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Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
Social behavior problems in boys with Duchenne muscular dystrophy
Veronica J Hinton1, Nancy E Nereo, Robert J Fee
1Gertrude H. Sergievsky Center and Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA. vjh9@columbia.edu
Insights
Boys with Duchenne muscular dystrophy (DMD) exhibit more social behavior problems than typically developing peers. This heightened risk may stem from neurological factors related to DMD, not just the chronic illness itself.
Area of Science:
- Pediatric Neurology
- Behavioral Science
- Genetics
Background:
- Duchenne muscular dystrophy (DMD) is a progressive genetic disorder impacting muscles and the brain.
- Understanding behavioral challenges in children with DMD is crucial for comprehensive care.
- Previous research has not fully elucidated the specific behavioral profile associated with DMD.
Purpose of the Study:
- To assess parent-reported behaviors in boys with DMD.
- To investigate the impact of chronic illness, intellectual ability, and disease etiology on behavior.
- To identify a distinct behavioral profile associated with Duchenne muscular dystrophy.
Main Methods:
- Parental ratings on the Child Behavior Checklist were analyzed for 181 boys with DMD.
- Data were compared with those of children with other chronic illnesses, unaffected siblings, and children with cerebral palsy.
- Statistical analyses examined group differences and correlations between behavioral ratings and disease factors.
Main Results:
- Boys with DMD showed increased general behavior problems.
- Social behavior problems were most frequently rated as clinically significant (34%) among DMD cases.
- Significantly higher rates of social behavior problems were observed in boys with DMD compared to sibling and cerebral palsy groups.
Conclusions:
- Boys with Duchenne muscular dystrophy may face a heightened risk for specific social behavior challenges.
- These behavioral differences may be linked to the neurological impact of DMD, including the absence of dystrophin isoforms in the central nervous system.
- Findings suggest that behavioral issues in DMD are not solely reactive responses to chronic illness.
Abstract:
Duchenne muscular dystrophy (DMD) is a chronic, progressive pediatric disease that affects both muscle and brain. The objectives of the study were to examine parent reported behavior in children with DMD, investigate the influence of chronic illness, intellectual ability and etiology on behavior, and determine whether a specific behavioral profile is associated with DMD. Parental ratings of boys with DMD (n = 181) on the Child Behavior Checklist behavior scales were examined and compared to reported findings of children with other chronic illnesses, unaffected siblings of boys with DMD (n = 86), and children with cerebral palsy (CP) (n = 42). Increased ratings of general behavior problems were reported, and neither physical progression nor intellectual level contributed to behavioral ratings. Among the children with DMD, the Social Problem behavior scale had the greatest number of "clinically significant" ratings (34%). Between-group comparisons showed significantly more boys with DMD were rated as having social behavior problems than either the sibling or CP comparison groups. In addition to the increase in reported behavioral problems likely related to the effects of chronic illness, boys with DMD may be at heightened risk for specific social behavior problems. The specificity of the findings of the behavior profile in DMD may be partially due to the lack of dystrophin isoforms in the central nervous system, and not solely a reactive response to the illness.
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