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We report a first study of brain activity linked to task switching in individuals with Prader-Willi syndrome (PWS). PWS individuals show a specific cognitive deficit in task switching which may be associated with the display of temper outbursts and repetitive questioning. The performance of participants with PWS and typically developing controls was matched in a cued task switching procedure, and brain activity was contrasted on switching and non-switching blocks using fMRI. Individuals with PWS did not show the typical frontal-parietal pattern of neural activity associated with switching blocks, with significantly reduced activation in regions of the posterior parietal and ventromedial prefrontal cortices. We suggest that this is linked to a difficulty in PWS in setting appropriate attentional weights to enable task-set reconfiguration. In addition to this, PWS individuals did not show the typical pattern of deactivation, with significantly less deactivation in an anterior region of the ventromedial prefrontal cortex. One plausible explanation for this is that individuals with PWS show dysfunction within the default mode network, which has been linked to attentional control. The data point to functional changes in the neural circuitry supporting task switching in PWS even when behavioural performance is matched to controls and thus highlight neural mechanisms that may be involved in a specific pathway between genes, cognition and behaviour.

Original publication

DOI

10.1016/j.brainres.2010.09.093

Type

Journal article

Journal

Brain Res

Publication Date

02/12/2010

Volume

1363

Pages

128 - 142

Keywords

Adolescent, Adult, Attention, Chromosome Deletion, Chromosomes, Human, Pair 15, Female, Humans, Male, Neural Pathways, Parietal Lobe, Prader-Willi Syndrome, Prefrontal Cortex, Psychomotor Performance, Young Adult