Favata Alessandra, Gallart-Agut Roger, van Noort Luc, Exposito-Escudero Jesica, Medina-Cantillo Julita, Torras Carme, Natera-de Benito Daniel, Font-Llagunes Josep M, Pàmies-Vilà Rosa
Department of Mechanical Engineering and Institute for Research and Innovation in Health (IRIS), Universitat Politècnica de Catalunya - BarcelonaTech (UPC), Barcelona, Spain.
Institut de Recerca Sant Joan de Déu, Santa Rosa 39-57, 08950, Esplugues de Llobregat, Spain.
J Neuroeng Rehabil. 2025 Mar 18;22(1):63. doi: 10.1186/s12984-025-01602-2.
Duchenne muscular dystrophy (DMD) and spinal muscular atrophy (SMA) are neuromuscular diseases that lead to progressive muscle degeneration and weakness. Recent therapeutic advances for DMD and SMA highlight the need for accurate clinical evaluation. Traditionally, motor function of the upper limbs is assessed using motor function scales. However, these scales are influenced by clinician's interpretation and may lack accuracy. For this reason, clinicians are becoming interested in finding alternative solutions. In this context, Inertial Measurement Units (IMUs) have gained popularity, offering the possibility to quantitatively and objectively analyze motor function of patients to support clinicians' assessments. We analyzed upper limb kinematics of two groups of children with neuromuscular diseases, seventeen DMD patients and fifteen SMA patients, while performing the corresponding clinical assessment. These two groups were further subdivided into two categories (Category A and Category B), according to disease severity (Brooke scores and Brooke scores , respectively). The results were compared against a group of ten healthy children. The metrics showing the strongest correlation with the clinical score were the workspace area in the frontal and transverse plane (DMD: = 0.94 and = 0.90; SMA: = 0.78 and = 0.81) and the workspace volume (DMD: = 0.92; SMA = 0.81). Additionally, statistically significant differences were found not only between healthy children and those with neuromuscular disease, but also across severity levels within the patient group. These results represent a first step toward validating IMU-based systems to helping clinicians to accurately quantify the motor status of children with neuromuscular diseases. Furthermore, data collected with inertial sensors can provide clinicians with additional information not available through subjective observation.
杜氏肌营养不良症（DMD）和脊髓性肌萎缩症（SMA）是导致进行性肌肉退化和无力的神经肌肉疾病。DMD和SMA最近的治疗进展凸显了准确临床评估的必要性。传统上，上肢运动功能使用运动功能量表进行评估。然而，这些量表受临床医生解释的影响，可能缺乏准确性。因此，临床医生开始对寻找替代解决方案感兴趣。在这种背景下，惯性测量单元（IMU）受到欢迎，它为定量和客观分析患者的运动功能以支持临床医生的评估提供了可能性。我们在对两组患有神经肌肉疾病的儿童（17名DMD患者和15名SMA患者）进行相应临床评估时，分析了他们的上肢运动学。根据疾病严重程度（分别为布鲁克评分 和布鲁克评分 ），这两组又进一步细分为两类（A类和B类）。结果与一组10名健康儿童进行了比较。与临床评分相关性最强的指标是额面和横断面的工作区面积（DMD： = 0.94和 = 0.90；SMA： = 0.78和 = 0.81）以及工作区体积（DMD： = 0.92；SMA = 0.81）。此外，不仅在健康儿童与患有神经肌肉疾病的儿童之间发现了统计学上的显著差异，而且在患者组内不同严重程度水平之间也发现了显著差异。这些结果代表了验证基于IMU的系统以帮助临床医生准确量化神经肌肉疾病儿童运动状态的第一步。此外，通过惯性传感器收集的数据可以为临床医生提供主观观察无法获得的额外信息。
