Department of Rehabilitation, Józef Piłsudski University of Physical Education, Marymoncka Str., 00-968 Warszawa, Poland.
Department of Developmental Neurology, Poznan University of Medical Sciences, 49 Przybyszewskiego Str, 60-355 Poznan, Poland.
Int J Environ Res Public Health. 2021 Aug 30;18(17):9134. doi: 10.3390/ijerph18179134.
The purpose of this study was to investigate the functional relationships between selected ranges of motion of the neck, upper and lower limbs, the strength of the neck and trunk muscles, postural parameters, and the motor function of children with SMA1 and SMA2-27 children, aged 6 months-15 years, with genetically confirmed spinal muscular atrophy type 1 (19 children) and 2 (8 children) undergoing pharmacological treatment. All children were examined, according to the methodology, including the motor function evaluation, measurement of selected ranges of motion, assessment of postural parameters, and measurement of neck and trunk muscle strength. The functional status of 15 children was assessed with the CHOP INTEND (CHOP group) scale and of 12 children with the HFMSE (HFMSE group). The results obtained showed that, in children examined with the CHOP scale, greater limitation of flexion in the shoulder joints was observed. As the deformation of the chest increased, the functional abilities of children deteriorated. In participants examined with the CHOP group, the ranges of neck rotation decreased with the increase of the chest deformity. In the HFMSE group, the ranges of head rotation showed a strong relationship with some parameters of muscle strength and the sum of the R coefficients. Participants showed many significant relationships between the range of motion in the neck and joints of the limbs, with more significant relationships in the CHOP group. The following conclusions were made: motor skills of children with SMA depend on muscle strength, range of motion, and deformities of the spine and chest; the development of scoliosis adversely affects the motor function, ranges of motion, and muscle strength; and movement ranges are related to motor skills and strength values.
本研究旨在探讨颈部、上下肢的特定活动范围、颈部和躯干肌肉力量、姿势参数与脊髓性肌萎缩症 1 型（SMA1）和 2 型-27 型（SMA2-27）儿童运动功能之间的功能关系。对接受基因确认的脊髓性肌萎缩症 1 型（19 名儿童）和 2 型（8 名儿童）治疗的 6 个月至 15 岁儿童进行了药物治疗。所有儿童均按运动功能评估、特定活动范围测量、姿势参数评估和颈部及躯干肌肉力量测量的方法进行检查。15 名儿童的功能状态采用 CHOP INTEND（CHOP 组）量表进行评估，12 名儿童采用 HFMSE（HFMSE 组）量表进行评估。研究结果表明，在接受 CHOP 量表评估的儿童中，肩关节屈伸受限程度较大。随着胸廓变形程度的增加，儿童的功能能力恶化。在接受 CHOP 组检查的参与者中，颈部旋转范围随胸廓畸形程度的增加而减小。在 HFMSE 组中，头部旋转范围与肌肉力量和 R 系数之和的某些参数具有很强的相关性。参与者在颈部运动范围与四肢关节之间表现出许多显著的关系，而在 CHOP 组中这些关系更为显著。得出以下结论：SMA 儿童的运动技能取决于肌肉力量、活动范围和脊柱及胸廓的畸形；脊柱侧凸的发展对运动功能、活动范围和肌肉力量有不利影响；运动范围与运动技能和力量值有关。
