The Relationship between Components of the Behavioural Phenotype in Prader-Willi Syndrome

Chris Oliver, Kate A. Woodcock*, Glyn W. Humphreys

*Corresponding author for this work

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Background

Repetitive questions and temper outbursts form part of the behavioural phenotype of Prader-Willi syndrome (PWS). We investigated the phenomenology of temper outbursts in PWS and their relationship with other PWS behavioural characteristics.

Method

Four individuals with PWS were observed (5-10 h), during a number of experimental and natural environment challenges, some of which were expected to trigger temper outbursts. Individual behaviours including crying, ignoring, arguing, questioning, stereotypy, frowning and posture changes were recorded and subjected to lag sequential analysis.

Results

All participants were significantly more likely to show repetitive questioning before more challenging behaviours such as crying, arguing or ignoring requests. Precursor behaviours such as frowning and stereotypical behaviour were identified in three participants.

Conclusions

Temper outbursts in PWS may be associated with other PWS behavioural phenotypic characteristics such as repetitive questions and 'stubbornness'. A progression of behaviours may lead up to the most challenging temper outburst behaviours. This may have important implications for effective coping strategies.

Original languageEnglish
Pages (from-to)403-407
Number of pages5
JournalJournal of Applied Research in Intellectual Disabilities
Volume22
Issue number4
DOIs
Publication statusPublished - Jul 2009

Keywords

  • behavioural hierarchy
  • functional equivalence
  • precursor behaviours
  • repetitive questions
  • stubbornness
  • temper tantrum
  • DISTURBANCE
  • CHILDREN
  • PEOPLE

Cite this

Oliver, Chris ; Woodcock, Kate A. ; Humphreys, Glyn W. / The Relationship between Components of the Behavioural Phenotype in Prader-Willi Syndrome. In: Journal of Applied Research in Intellectual Disabilities. 2009 ; Vol. 22, No. 4. pp. 403-407.
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abstract = "BackgroundRepetitive questions and temper outbursts form part of the behavioural phenotype of Prader-Willi syndrome (PWS). We investigated the phenomenology of temper outbursts in PWS and their relationship with other PWS behavioural characteristics.MethodFour individuals with PWS were observed (5-10 h), during a number of experimental and natural environment challenges, some of which were expected to trigger temper outbursts. Individual behaviours including crying, ignoring, arguing, questioning, stereotypy, frowning and posture changes were recorded and subjected to lag sequential analysis.ResultsAll participants were significantly more likely to show repetitive questioning before more challenging behaviours such as crying, arguing or ignoring requests. Precursor behaviours such as frowning and stereotypical behaviour were identified in three participants.ConclusionsTemper outbursts in PWS may be associated with other PWS behavioural phenotypic characteristics such as repetitive questions and 'stubbornness'. A progression of behaviours may lead up to the most challenging temper outburst behaviours. This may have important implications for effective coping strategies.",
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author = "Chris Oliver and Woodcock, {Kate A.} and Humphreys, {Glyn W.}",
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The Relationship between Components of the Behavioural Phenotype in Prader-Willi Syndrome. / Oliver, Chris; Woodcock, Kate A.; Humphreys, Glyn W.

In: Journal of Applied Research in Intellectual Disabilities, Vol. 22, No. 4, 07.2009, p. 403-407.

Research output: Contribution to journalArticle

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N2 - BackgroundRepetitive questions and temper outbursts form part of the behavioural phenotype of Prader-Willi syndrome (PWS). We investigated the phenomenology of temper outbursts in PWS and their relationship with other PWS behavioural characteristics.MethodFour individuals with PWS were observed (5-10 h), during a number of experimental and natural environment challenges, some of which were expected to trigger temper outbursts. Individual behaviours including crying, ignoring, arguing, questioning, stereotypy, frowning and posture changes were recorded and subjected to lag sequential analysis.ResultsAll participants were significantly more likely to show repetitive questioning before more challenging behaviours such as crying, arguing or ignoring requests. Precursor behaviours such as frowning and stereotypical behaviour were identified in three participants.ConclusionsTemper outbursts in PWS may be associated with other PWS behavioural phenotypic characteristics such as repetitive questions and 'stubbornness'. A progression of behaviours may lead up to the most challenging temper outburst behaviours. This may have important implications for effective coping strategies.

AB - BackgroundRepetitive questions and temper outbursts form part of the behavioural phenotype of Prader-Willi syndrome (PWS). We investigated the phenomenology of temper outbursts in PWS and their relationship with other PWS behavioural characteristics.MethodFour individuals with PWS were observed (5-10 h), during a number of experimental and natural environment challenges, some of which were expected to trigger temper outbursts. Individual behaviours including crying, ignoring, arguing, questioning, stereotypy, frowning and posture changes were recorded and subjected to lag sequential analysis.ResultsAll participants were significantly more likely to show repetitive questioning before more challenging behaviours such as crying, arguing or ignoring requests. Precursor behaviours such as frowning and stereotypical behaviour were identified in three participants.ConclusionsTemper outbursts in PWS may be associated with other PWS behavioural phenotypic characteristics such as repetitive questions and 'stubbornness'. A progression of behaviours may lead up to the most challenging temper outburst behaviours. This may have important implications for effective coping strategies.

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