Stępień Agnieszka, Mazurkiewicz Łucja, Maślanko Katarzyna, Rekowski Witold, Jędrzejowska Maria
Faculty of Rehabilitation, Józef Piłsudski University of Physical Education, Marymoncka 34, 00-968, Warsaw, Poland.
2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland.
BMC Musculoskelet Disord. 2020 Nov 7;21(1):726. doi: 10.1186/s12891-020-03710-7.
Musculoskeletal disorders are often observed in patients with spinal muscular atrophy (SMA). The aim of the study was to assess passive ranges of rotation in the cervical spine, chest deformity and pelvic obliquity in SMA patients, and to compare these results to the norms obtained in the group of healthy individuals. The second aim was to review these measurements and Cobb angle values for correlations in SMA patients.
The study included 74 patients with SMA and 89 healthy individuals aged 2 to 18 years. Cervical Rotation (CR), Supine Angle of Trunk Rotation (SATR) and Pelvic Obliquity (PO) tests were carried out.
Cervical rotation ranges were significantly higher in the control group than in SMA patients (p < 0.05). Differences between cervical rotation ranges to the left and to the right were significantly larger in SMA I and SMA II groups than in healthy individuals (p = 0.000). Chest asymmetry and pelvic obliquity were bigger in SMA groups than in the control (p < 0.05). Significant correlations between cervical rotation measurements, chest deformity, pelvic obliquity and Cobb angle were found in SMA individuals, depending on the type.
The results of the study suggest that CR, SATR and PO tests may assist in the assessment of SMA patients in addition to the radiographic evaluation of the spine. Biomechanical relationships between disorders located in various skeletal structures should be taken into account in the treatment of SMA patients. Special attention should be given to assessing postural parameters in non- sitters and sitters. Treatment of patients with SMA and associated musculoskeletal disorders requires a multi-specialist approach.
脊髓性肌萎缩症（SMA）患者常出现肌肉骨骼疾病。本研究旨在评估SMA患者颈椎的被动旋转范围、胸廓畸形和骨盆倾斜情况，并将这些结果与健康个体组的标准值进行比较。第二个目的是回顾这些测量值和Cobb角值，以研究SMA患者之间的相关性。
本研究纳入了74例年龄在2至18岁的SMA患者和89名健康个体。进行了颈椎旋转（CR）、仰卧位躯干旋转角度（SATR）和骨盆倾斜（PO）测试。
对照组的颈椎旋转范围显著高于SMA患者（p < 0.05）。SMA I组和SMA II组左右颈椎旋转范围的差异明显大于健康个体（p = 0.000）。SMA组的胸廓不对称和骨盆倾斜比对照组更大（p < 0.05）。在SMA个体中，根据类型不同，颈椎旋转测量值、胸廓畸形、骨盆倾斜和Cobb角之间存在显著相关性。
研究结果表明，除了对脊柱进行影像学评估外，CR、SATR和PO测试可能有助于评估SMA患者。在治疗SMA患者时，应考虑不同骨骼结构疾病之间的生物力学关系。对于不能独坐和能独坐的患者，应特别注意评估姿势参数。SMA及相关肌肉骨骼疾病患者的治疗需要多专科方法。
