Multidimensional Biomechanics-Based Score to Assess Disease Progression in Duchenne Muscular Dystrophy

Carolina Migliorelli, ,Meritxell Gómez-Martinez, Paula Subías-Beltrán, Mireia Claramunt-Molet,Sebastian Idelsohn-Zielonka, Eudald Mas-Hurtado, Felip Miralles, Marisol Montolio, Marina Roselló-Ruano, Julita Medina-Cantillo

Unit of Digital Health, Eurecat, Barcelona


Duchenne muscular dystrophy, becker muscular dystrophy, six minute-walk test, north star ambulatory assessment, biomechanics, biomechanics data analysis, gait


(1) Background: Duchenne (DMD) is a rare neuromuscular disease that progressively weakens muscles, which severely impairs gait capacity. The Six Minute-Walk Test (6MWT), which is commonly used to evaluate and monitor the disease’s evolution, presents significant variability due to extrinsic factors such as patient motivation, fatigue, and learning effects. Therefore, there is a clear need for the establishment of precise clinical endpoints to measure patient mobility. (2) Methods: A novel score (6M+ and 2M+) is proposed, which is derived from the use of a new portable monitoring system capable of carrying out a complete gait analysis. The system includes several biomechanical sensors: a heart rate band, inertial measurement units, electromyography shorts, and plantar pressure insoles. The scores were obtained by processing the sensor signals and via gaussian-mixture clustering. (3) Results: The 6M+ and 2M+ scores were evaluated against the North Star Ambulatory Assessment (NSAA), the gold-standard for measuring DMD, and six- and two-minute distances. The 6M+ and 2M+ tests led to superior distances when tested against the NSAA. The 6M+ test and the 2M+ test in particular were the most correlated with age, suggesting that these scores better characterize the gait regressions in DMD. Additionally, the 2M+ test demonstrated an accuracy and stability similar to the 6M+ test. (4) Conclusions: The novel monitoring system described herein exhibited good usability with respect to functional testing in a clinical environment and demonstrated an improvement in the objectivity and reliability of monitoring the evolution of neuromuscular diseases.

Moticon's Summary

In this study the authors propose and evaluate novel scores for detecting Duchenne muscular dystrophy (DMD). The novel score is derived form a portable monitoring system for gait analysis. This monitoring systems includes a heart rate band, IMUs, EMG shorts and Moticon sensor insoles. The resulting scores were evaluated against the North Star Ambulatory Assessment which constitutes the gold standard for measuring DMD. The novel scores showed promising validity and reliability as well as improved sensitivity in measuring DMD and neuromuscular diseases.

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