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[Motor development in children with muscular dystrophy of the Duchenne type]
Insights
Duchenne muscular dystrophy (DMD) often causes delayed motor development, with onset typically around age one. Importantly, the age of disease onset does not influence the progression to immobilization, which averages around ten years.
Area of Science:
- Neurology
- Pediatrics
- Genetics
Context:
- Duchenne muscular dystrophy (DMD) is a progressive genetic disorder affecting motor development.
- Understanding the timeline of DMD is crucial for patient management and prognosis.
Purpose:
- To determine the age of onset and age of immobilization in Duchenne muscular dystrophy.
- To analyze the relationship between motor development, age of onset, and disease progression.
Summary:
- Motor development was assessed in 129 children with DMD, revealing delays and abnormalities in 58% of cases.
- The average age for walking was 19 months. Disease onset occurred around age one in 83% of patients.
- Earlier onset did not correlate with faster disease progression; immobilization typically occurred around age 10, irrespective of onset age.
Impact:
- Provides critical data on the natural history of Duchenne muscular dystrophy.
- Informs clinical expectations and management strategies for children with DMD.
- Highlights the consistent progression to immobilization around age 10 in DMD patients.
Abstract:
For determination of the age of onset and the age of immobilization in Duchenne's progressive muscular dystrophy the motor development was studied in 129 affected children. It was demonstrated that motor development was delayed and abnormal in 58% of the cases. On the average the affected children started to walk in the 19th month of life. According to the history data in 83% of the cases the age of onset of the disease was 1 year; in 10% of the cases the onset was placed before and in 7% after the 5th year of life. It was observed that earlier age of onset was not associated with a more rapid development of the disease and independently of the onset the age of immobilization was about 10 years. The results of investigation were compared with reports in the literature.