PDF(Pubmed)
Abstract：
Spinal muscular atrophy (SMA) is an orphan disease characterized by the progressive degeneration of spinal alpha motor neurons. In recent years, nusinersen and several other drugs have been approved for the treatment of this disease. Transcutaneous spinal cord stimulation (tSCS) modulates spinal neuronal networks, resulting in changes in locomotion and posture in patients with severe spinal cord injury and stroke. We hypothesize that tSCS can activate motor neurons that are intact and restored by medication, slow the decline in motor activity, and contribute to the development of motor skills in SMA patients. Thirty-seven children and adults with SMA types 2 and 3 participated in this study. The median duration of drug treatment was over 20 months. The application of tSCS was performed during physical therapy for 20-40 min per day for ~12 days. Outcome measures were specific SMA motor scales, goniometry of contractured joints, and forced vital capacity. Significant increases in motor function, improved respiratory function, and decreased contracture were observed in both type 2 and 3 SMA participants. The magnitude of functional changes was not associated with participant age. Further studies are needed to elucidate the reasons for the beneficial effects of spinal cord electrical stimulation on SMA.
摘要：
脊髓性肌萎缩症(SMA)是一种以脊髓α运动神经元进行性变性为特征的孤儿疾病。近年来,nusinersen和其他几种药物已被批准用于治疗这种疾病。经皮脊髓刺激（tSCS）调节脊髓神经元网络，导致严重脊髓损伤和中风患者的运动和姿势改变。我们假设tSCS可以激活运动神经元，这些运动神经元是完整的，并通过药物恢复，减缓运动活动的下降，并有助于SMA患者运动技能的发展。37名患有2型和3型SMA的儿童和成人参加了这项研究。药物治疗的中位持续时间超过20个月。在物理疗法期间进行tSCS的应用，每天20-40分钟，持续约12天。结果指标是特定的SMA运动量表，挛缩关节测角,和强制肺活量。运动功能显著增加，改善呼吸功能，在2型和3型SMA参与者中均观察到挛缩减少。功能变化的幅度与参与者年龄无关。需要进一步的研究来阐明脊髓电刺激对SMA有益作用的原因。
