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Duchenne muscular dystrophy: a study of wrist and hand function
M B Wagner1, P J Vignos, C Carlozzi
1Department of Medicine, University Hospitals of Cleveland, Ohio.
Muscle & Nerve
|March 1, 1989
Summary
Early hand and wrist abnormalities, including contractures and joint deformities, are common in Duchenne muscular dystrophy (DMD) patients. Prompt assessment and intervention are crucial to manage these progressive conditions in individuals with DMD.
Area of Science:
- Orthopedics
- Neurology
- Pediatrics
Background:
- Duchenne muscular dystrophy (DMD) is a progressive genetic disorder affecting muscle strength.
- Hand and wrist involvement in DMD can significantly impact daily function and quality of life.
Purpose of the Study:
- To investigate the spectrum and age of onset of wrist and hand abnormalities in Duchenne muscular dystrophy patients.
- To highlight the need for early clinical assessment and potential interventions for hand deformities in DMD.
Main Methods:
- Retrospective analysis of 18 Duchenne muscular dystrophy patients.
- Categorization of patients into three age groups for comparative assessment.
- Clinical evaluation of wrist and hand deformities, including contractures and joint abnormalities.
Main Results:
- Wrist flexion and ulnar deviation contractures were observed starting in the youngest age group (8-14 years).
- Extrinsic and intrinsic digital muscle shortness, boutonniere and swan neck deformities, and digital joint hyperextension were prevalent across all age groups.
- New finding: Pain associated with passive proximal interphalangeal joint flexion was noted.
Conclusions:
- Early and regular assessment of the wrist and hand is essential for managing Duchenne muscular dystrophy.
- Intervention strategies should be considered early to potentially slow the progression of hand and wrist deformities in DMD.
- The study identifies previously unreported pain during specific joint movements, underscoring the need for comprehensive evaluation.