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Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
Quantitative coordination evaluation for screening children with Duchenne muscular dystrophy
Jian An1, Zhiying Xie2, Fan Jia1
1Academy of Advanced Interdisciplinary Studies, Peking University, Beijing 100871, China.
Insights
Early Duchenne muscular dystrophy (DMD) diagnosis is crucial. A new gait analysis method using relative coupling coefficient (RCC) effectively distinguishes DMD from typically developing children, aiding early screening.
Area of Science:
- Biomedical Engineering
- Neurology
- Pediatrics
Background:
- Early diagnosis of Duchenne muscular dystrophy (DMD) is vital for timely treatment.
- Children with DMD often exhibit early motor deficits, particularly in balance and coordination.
- Quantifying these coordination differences is key for developing effective screening tools.
Purpose of the Study:
- To develop and validate a novel method for quantifying coordination differences between children with DMD and typically developing (TD) children.
- To introduce a new index, the relative coupling coefficient (RCC), derived from gait patterns.
- To assess the feasibility and reliability of RCC for early DMD screening.
Main Methods:
- Utilized a Local Manifold Structure Mapping approach in phase space to analyze gait data.
- Extracted the relative coupling coefficient (RCC) from gait signals acquired via wearable accelerometers.
- Compared RCC values between 100 children with DMD and 100 TD children across four age groups.
Main Results:
- Significant differences in RCC were observed between DMD and TD children in all age groups (p < 0.001).
- RCC demonstrated a gradual decrease in coordination ability with age in DMD patients, aligning with clinical observations.
- The study verified the feasibility and reliability of RCC in distinguishing DMD from TD children.
Conclusions:
- The relative coupling coefficient (RCC) serves as a sensitive functional biomarker for differentiating DMD from TD children.
- RCC extracted from gait data offers a valuable tool for the early screening and potential diagnosis of DMD.
- This approach provides novel insights into the motor coordination impairments associated with DMD.
Abstract:
As the potential for a treatment of Duchenne muscular dystrophy (DMD) grows, the need for methods for the early diagnosis of DMD becomes more and more important. Clinical experiences suggest that children with DMD will show some lack of motor ability in the early stage when compared with children at the same age, especially in balance and coordination abilities. Is it possible to quantify the coordination differences between DMD and typically developing (TD) children to achieve the goal of screening for DMD diseases? In this study, we introduced a Local Manifold Structure Mapping approach in phase space and extracted a novel index, relative coupling coefficient (RCC), from gait pattern signals, which were acquired by wearable accelerometers to evaluate the coordination of children with DMD during a walking task. Furthermore, we compared the RCC of 100 children with DMD and 100 TD children in four different age groups and verified the feasibility and reliability of the proposed indices to distinguish children with TD from DMD. T-test results show that, for all age groups, children of the same age with DMD and TD show significant differences in RCC (p < 0.001). Moreover, RCC comprehensively reflects that the coordination ability of DMD patients under walking tasks gradually decreases with age, which is consistent with clinical experience. As a functional biomarker extracted in the phase space of the gait data, the proposed coupling degree index RCC could sensitively distinguish between DMD and TD children at the same age and provide alternative insights and potentially valuable tools for the screening of DMD.

