title: The relationship between specific cognitive impairment and behaviour in Prader-Willi syndrome.
abstract: BACKGROUND
  Individuals with Prader-Willi syndrome (PWS) have been shown to demonstrate a particular cognitive deficit in attention switching and high levels of preference for routine and temper outbursts. This study assesses whether a specific pathway between a cognitive deficit and behaviour via environmental interaction can exist in individuals with PWS.
  
  
  METHODS
  Four individuals with PWS participated in a series of three single-case experiments including laboratory-based and natural environment designs. Cognitive (computer-based) challenges placed varying demands on attention switching or controlled for the cognitive demands of the tasks while placing no demands on switching. Unexpected changes to routines or expectations were presented in controlled games, or imposed on participants' natural environments and compared with control conditions during which no unexpected changes occurred. Behaviour was observed and heart rate was measured.
  
  
  RESULTS
  Participants showed significantly increased temper outburst related behaviours during cognitive challenges that placed demands on attention switching, relative to the control cognitive challenges. Participants showed significantly increased temper outburst related behaviours when unexpected changes occurred in an experimental or the natural environment compared with when no changes occurred.
  
  
  CONCLUSIONS
  Difficult behaviours that could be triggered reliably in an individual by a specific cognitive demand could also be triggered via manipulation of the environment. Results suggest that a directional relationship between a specific cognitive deficit and behaviour, via environmental interaction, can exist in individuals with PWS.
authors.affiliation: Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Birmingham, West Midlands, UK. papers@katewoodcock.com
authors.forename: K A
authors.initials: KA
authors.lastname: Woodcock
authors.forename: C
authors.initials: C
authors.lastname: Oliver
authors.forename: G W
authors.initials: GW
authors.lastname: Humphreys
mesh_terms: D000223:Adaptation, Psychological; D000293:Adolescent; D000786:Anger; D001288:Attention; D002648:Child; D004777:Environment; D005260:Female; D005364:Field Dependence-Independence; D006801:Humans; D008607:Intellectual Disability / Q000150:complications* / Q000175:diagnosis / Q000523:psychology; D011218:Prader-Willi Syndrome / Q000150:complications / Q000523:psychology*; D013239:Stereotyped Behavior; D013694:Temperament