Decision making and executive function in Cornelia de Lange syndrome
Funded by the Cornelia de Lange Syndrome Foundation (UK and Ireland) from 2011 – 2014.
Recent research has suggested that there is an increase in behavioural difficulties in people with CdLS as they enter late adolescence and early adulthood (Basile, Villa, Selicorni & Molteni, 2007). More specifically, this manifests in a decrease in mood, interest and pleasure, an increase in preference for routine and sameness and an increase in challenging behaviour. It has been suggested that a decline in cognitive skills such as executive function occurs concurrently with the increase in behavioural difficulties and age (Reid, 2010). One area of executive function that has not been fully investigated in CdLS is decision making. Anecdotal reports suggest that people with CdLS particularly struggle with decision making, which is a fundamental skill that needs to be employed in everyday tasks. This difficulty becomes especially problematic when one considers the drive towards a ‘freedom of choice’ environment in most residential and educational facilities. If a person has a difficulty making decisions, they may find the ‘freedom of choice’ aspect of these facilities to be overwhelming and struggle to participate in activities at all, as it requires decision making to do so. Therefore, research is needed to create a profile of executive function in CdLS and to develop a developmental trajectory for executive function skills and how this links to behavioural difficulties. In addition, there is a need to evaluate decision making skills in people with CdLS and how this impacts on their quality of life.
The main aims of my PhD are to investigate the nature and developmental trajectory of executive function skills in Cornelia de Lange syndrome and how this relates to repetitive behaviour and decision making. I am also examining the impact of impaired executive function on quality of life, and am hoping to test strategies to reduce this impact.