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Title: An investigation of executive function abilities in adults with Prader-Willi syndrome
Author: Walley, Robert M.
Awarding Body: University of Edinburgh
Current Institution: University of Edinburgh
Date of Award: 2006
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Prader-Willi syndrome (PWS) is a genetic disorder caused by the absence of expression of maternally imprinted genes on chromosome 15. There are two main sub-types, deletion and uniparental disomy. In addition to mild/borderline intellectual disability and the almost universal feature of hyperphagia, PWS is associated with high rates of behaviour problems. The present study seeks to explore whether these behaviour problems are associated with relative deficits in executive function (EF), which comprises the non-automatic processes utilized by an individual when faced with a novel situation. Nineteen adult participants with a clinical diagnosis of PWS (12 with deletion sub-type, 6 with UPD, and 1 with an uncertain genetic diagnosis) were recruited from a PWS clinic, and compared with 15 participants of similar age and verbal ability on a series of EF tasks and Digit Span Forwards. An informant completed two ratings of behaviour; the Aberrant Behaviour Checklist (ABC) and the Dysexecutive questionnaire (DEX). The PWS group had significantly higher scores on the ABC not the DEX. There were no significant differences between the whole PWS group and the comparison group on any of the EF tasks, but there was a non-significant trend for the deletion group to show more efficient performance on a planning task. The deletion group was significantly poorer at Digit Span Forwards. The lack of relative deficits in EF task performance does not support the hypotheses that EF differences could account for the high levels of behaviour problems found in PWS. Applying the Baddeley and Hitch model of working memory it is suggested the PWS deletion group may have a relative impairment in the capacity of the phonological store. As differences in EF ability were not found, it is suggested that the orbitofrontal cortex, which is involved in the modulation of emotion but not EF, may be implicated in the behaviour problems reported in PWS.
Supervisor: Not available Sponsor: Not available
Qualification Name: Thesis (D.Psych.) Qualification Level: Doctoral
EThOS ID:  DOI: Not available