Daily Quantity and Kinematic Characteristics of Leg Movement in a Child With SMA (2 Copies SMN2)

Abstract

Purpose: 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. Key Points: 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. Conclusions: 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. Recommendations: 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.

A Five-Year Review of Newborn Screening for Spinal Muscular Atrophy in the State of Utah: Lessons Learned

“…Emerging technologies such as wearable sensors have been proposed as a tool for the early identification of motor impairment and developmental delay [ 27 , 28 , 29 ]. These technologies may have particular application for prodromal infants with SMA identified via NBS and other neuromuscular disorders to aid in monitoring disease progression and treatment response in these youngest patients [ 30 ].…”