. 2024 Jul 22;13(14):1231. doi: 10.3390/cells13141231.
Lower motor neuron (LMN) damage results in denervation of the associated muscle targets and is a significant yet under-appreciated component of spinal cord injury (SCI). Denervated muscle undergoes a progressive degeneration and fibro-fatty infiltration that eventually renders the muscle non-viable unless reinnervated within a limited time window. The distal nerve deprived of axons also undergoes degeneration and fibrosis making it less receptive to axons. In this review, we describe the LMN injury associated with SCI and its clinical consequences. The process of degeneration of the muscle and nerve is broken down into the primary components of the neuromuscular circuit and reviewed, including the nerve and Schwann cells, the neuromuscular junction, and the muscle. Finally, we discuss three promising strategies to reverse denervation atrophy. These include providing surrogate axons from local sources; introducing stem cell-derived spinal motor neurons into the nerve to provide the missing axons; and finally, instituting a training program of high-energy electrical stimulation to directly rehabilitate these muscles. Successful interventions for denervation atrophy would significantly expand reconstructive options for cervical SCI and could be transformative for the predominantly LMN injuries of the conus medullaris and cauda equina.
运动神经元(LMN)损伤会导致相关肌肉靶的去神经支配,这是脊髓损伤(SCI)的一个重要但未被充分认识的组成部分。去神经支配的肌肉会发生进行性退化和纤维脂肪浸润,如果不在有限的时间窗口内重新支配,最终会导致肌肉失活。失去轴突的远端神经也会发生退化和纤维化,使其对轴突的接受能力降低。在这篇综述中,我们描述了与 SCI 相关的 LMN 损伤及其临床后果。肌肉和神经退化的过程被分解为运动神经元回路的主要组成部分进行综述,包括神经和施万细胞、神经肌肉接头和肌肉。最后,我们讨论了三种有前途的逆转去神经萎缩的策略。这些策略包括提供来自局部来源的替代轴突;将干细胞衍生的脊髓运动神经元引入神经以提供缺失的轴突;最后,采用高能电刺激的训练方案直接对这些肌肉进行康复。成功干预去神经萎缩将显著扩大颈 SCI 的重建选择,并可能对圆锥和马尾的主要 LMN 损伤产生变革性影响。