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脊髓性肌萎缩症患者的运动模式表明感觉和皮质脊髓对运动肌肉协同发展的贡献。

Motor patterns of patients with spinal muscular atrophy suggestive of sensory and corticospinal contributions to the development of locomotor muscle synergies.

机构信息

School of Biomedical Sciences, and Gerald Choa Neuroscience Institute, The Chinese University of Hong Kong, Hong Kong, China.

Joint Laboratory of Bioresources and Molecular Research of Common Diseases, The Chinese University of Hong Kong and Kunming Institute of Zoology of the Chinese Academy of Sciences, Hong Kong, China.

出版信息

J Neurophysiol. 2024 Feb 1;131(2):338-359. doi: 10.1152/jn.00513.2022. Epub 2024 Jan 17.

Abstract

Complex locomotor patterns are generated by combination of muscle synergies. How genetic processes, early sensorimotor experiences, and the developmental dynamics of neuronal circuits contribute to the expression of muscle synergies remains elusive. We shed light on the factors that influence development of muscle synergies by studying subjects with spinal muscular atrophy (SMA, types II/IIIa), a disorder associated with degeneration and deafferentation of motoneurons and possibly motor cortical and cerebellar abnormalities, from which the afflicted would have atypical sensorimotor histories around typical walking onset. Muscle synergies of children with SMA were identified from electromyographic signals recorded during active-assisted leg motions or walking, and compared with those of age-matched controls. We found that the earlier the SMA onset age, the more different the SMA synergies were from the normative. These alterations could not just be explained by the different degrees of uneven motoneuronal losses across muscles. The SMA-specific synergies had activations in muscles from multiple limb compartments, a finding reminiscent of the neonatal synergies of typically developing infants. Overall, while the synergies shared between SMA and control subjects may reflect components of a core modular infrastructure determined early in life, the SMA-specific synergies may be developmentally immature synergies that arise from inadequate activity-dependent interneuronal sculpting due to abnormal sensorimotor experience and other factors. Other mechanisms including SMA-induced intraspinal changes and altered cortical-spinal interactions may also contribute to synergy changes. Our interpretation highlights the roles of the sensory and descending systems to the typical and abnormal development of locomotor modules. This is likely the first report of locomotor muscle synergies of children with spinal muscular atrophy (SMA), a subject group with atypical developmental sensorimotor experience. We found that the earlier the SMA onset age, the more the subjects' synergies deviated from those of age-matched controls. This result suggests contributions of the sensory/corticospinal activities to the typical expression of locomotor modules, and how their disruptions during a critical period of development may lead to abnormal motor modules.

摘要

复杂的运动模式是通过肌肉协同作用的组合产生的。遗传过程、早期感觉运动经验以及神经元回路的发育动态如何促进肌肉协同作用的表达仍然难以捉摸。我们通过研究患有脊髓性肌萎缩症 (SMA,II/IIIa 型) 的受试者,揭示了影响肌肉协同作用发展的因素,这种疾病与运动神经元的退化和去传入以及可能的运动皮层和小脑异常有关,受影响者在典型的行走开始前后会有异常的感觉运动史。从患有 SMA 的儿童在主动辅助腿部运动或行走过程中记录的肌电图信号中识别出肌肉协同作用,并将其与年龄匹配的对照组进行比较。我们发现,SMA 的发病年龄越早,SMA 协同作用与正常情况的差异就越大。这些变化不能仅仅用肌肉中不同程度的不均匀运动神经元丧失来解释。SMA 特有的协同作用在来自多个肢体隔室的肌肉中具有激活作用,这一发现让人联想到典型发育中婴儿的新生儿协同作用。总的来说,虽然 SMA 和对照组之间共享的协同作用可能反映了生命早期确定的核心模块化结构的组成部分,但 SMA 特有的协同作用可能是由于异常感觉运动经验和其他因素导致的活动依赖性中间神经元塑造不足而发育不成熟的协同作用。其他机制,包括 SMA 引起的脊髓内变化和皮质脊髓相互作用的改变,也可能导致协同作用的改变。我们的解释强调了感觉和下行系统对运动模块的典型和异常发育的作用。这可能是第一篇关于患有脊髓性肌萎缩症 (SMA) 的儿童运动肌肉协同作用的报告,这是一个具有异常发育感觉运动经验的受试者群体。我们发现,SMA 的发病年龄越早,受试者的协同作用与年龄匹配的对照组的差异就越大。这一结果表明,感觉/皮质脊髓活动对运动模块的典型表达有贡献,以及它们在发育关键期的中断如何导致异常的运动模块。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c5c/11321722/d3c2a440b6ae/jn-00513-2022r01.jpg

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