PDF(Sci-hub)
Abstract：
Therapeutic management of the upper extremity (UE) function of people with spinal muscular atrophy (SMA) requires sensitive and objective assessment. Therefore, we aimed to measure physiologic UE function of SMA patients with different functional abilities and evaluate the relation between these physiologic measures and functional UE scales.
12 male and 5 female SMA patients (mean age 42 years; range 6-62 years) participated in this explorative study. Concerning the physiologic level, the maximal muscle torque, the maximal and normalized surface electromyography (sEMG) amplitudes, and the maximal passive and active joint angles were measured. Concerning the activity level, the Performance of the Upper Limb (PUL) scale was used, and hand function was examined using the Nine-Hole Peg Test and the Timed Test of In-Hand Manipulation (TIHM).
Outcome measures that significantly related to the functional ability were: the PUL score (all dimensions); the finger to palm task of the Timed TIHM; biceps, triceps, and forearm extensor strength; and the active range of motion of shoulder abduction, shoulder flexion, and wrist extension. In addition, the following physiologic variables were related to the activity level (PUL score): hand function (the Nine-Hole Peg Test; Rs = - 0.61), the Timed TIHM (Rs = - 0.53), the maximal muscle torque (Rs = 0.74), the maximal sEMG amplitude (Rs = 0.79), and the maximal active joint angle (Rs = 0.88).
Muscle functions in SMA patients are already affected before activity limitations are noticeable. Consequently, monitoring the maximal muscle strength and the normalized muscle activity during task performance could play a role in the early detection of UE limitations. The mechanism behind the loss of arm activities due to SMA is primarily caused by decreasing muscle capacity, which influences the ability to move an arm actively. In clinical practices, these dimensions should be considered separately when monitoring disease progression in order to better evaluate the need for interventions.
12 male and 5 female SMA patients (mean age 42 years; range 6-62 years) participated in this explorative study. Concerning the physiologic level, the maximal muscle torque, the maximal and normalized surface electromyography (sEMG) amplitudes, and the maximal passive and active joint angles were measured. Concerning the activity level, the Performance of the Upper Limb (PUL) scale was used, and hand function was examined using the Nine-Hole Peg Test and the Timed Test of In-Hand Manipulation (TIHM).
Outcome measures that significantly related to the functional ability were: the PUL score (all dimensions); the finger to palm task of the Timed TIHM; biceps, triceps, and forearm extensor strength; and the active range of motion of shoulder abduction, shoulder flexion, and wrist extension. In addition, the following physiologic variables were related to the activity level (PUL score): hand function (the Nine-Hole Peg Test; Rs = - 0.61), the Timed TIHM (Rs = - 0.53), the maximal muscle torque (Rs = 0.74), the maximal sEMG amplitude (Rs = 0.79), and the maximal active joint angle (Rs = 0.88).
Muscle functions in SMA patients are already affected before activity limitations are noticeable. Consequently, monitoring the maximal muscle strength and the normalized muscle activity during task performance could play a role in the early detection of UE limitations. The mechanism behind the loss of arm activities due to SMA is primarily caused by decreasing muscle capacity, which influences the ability to move an arm actively. In clinical practices, these dimensions should be considered separately when monitoring disease progression in order to better evaluate the need for interventions.
摘要：
脊髓性肌萎缩症（SMA）患者的上肢（UE）功能的治疗管理需要敏感而客观的评估。因此,我们旨在测量具有不同功能能力的SMA患者的生理UE功能，并评估这些生理指标与功能UE量表之间的关系。
12名男性和5名女性SMA患者（平均年龄42岁；范围6-62岁）参加了这项探索性研究。关于生理水平，最大肌肉扭矩，最大和归一化表面肌电图(sEMG)振幅，并测量了最大被动和主动关节角度。关于活动水平，使用上肢性能(PUL)量表，并使用九孔钉测试和手操作定时测试（TIHM）检查手功能。
与功能能力显着相关的结果指标是：PUL评分（所有维度）；定时TIHM的手指到手掌任务；二头肌，三头肌,和前臂伸肌力量；以及肩关节外展的活动范围，肩部屈曲,和手腕延伸。此外,以下生理变量与活动水平（PUL评分）相关：手功能（九孔钉测试；Rs=-0.61），定时TIHM(Rs=-0.53)，最大肌肉扭矩（Rs=0.74），最大sEMG振幅（Rs=0.79），和最大活动关节角(Rs=0.88)。
在活动限制明显之前，SMA患者的肌肉功能已经受到影响。因此,在任务执行期间监测最大肌肉力量和标准化肌肉活动可能在UE限制的早期检测中发挥作用。由于SMA而导致手臂活动丧失的机制主要是由肌肉容量降低引起的，这影响了主动移动手臂的能力。在临床实践中，在监测疾病进展时,应分别考虑这些维度,以便更好地评估干预措施的必要性.
12名男性和5名女性SMA患者（平均年龄42岁；范围6-62岁）参加了这项探索性研究。关于生理水平，最大肌肉扭矩，最大和归一化表面肌电图(sEMG)振幅，并测量了最大被动和主动关节角度。关于活动水平，使用上肢性能(PUL)量表，并使用九孔钉测试和手操作定时测试（TIHM）检查手功能。
与功能能力显着相关的结果指标是：PUL评分（所有维度）；定时TIHM的手指到手掌任务；二头肌，三头肌,和前臂伸肌力量；以及肩关节外展的活动范围，肩部屈曲,和手腕延伸。此外,以下生理变量与活动水平（PUL评分）相关：手功能（九孔钉测试；Rs=-0.61），定时TIHM(Rs=-0.53)，最大肌肉扭矩（Rs=0.74），最大sEMG振幅（Rs=0.79），和最大活动关节角(Rs=0.88)。
在活动限制明显之前，SMA患者的肌肉功能已经受到影响。因此,在任务执行期间监测最大肌肉力量和标准化肌肉活动可能在UE限制的早期检测中发挥作用。由于SMA而导致手臂活动丧失的机制主要是由肌肉容量降低引起的，这影响了主动移动手臂的能力。在临床实践中，在监测疾病进展时,应分别考虑这些维度,以便更好地评估干预措施的必要性.
