Understanding the Characteristics and Causal Mechanisms of Temper Outbursts in Prader-Willi Syndrome
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USyd Access
Type
ThesisThesis type
Doctor of PhilosophyAuthor/s
Rice, LaurenAbstract
Temper outbursts are a persistent cause of morbidity for people with Prader-Willi syndrome (PWS), for which there is no known treatment. Understanding the causal mechanisms of these behaviours is essential for the development of effective and targeted interventions. However, at ...
See moreTemper outbursts are a persistent cause of morbidity for people with Prader-Willi syndrome (PWS), for which there is no known treatment. Understanding the causal mechanisms of these behaviours is essential for the development of effective and targeted interventions. However, at present, there is a paucity of research investigating the pathophysiology of PWS. There are also observable aspects of outbursts that are not yet understood. This thesis aimed to bridge the gaps in our understanding of the observable characteristics of PWS outbursts through a purpose-developed survey completed by 100 parents. The findings suggest the features of outbursts displayed by children with PWS are similar to that of tantrums displayed by typically developing toddlers. As children with PWS moved into adolescence and adulthood outburst characteristics became more similar to outbursts displayed by clinical populations. Two literature reviews were conducted to explore the mechanisms of tantrums in the typically developing and outbursts in clinical populations. Two mechanisms that might play a role in PWS outbursts were identified, namely gamma-aminobutyric acid (GABA) and oxytocin. Magnetic resonance spectroscopy was used to evaluate the levels of GABA in the PWS brain. Compared to controls and individuals with PWS with moderate to low behaviour problems individuals with PWS with high rates of behaviour problem had reduced GABA, which was associated with outbursts. A randomised, double-blind placebo-controlled trial was conducted to evaluate whether intranasal oxytocin could improve symptoms of PWS. The only difference found between the oxytocin and control phase was an increase in outbursts for participants on the higher oxytocin dose. The findings from this thesis have increased our understanding of PWS outburst characteristics, identified potential mechanisms for these behaviours and led to the development of four new studies.
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See moreTemper outbursts are a persistent cause of morbidity for people with Prader-Willi syndrome (PWS), for which there is no known treatment. Understanding the causal mechanisms of these behaviours is essential for the development of effective and targeted interventions. However, at present, there is a paucity of research investigating the pathophysiology of PWS. There are also observable aspects of outbursts that are not yet understood. This thesis aimed to bridge the gaps in our understanding of the observable characteristics of PWS outbursts through a purpose-developed survey completed by 100 parents. The findings suggest the features of outbursts displayed by children with PWS are similar to that of tantrums displayed by typically developing toddlers. As children with PWS moved into adolescence and adulthood outburst characteristics became more similar to outbursts displayed by clinical populations. Two literature reviews were conducted to explore the mechanisms of tantrums in the typically developing and outbursts in clinical populations. Two mechanisms that might play a role in PWS outbursts were identified, namely gamma-aminobutyric acid (GABA) and oxytocin. Magnetic resonance spectroscopy was used to evaluate the levels of GABA in the PWS brain. Compared to controls and individuals with PWS with moderate to low behaviour problems individuals with PWS with high rates of behaviour problem had reduced GABA, which was associated with outbursts. A randomised, double-blind placebo-controlled trial was conducted to evaluate whether intranasal oxytocin could improve symptoms of PWS. The only difference found between the oxytocin and control phase was an increase in outbursts for participants on the higher oxytocin dose. The findings from this thesis have increased our understanding of PWS outburst characteristics, identified potential mechanisms for these behaviours and led to the development of four new studies.
See less
Date
2016-06-30Licence
The author retains copyright of this thesis. It may only be used for the purposes of research and study. It must not be used for any other purposes and may not be transmitted or shared with others without prior permission.Faculty/School
Faculty of Health SciencesAwarding institution
The University of SydneyShare