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Prader-Willi syndrome (PWS) and Fragile X syndrome (FraX) are associated with distinctive cognitive and behavioural profiles. We examined whether repetitive behaviours in the two syndromes were associated with deficits in specific executive functions. PWS, FraX, and typically developing (TD) children were assessed for executive functioning using the Test of Everyday Attention for Children and an adapted Simon spatial interference task. Relative to the TD children, children with PWS and FraX showed greater costs of attention switching on the Simon task, but after controlling for intellectual ability, these switching deficits were only significant in the PWS group. Children with PWS and FraX also showed significantly increased preference for routine and differing profiles of other specific types of repetitive behaviours. A measure of switch cost from the Simon task was positively correlated to scores on preference for routine questionnaire items and was strongly associated with scores on other items relating to a preference for predictability. It is proposed that a deficit in attention switching is a component of the endophenotypes of both PWS and FraX and is associated with specific behaviours. This proposal is discussed in the context of neurocognitive pathways between genes and behaviour.

Original publication




Journal article


Cogn Neuropsychol

Publication Date





172 - 194


Adolescent, Attention, Child, Child, Preschool, Chromosome Deletion, Chromosomes, Human, Pair 15, Cognition, Female, Fragile X Syndrome, Humans, Male, Neuropsychological Tests, Prader-Willi Syndrome, Stereotyped Behavior, Surveys and Questionnaires, Task Performance and Analysis, Wechsler Scales, Young Adult