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Self-injurious and aggressive behaviour in Angelman, Cri du Chat and Cornelia de Lange syndromes

Tunnicliffe, Penelope Louisa (2010)
Ph.D. thesis, University of Birmingham.

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Abstract

In a series of studies, the role of operant reinforcement of phenotypic problem behaviours in Angelman, Cri du Chat and Cornelia de Lange syndromes was explored. Firstly, a systematic review of the literature highlighted papers with robust experimental functional analytic designs; providing appropriate methodology for the subsequent studies. The review also showed a trend towards an increase in the number of published papers that linked facets of the behavioural phenotype to challenging behaviour (gene-environment interactions). Next, the phenomenology and correlates of self-injurious and aggressive behaviour in the syndromes were explored at a given level of behavioural specificity. Results showed that self-injury was more common in Cornelia de Lange syndrome and specific forms of aggressive behaviour were common in Angelman syndrome. Experimental functional analysis and structured descriptive assessments were utilised to examine gene-environment interactions in the syndromes and broadly, challenging behaviour in the Cornelia de Lange syndrome group evidenced a stronger association with pain, whereas challenging behaviour in the Angelman syndrome group evidenced a stronger association with positive social reinforcement. Overall, the studies provide evidence that challenging behaviour in genetic syndromes can be influenced by environmental factors. Implications for practice and for informing a comprehensive model of challenging behaviour are discussed.

Type of Work:Ph.D. thesis.
Supervisor(s):Oliver, Christopher
School/Faculty:Colleges (2008 onwards) > College of Life & Environmental Sciences
Department:School of Psychology
Subjects:BF Psychology
Institution:University of Birmingham
Library Catalogue:Check for printed version of this thesis
ID Code:768
This unpublished thesis/dissertation is copyright of the author and/or third parties. The intellectual property rights of the author or third parties in respect of this work are as defined by The Copyright Designs and Patents Act 1988 or as modified by any successor legislation. Any use made of information contained in this thesis/dissertation must be in accordance with that legislation and must be properly acknowledged. Further distribution or reproduction in any format is prohibited without the permission of the copyright holder.
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