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Body Weight and Range of Motion as Predictors of Trunk Asymmetry in Children With Spinal Muscular Atrophy: A
Ewa Gajewska1, Aleksandra Bieniaszewska1, Magdalena Sobieska2
1Department of Developmental Neurology, Poznań University of Medical Sciences, Poznań, Poland.
Insights
Increased body weight in children with spinal muscular atrophy (SMA) correlates with higher trunk asymmetry risk. Higher BMI in SMA patients is linked to hip contractures and improved foot positioning, highlighting the need for contracture assessment.
Area of Science:
- Pediatric Neurology
- Rehabilitation Medicine
- Biomechanical Analysis
Background:
- Spinal muscular atrophy (SMA) is a progressive neuromuscular disorder affecting motor neurons.
- SMA leads to motor impairment, contractures, and scoliosis, impacting quality of life.
- Understanding the biomechanical consequences of SMA is crucial for patient management.
Purpose of the Study:
- To investigate the relationship between body mass index (BMI), body weight, and structural trunk parameters in children with SMA.
- To assess the impact of BMI and body weight on contractures and joint range of motion in different SMA motor function categories.
- To determine the clinical significance of contracture and range of motion measurements in SMA evaluations.
Main Methods:
- Prospective study of 38 children with SMA (types 1, 2, and 3), categorized by motor function (non-sitters, sitters, walkers).
- Collected anthropometric data (BMI, BMI z-scores) and assessed structural trunk parameters, contractures, and joint range of motion.
- Analyzed correlations between BMI, body weight, and musculoskeletal parameters.
Main Results:
- No deterioration in structural trunk elements observed across SMA types.
- Increased lower limb contractures noted in non-sitters and sitters.
- Higher body weight correlated with increased trunk asymmetry risk; increased BMI associated with hip contractures and improved foot positioning.
Conclusions:
- Body weight is a significant factor influencing trunk asymmetry in children with SMA.
- BMI impacts hip and foot joint mobility, suggesting a need for standardized contracture and range of motion assessments.
- Integrating contracture and range of motion measurements into routine SMA evaluations is recommended.
Abstract:
BACKGROUND Spinal muscular atrophy (SMA) is a lower motor neuron disease characterized primarily by motor function impairment, as well as the development of contractures and scoliosis. MATERIAL AND METHODS A prospective study was conducted involving 38 children with SMA, including 8 with type 1, 20 with type 2, and 10 with type 3 SMA. Patients were categorized based on motor function into non-sitters (n=9), sitters (n=23), and walkers (n=6). Anthropometric measurements, including body mass index (BMI) and BMI z-scores, were recorded according to World Health Organization standards. Structural trunk parameters, the presence of contractures, and joint range of motion were assessed in all participants. RESULTS Changes in trunk parameters in all 3 groups showed no deterioration of structural elements. There was an increase in lower limb contractures measured at all 3 joints in the non-sitters and sitters groups. As BMI increased, the range of motion of the hips and right knee increased, while the left knee and feet remain unchanged. As body weight increased, the risk of trunk asymmetry increased in all patients. In the non-sitters group, there was an increase in hip and knee contractures regardless of BMI category. CONCLUSIONS As body weight increased, the risk of trunk asymmetry increased. As BMI increased, the neutral position of the hips changed (contracture increased), and the position of the knees did not change, while the neutral position of the feet improved. The measurement of contractures and range of motion should be introduced as a standard of practice in the evaluation of children with SMA.
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