Coffin-Siris syndrome (CSS) is a rare genetic disorder presenting physical abnormalities and cognitive developmental challenges. Research into cognitive and developmental aspects of CSS could shed...Show moreCoffin-Siris syndrome (CSS) is a rare genetic disorder presenting physical abnormalities and cognitive developmental challenges. Research into cognitive and developmental aspects of CSS could shed light on the development of emotional and behavioural problems which could contribute to early detection and preventive measures. This study investigates core executive functions (Behaviour Rating Inventory of Executive Functions) and emotional and behavioural problems (Child Behavioural Checklist) in 15 CSS children aged 3-18 years. Core executive functions are compared between 12 children with CSS children and 26 TD children matched on mental age. Additionally, this study delves into the relationship between core executive functions and emotional and behavioural problems. Children with CSS exhibited attention deficit/hyperactivity, anxiety and oppositional defiant problems. The majority of children with CSS also exhibited problems in cognitive flexibility and working memory. However, inhibition problems were in line with TD children. Children with CSS displayed equal levels of inhibition and working memory problems compared to their peers with a similar developmental level but showed increased levels of cognitive flexibility problems. Findings suggest that there is no correlation between total executive functioning and emotional and behavioural problems in children with CSS. However, a relationship was found between inhibition and overall emotional and behavioural problems, and specifically attention deficit/hyperactivity. Additional investigations are necessary to conduct comprehensive assessments of executive functions in children with CSS, particularly considering their intellectual development. Additionally, recommendations for future research are provided to further explore the connection between executive functions and emotional and behavioural problems in children with CSS. These findings provide first insight into executive functions in children with CSS and their relationship with emotional and behavioural problems. These findings may aid the development of tailored interventions aimed at improving adaptive functioning, reduce emotional and behavioural problems, and enhance quality of life.Show less
ABSTRACT This thesis examined the possibility of a relationship between exploratory behaviour and executive functions (i.e. cognitive development) in 137 children (76 boys and 61 girls (Mpre = 5.3...Show moreABSTRACT This thesis examined the possibility of a relationship between exploratory behaviour and executive functions (i.e. cognitive development) in 137 children (76 boys and 61 girls (Mpre = 5.3 years; SD = .6; Mpost = 6.3 years; SD = .6)). Additionally, the effect of an intervention, aimed at improving children’s executive functioning, on their exploratory behaviour was examined using a pretest posttest intervention design. This question was tested on a sample of 247 children containing 137 boys and 110 girls (Mpre = 5.3 years; SD = .6; Mpost = 6.3 years; SD = .6). Parents were randomly assigned to the intervention- or control group. Parents of forty children were trained on how to stimulate their children’s social- and cognitive functioning, with a major focus on executive functioning, and parents of 207 children were not. Executive functions are known to be trainable and believed to be related to the development of exploratory behaviour. Playhouse, a newly developed task, assesses the quality of exploration. The executive functions; inhibition, working memory and attention, were measured with the Amsterdam Psychological Tasks. The results suggest that a relationship between children’s executive functioning and their quality of exploratory behaviour exists. However, these relationships had a small effect. Additionally, it was found that children’s executive functioning and quality of exploratory behaviour depend on their age. However, it remains unclear whether the improved executive functions with increasing age affect the increasing quality of exploratory behaviour with increasing age. Furthermore, the results in this study suggest that training parents to stimulate their children’s social- and cognitive functioning, with a major focus on executive functioning does not affect their quality of exploratory behaviour. Further research in this area is necessary in order to get a more accurate understanding of the relationship between children’s executive functioning and their explorative behaviour, and the psychometric properties of Playhouse.Show less
