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Disabilities in children with Duchenne muscular dystrophy: a profile
K P Nair1, A Vasanth, M Gourie-Devi
1Department of Psychiatric and Neurological Rehabilitation, National Institute of Mental Health and Neurosciences, Bangalore, India.
Insights
Assessing disabilities in Duchenne muscular dystrophy (DMD) is key for rehabilitation. This study found motor function significantly impacts daily life disabilities in DMD children, with the Barthel index effectively quantifying these challenges.
Area of Science:
- Pediatric Rehabilitation
- Neuromuscular Disorders
- Clinical Assessment
Background:
- Duchenne muscular dystrophy (DMD) is a progressive genetic disorder affecting muscle strength.
- Effective rehabilitation requires accurate assessment of functional limitations.
- Quantifying disabilities is crucial for tailoring interventions in pediatric DMD patients.
Purpose of the Study:
- To identify and quantify disabilities in children with Duchenne muscular dystrophy.
- To correlate functional impairments with specific disabilities.
- To evaluate the utility of the Barthel index in assessing DMD-related disabilities.
Main Methods:
- Study included 31 pediatric patients diagnosed with Duchenne muscular dystrophy (age ≥ 4 years).
- Motor function was assessed using Total Motor Score, upper/lower extremity function grades, and timed function tests.
- Disability was quantified using the Barthel index.
Main Results:
- Mean scores for motor scales: Total Motor Score (52 ± 7.8), Total Functional Grade (4.4 ± 1.9), Timed Function Score (12.5 ± 5.8).
- Barthel index scores ranged from 45-95 (mean 70.8 ± 12.7).
- Motor function scales correlated with each other and the Barthel index; 30 children exhibited multiple disabilities influenced by motor power.
Conclusions:
- Motor impairment significantly influences the extent of daily life disabilities in children with Duchenne muscular dystrophy.
- The Barthel index is a valuable tool for identifying and quantifying specific functional limitations.
- Comprehensive disability assessment is vital for effective management and rehabilitation planning in DMD.
Abstract:
Proper assessment of disabilities is essential for rehabilitation of patients with Duchenne muscular dystrophy. The aim of this study was to identify and quantify the disabilities in children with Duchenne muscular dystrophy and correlate them with impairment. Thirty-one patients with Duchenne muscular dystrophy of age four years and above were studied. The motor functions were evaluated using total motor score, upper and lower extremity function grades and timed function tests. Disability was quantified with Barthel index. The mean scores of motor scales were: total motor score -52 +/- 7.8, total functional grade -4.4 +/- 1.9 and timed function score -12.5 +/- 5.8. Barthel index scores ranged from 45-95 with a mean of 70.8 +/- 12.7. Motor scales correlated with each other and with Barthel index. Thirty children had disabilities in multiple spheres of life, which were significantly influenced by the motor power. Barthel index was useful in identifying and quantifying specific areas of disabilities in these children. Evaluation of disabilities using specific measures may be crucial for planning comprehensive management.