Key result
Declining motor function links to worse subclinical LV dysfunction in ambulatory Duchenne muscular dystrophy.
Why the study?
Heart problems may begin early in Duchenne muscular dystrophy and may be associated with declining motor function, but the relationship is not well defined.
Observational (n=32)
No
Effect estimate: r = -0.51
p-value: p=0.002
In ambulatory boys with Duchenne muscular dystrophy, declining motor function is significantly associated with early subclinical myocardial dysfunction, suggesting that motor function scores can help identify patients needing early cardiological screening.
Motor decline may flag early cardiac dysfunction in ambulatory DMD boys; hypothesis-generating and requires prospective validation before guiding care.
Background Duchenne muscular dystrophy (DMD) is an X-linked recessive neuromuscular disorder caused by mutations in the dystrophin gene. It is a severe form of muscular dystrophy that is characterized by increasing muscle weakness. Heart problems may begin early in the course of the disease and may be associated with declining motor function. Aim To determine if there is a relationship between declining motor function and early cardiac dysfunction in ambulatory boys with DMD. Subjects and methods This analytic observational study consisted of 32 ambulatory boys diagnosed with genetically diagnosed DMD and who sought Neurology Clinic services at Fayoum University Hospitals. All subjects underwent clinical evaluation, including evaluation using the Motor Function Measure (MFM) scale and electrocardiogram and echocardiogram studies. Results The study revealed that the average age of the patients was 8.9 ± 1.8 years, with the average symptom duration being 4.1 ± 1.5 years. The average total Motor Function Measure was found to be 72.8 ± 13. The average left ventricular ejection fraction was found to be 58.4 ± 5.6%, whereas the average global longitudinal strain was found to be − 18.1 ± 2.3%. Mild systolic dysfunction was observed in 25% of the patients. Significant correlations were observed between total motor score and left ventricular ejection fraction ( r = 0.46, p = 0.004), total motor score, and global longitudinal strain ( r = − 0.51, p = 0.002). Conclusion It was observed that the deterioration of motor functions in ambulatory boys with DMD is associated with the early signs of cardiac dysfunction. It was found that the motor functions, if monitored by the MFM scale, would help to identify the patients at a high risk of developing cardiac dysfunction, thereby highlighting the need for early cardiological examination.
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Elawady et al. (2026) conducted an observational in Duchenne muscular dystrophy (n=32). Motor function decline (MFM score) was evaluated on Correlation between total Motor Function Measure (MFM) score and global longitudinal strain (GLS) (r = -0.51, p=0.002). Lower total motor function scores in ambulatory boys with Duchenne muscular dystrophy were significantly correlated with worse global longitudinal strain (r = -0.51) and left ventricular ejection fraction (r = 0.46).
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