Social Behavior Problems in Boys with Duchenne Muscular Dystrophy

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.

Quality of life of patients with Duchenne muscular dystrophy: from adolescence to young men

“…Social behavior problems may occur earlier in children with DMD, who tend to have poor social and interpersonal behavior skills. [32] In this study, the scores of the SF and Social Relationships were correlated well with the functional status ratings of the MDFRS. Therefore, strategies for improving functional performance may yield improvements in their social Indicates statistical significance between young and oldest groups (p < 0.05).…”

Neurobehavioral Concerns Among Males with Dystrophinopathy Using Population-Based Surveillance Data from the Muscular Dystrophy Surveillance, Tracking, and Research Network

“…4–7 Among the neurobehavioral concerns studied, higher frequencies of ADHD, 6,8,9 autism spectrum disorder, 8,10–13 and obsessive-compulsive disorder (OCD) 8 have been reported for those affected with a dystrophinopathy compared to the general population. 8,11 Other studies have reported high levels of clinically significant externalizing and internalizing behaviors 11,13–15 and difficulties with social functioning 11,14–16 among affected individuals. These neurobehavioral and psychosocial concerns have been attributed, in part, to underlying neurocognitive deficits 17 and physical limitations that may arise with greater disease severity.…”

Behavioral problems in children with motor and intellectual disabilities: Prevalence and associations with maladaptive personality and marital relationship

“…Accordingly, the types of behavioral problems in children with motor disabilities also varied. The most reported problems are anxiety (Hendriks et al, 2001;Van Handel, Swaab, De Vries, & Jongmans, 2010), attention problems (Peters et al, 1999;Sigurdardottir et al, 2010), social problems (Hinton, Nereo, Fee, & Cyrulnik, 2006) and hyperactivity (Brossard-Racine et al, 2012;McDermott et al, 1996;Parkes et al, 2008). However, the use of different questionnaires hampers the comparability of studies addressing the prevalence of different types of problem behavior.…”