Executive function and decision making in Cornelia De Lange syndrome

Johnson, Victoria Ruth (2015). Executive function and decision making in Cornelia De Lange syndrome. University of Birmingham. Ph.D.

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Abstract

Executive functions are cognitive processes that are crucial for navigating the unpredictability of everyday life. Across two studies employing different methodologies, components of executive functioning were described in Cornelia de Lange syndrome, and compared to Fragile X (FXS) and Rubinstein-Taybi (RTS) syndromes and typically developing children. Significant differences in executive function impairments were found between CdLS, FXS and RTS, and the syndrome groups showed significant impairments relative to mental age expectations. Relationships between specific executive functions and repetitive behaviour differed in CdLS, RTS and FXS. Decision making, a skill underpinned by executive functioning was then tested experimentally using a novel decision making paradigm and compared across CdLS, FXS, RTS and typically developing children. Different decision making behaviour was observed between participant groups, and results suggested increased anxiety when working memory demands increased in CdLS. Finally, an interview study was conducted which implicates anticipatory anxiety in decision making difficulties, and differentiates between difficulties in different decision making scenarios. These findings further the understanding of executive function impairments, decision making difficulties and the relationships between them in CdLS, and highlight theoretical and clinical implications of these relationships.

Type of Work:

Thesis (Doctorates > Ph.D.)

Award Type:

Doctorates > Ph.D.

Supervisor(s):