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Daily Quantity and Kinematic Characteristics of Leg Movement in a Child With SMA (2 Copies SMN2)
Melissa McIntyre1, Lauren Dunn, Jed David
1Department 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.
Insights
Wearable sensors revealed distinct leg movement patterns in a child with spinal muscular atrophy (SMA) compared to typically developing infants. These findings highlight the potential of sensor technology for assessing motor function in SMA.
Area of Science:
- Biomedical Engineering
- Neurology
- Pediatrics
Background:
- Spinal muscular atrophy (SMA) is a genetic neuromuscular disorder affecting motor neurons.
- Disease-modifying therapies are improving outcomes for children with SMA.
- Objective assessment of motor function is crucial for tracking disease progression and treatment efficacy.
Observation:
- This case report details the use of wearable sensors to quantify daily leg movement in a child with SMA (2 copies of SMN2) receiving treatment.
- Movement quantity and kinematic characteristics, including duration, average acceleration, and peak acceleration, were measured over full days in a natural environment.
- The child's movement parameters differed significantly from published data on infants with typical development.
Findings:
- Wearable sensors captured unique movement quantity and kinematic data in a treated infant with SMA.
- These parameters were consistently different from those observed in typically developing infants.
- Movement quantity and clinician-rated outcomes showed an increase with age.
Implications:
- Wearable sensor data can provide complementary information to traditional assessments of motor function in SMA.
- This technology holds potential for objective, sensitive monitoring of motor development in children with SMA.
- Further longitudinal studies are warranted to validate wearable sensors as an assessment tool and early predictor of motor outcomes in SMA.
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.
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