Social Competence in Persons with Prader‐Willi, Williams and Down's Syndromes

Abstract

Background This study explores everyday social competence in the lives of persons with three genetic intellectual disability syndromes. Methods Using parent reports on the Social and Activity Competence domains of Achenbach's Child Behavior Checklist (CBCL), socially competent behaviours were examined in 58 persons with Williams syndrome, 54 persons with Prader‐Willi syndrome, and 65 persons with Down's syndrome. Results The Down's syndrome group showed the highest overall social competence scores, while individuals with Prader‐Willi syndrome fared worst in terms of their behaviour with others. The Williams syndrome group scored lowest in terms of skill with jobs and chores, and the Down's syndrome group showed the least skill in non‐sport activities. Regarding specific activities, musical activities were popular in both Williams syndrome and Down's syndrome, but more participants with Williams syndrome played musical instruments. Compared with the other groups, individuals with Prader‐Willi syndrome were five to 25 times more likely to play with jigsaw puzzles, and those with Williams syndrome were least likely to engage in any visual–spatial leisure activities. Age was found to be a positive correlate of social competence for the Williams and Down's syndrome groups, but not for the Prader‐Willi group. Conclusions Implications are discussed for interventions and for future behavioural phenotype work.

Genomic Imprinting Is Implicated in the Psychology of Music

“…Parents of people with PWS are also less likely to report musical activities, when freely listing "non-sport activities" that their children enjoy, than are parents of people with Williams or Down syndromes (Rosner, Hodapp, Fidler, Sagun, & Dykens, 2004). It is unclear, however, whether reduced parental reporting of active musical activities reflects a general lack of interest in music or a more specifically altered psychology of music in PWS.…”

Emerging Behavioral Phenotypes and Dynamic Systems Theory

“…In particular, Hodapp and Burack (2006) describe that children may choose to engage in activities that involve areas of relative strength, and as such, they may practice those skills more intensely than areas of weakness. Indeed, Rosner, Hodapp, Fidler, Sagun, and Dykens (2004) found differences in patterns of participation in various activities and sports. From a dynamic systems perspective, these patterns of strength and weakness are attractor states that become stronger over time, as they are practiced.…”

Measuring behavior in genetic disorders of mental retardation

“…In terms of profiles, why do strengths become relatively stronger and weaknesses relatively weaker? In one study, Rosner et al [2004] found that, compared to those with either Down syndrome or Prader-Willi syndrome, fewer children with Williams syndrome routinely engaged in drawing, puzzles, arts and crafts, and other visually based leisure time activities. But, in addition to avoiding etiology-related weaknesses, do children with any of these syndromes capitalize on their strengths?…”