The prevalence and phenomenology of self-injurious and aggressive behaviour in genetic syndromes

J Intellect Disabil Res. 2011 Feb;55(2):109-20. doi: 10.1111/j.1365-2788.2010.01337.x. Epub 2010 Oct 26.

Abstract

Background: Self-injurious and aggressive behaviours are reported as components of some behavioural phenotypes but there are few studies comparing across syndrome groups. In this study we examined the prevalence of these behaviours and the associated person characteristics in seven genetic syndromes.

Methods: Questionnaire data on self-injury and aggression, mood, hyperactivity, autism spectrum disorder and repetitive behaviour were collected on Angelman (AS, n=104), Cornelia de Lange (CdLS, 101), Cri du Chat (CdCS, 58), Fragile X (FXS, 191), Lowe (LS, 56), Prader-Willi (PWS, 189) and Smith-Magenis (SMS, 42) syndromes.

Results: A significantly higher prevalence of self-injury was evident in CdCS, CdLS, FXS, PWS, LS and SMS. The prevalence of aggression was significantly heightened in AS and SMS. Self-injury was associated with repetitive and impulsive behaviour in CdLS, FXS, PWS and LS. Impulsivity and overactivity were significantly higher in those showing aggression across all syndrome groups.

Conclusions: These data quantify the risk for self-injury and aggression in the syndromes studied with implications for early intervention. The associations between these behaviours and person characteristics both within and between syndromes warrant further research.

Publication types

  • Comparative Study

MeSH terms

  • Abnormalities, Multiple / epidemiology
  • Abnormalities, Multiple / genetics
  • Abnormalities, Multiple / psychology
  • Adolescent
  • Adult
  • Aggression*
  • Angelman Syndrome / epidemiology
  • Angelman Syndrome / psychology
  • Behavioral Symptoms / epidemiology*
  • Behavioral Symptoms / genetics
  • Behavioral Symptoms / psychology
  • Case-Control Studies
  • Child
  • Child Development Disorders, Pervasive / epidemiology*
  • Child Development Disorders, Pervasive / psychology
  • Child, Preschool
  • Chromosome Disorders / epidemiology
  • Chromosome Disorders / psychology
  • Comorbidity
  • Cri-du-Chat Syndrome / epidemiology
  • Cri-du-Chat Syndrome / psychology
  • De Lange Syndrome / epidemiology
  • De Lange Syndrome / psychology
  • Female
  • Fragile X Syndrome / epidemiology
  • Fragile X Syndrome / psychology
  • Genetic Diseases, Inborn / epidemiology*
  • Genetic Diseases, Inborn / psychology
  • Humans
  • Intellectual Disability / psychology
  • Male
  • Middle Aged
  • Oculocerebrorenal Syndrome / epidemiology
  • Oculocerebrorenal Syndrome / psychology
  • Prader-Willi Syndrome / epidemiology
  • Prader-Willi Syndrome / psychology
  • Prevalence
  • Self-Injurious Behavior / epidemiology*
  • Self-Injurious Behavior / psychology
  • Smith-Magenis Syndrome / epidemiology
  • Smith-Magenis Syndrome / psychology
  • Syndrome
  • Young Adult