Department of Pediatrics (Dr McIntyre), University of Utah, Salt Lake City, Utah; Department of Rehabilitation Services (Dr Dunn and Mr David), Children's Hospital Los Angeles, Los Angeles, California; Developmental Neuroscience and Neurogenetics Program (Dr Smith), The Saban Research Institute, Division of Developmental-Behavioral Pediatrics, Children's Hospital Los Angeles, Los Angeles, California; Department of Pediatrics (Dr Smith), Keck School of Medicine, University of Southern California, Los Angeles, California.
Pediatr Phys Ther. 2023 Oct 1;35(4):486-492. doi: 10.1097/PEP.0000000000001053.
This case report describes daily leg movement quantity and kinematic characteristics of a child with spinal muscular atrophy (SMA) with 2 copies of SMN2, who was symptomatic at the time of treatment with disease-modifying therapies.
Compared with infants with typical development, this child had differing values for leg movement quantity, duration, average acceleration, and peak acceleration measured across full days in the natural environment by wearable sensors. In addition, movement quantity and clinician-rated outcomes increased with age.
Wearable sensors recorded movement quantity and kinematic characteristics in a treated infant with SMA (2 copies SMN2). These movement parameters were consistently different compared anecdotally with published data from infants with typical development, demonstrating their potential to add unique and complementary information to the assessment of motor function in SMA.
Larger longitudinal studies are needed to determine the utility of wearable sensors as an assessment tool and an early predictor of motor outcomes in children with SMA.
本病例报告描述了一名患有脊髓性肌萎缩症（SMA）且携带 2 个 SMN2 拷贝的儿童在接受疾病修正治疗时的日常腿部运动数量和运动学特征。
与具有典型发育的婴儿相比，通过可穿戴传感器在自然环境中全天测量，该儿童的腿部运动数量、持续时间、平均加速度和峰值加速度值不同。此外，运动数量和临床医生评定的结果随年龄增长而增加。
可穿戴传感器记录了患有 SMA（2 个 SMN2 拷贝）的治疗中婴儿的运动数量和运动学特征。这些运动参数与典型发育婴儿的已发表数据相比存在差异，这表明它们有可能为 SMA 患儿的运动功能评估提供独特且互补的信息。
需要进行更大的纵向研究来确定可穿戴传感器作为评估工具和 SMA 患儿运动结果的早期预测指标的效用。
