Understanding and changing behaviour in Prader-Willi syndrome

Bull, Leah Elizabeth (2014). Understanding and changing behaviour in Prader-Willi syndrome. University of Birmingham. Ph.D.

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The thesis reviewed evidence of the behavioural characteristics associated with Prader-Willi syndrome (PWS) and how cross-sectional data has suggested that these may change with age, with a view to informing potential avenues and timescales for behavioural intervention. One highly plausible avenue identified that may be targeted for intervention arises from the finding that temper outbursts caused by change to routine or expectation are common within PWS. Longitudinal data collected over an eight year period explored changes in behaviour with age in people with PWS, finding that several phenotypic behaviours appear to peak around adolescence and decline in adulthood. In the remaining empirical studies, possible pathways to interventions to reduce temper outbursts in people with PWS were explored. It was shown that as someone has been exposed to a routine for longer they show more behavioural difficulties and higher arousal following a change. These data demonstrate potential utility for development of early interventions. An informant reported behaviour diary was validated and used to evaluate a stimulus control intervention to reduce temper outbursts. This intervention reduced temper outburst behaviour following specific changes as evidenced by structured observations, and reduced the number of temper outbursts shown in daily life by most participants.

Type of Work: Thesis (Doctorates > Ph.D.)
Award Type: Doctorates > Ph.D.
College/Faculty: Colleges (2008 onwards) > College of Life & Environmental Sciences
School or Department: School of Psychology
Funders: Other
Other Funders: Fondation Jérôme Lejeune
Subjects: B Philosophy. Psychology. Religion > BF Psychology
URI: http://etheses.bham.ac.uk/id/eprint/5037


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