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Updated: Jun 2, 2026
Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
Intellectual and behavioral functioning in a South african cohort of boys with duchenne muscular dystrophy
Kirsten A M Donald1, Hlengani Mathema, Kevin G F Thomas
1Red Cross Children's Hospital, School of Child and Adolescent Health, University of Cape Town, Cape Town, South Africa. Kirsty.donald@uct.ac.za
Insights
Children with Duchenne muscular dystrophy (DMD) show mild cognitive issues and behavioral problems. These findings in South Africa align with global data, though behavioral issues may be higher due to socioeconomic factors.
Area of Science:
- Neuroscience
- Pediatrics
- Genetics
Background:
- Duchenne muscular dystrophy (DMD) is a progressive genetic disorder affecting muscle function.
- Understanding the neurocognitive and behavioral impact of DMD is crucial for comprehensive patient care.
Purpose of the Study:
- To describe the neurocognitive and behavioral phenotype of South African children with DMD.
- To compare these findings with international data and explore potential contributing factors.
Main Methods:
- A cohort of 16 South African children (ages 4-16) with DMD was assessed.
- Cognitive abilities were evaluated using standardized tests.
- Behavioral assessments were conducted through parental reports.
Main Results:
- Preschoolers with DMD performed significantly worse on general developmental measures compared to controls.
- School-aged boys with DMD showed no significant differences in general intellectual abilities.
- Mild cognitive dysfunction was noted in visual memory, verbal, and nonverbal executive functioning.
- Parental reports indicated high rates of behavioral problems (54.5%) in boys with DMD.
- The cognitive profile is similar to global findings, but behavioral issues appear more prevalent in this cohort.
Conclusions:
- Boys with DMD experience mild cognitive dysfunction across several domains.
- Behavioral problems are common in children with DMD, potentially exacerbated by socioeconomic factors.
- The neurocognitive and behavioral phenotype of DMD in South Africa mirrors international observations, with a notable increase in behavioral challenges.
Abstract:
The authors describe the neurocognitive and behavioral phenotype of 16 South African children (ages 4-16 years) with Duchenne muscular dystrophy, compared with 13 controls. The preschool group performed significantly worse than the controls in measures of general developmental performance. The school-age group showed no significant differences in general intellectual abilities. However, our data identified that boys with Duchenne muscular dystrophy experience mild cognitive dysfunction across multiple domains, including visual memory, and verbal and nonverbal executive functioning. Parental reports of 11 boys with the disorder revealed high rates of general behavioral problems (54.5%). The cognitive profile of boys with Duchenne muscular dystrophy in South Africa is similar to that described elsewhere in the world. Behavioral problems in our cohort were higher than previously reported. This may be partially explained by the poor socioeconomic background of the majority of the cohort.
