Problem behaviors associated with deletion Prader-Willi, Smith-Magenis, and cri du chat syndromes
behavioral problems; Cri-du-chat; trajectory; characteristics
Problem behaviors of individuals who had one of three chromosome deletion disorders (5p- cri-du-chat, 15q- Prader-Willi, or 17p- Smith-Magenis) were investigated. The Aberrant Behavior Checklist was used. Results were contrasted with those of two groups of people with mental retardation who were described in other studies. The checklist rates many, but not all, potentially relevant behaviors. Eating abnormalities, known to be problematic in Prader-Willi syndrome, and sleep abnormalities, believed to be problematic in Smith-Magenis syndrome, were not included in the survey. All three disorders were associated with greater ratings of problem behaviors than the comparison groups on at least one subscale of the checklist. The results lend support to the partial specificity model of behaviors associated with genetically determined syndromes.
Clarke D J; Boer H
American Journal of Mental Retardation
1998
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1352/0895-8017(1998)103" target="_blank" rel="noreferrer noopener">10.1352/0895-8017(1998)103<0264:PBAWDP>2.0.CO;2</a>
Self-injurious behavior, self-restraint, and compulsive behaviors in Cornelia de Lange syndrome
Male; Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Compulsive Behavior; De Lange Syndrome/px [Psychology]; Restraint Physical/px [Psychology]; Self-Injurious Behavior/px [Psychology]; behavioral problems; De Lange syndrome; trajectory; characteristics; self-injurious behavior; self-restraint
Researchers have argued that individuals who show self-restraint are more likely to show self-injurious behavior (SIB) that is compulsive. Self-injurious behavior, self-restraint, and compulsive behaviors have been described as features of Cornelia de Lange syndrome. We examined whether individuals with this syndrome displaying SIB and self-restraint exhibit more compulsive behaviors than do those without SIB and self-restraint. Main caregivers of individuals with the syndrome completed questionnaires. A significant association was found between SIB and self-restraint, and those displaying both behaviors displayed significantly more compulsions than did those not exhibiting them. Findings extend the compulsive behavior theory and highlight areas for further research.
Hyman P; Oliver C; Hall S
American Journal of Mental Retardation
2002
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1352/0895-8017(2002)107" target="_blank" rel="noreferrer noopener">10.1352/0895-8017(2002)107<0146:SIBSRA>2.0.CO;2</a>