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

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.EThOS - Electronic Theses Online ServiceGBUnited Kingdo