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The relationship between a deficit in attention switching and specific behaviours in Prader‐Willi syndrome
Author(s) -
Woodcock K. A.,
Oliver C.,
Humphreys G. W.
Publication year - 2008
Publication title -
journal of intellectual disability research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.941
H-Index - 104
eISSN - 1365-2788
pISSN - 0964-2633
DOI - 10.1111/j.1365-2788.2008.01119_1.x
Subject(s) - arousal , audiology , psychology , typically developing , developmental psychology , cognition , attention deficit , attention deficit hyperactivity disorder , medicine , clinical psychology , psychiatry , neuroscience , autism
Background : We aimed to investigate the causes of phenotypic behaviours in Prader‐Willi syndrome (PWS). Interviews with carers of 46 children with PWS suggested that frequent temper outbursts and repetitive questions were related to a resistance to change. In a group comparison with FraX and typically developing children, PWS children showed a specific deficit in attention switching (direct assessment), which was correlated to informant scores of resistance to change and repetitive questions. We aimed to investigate neural correlates and hypothesized behavioural effects of the PWS deficit in attention switching. Method : Multiple single case experimental designs were employed with four individuals with PWS. Cognitive challenges placed demands on attention switching or controlled for these demands. Changes to routines or expectations were presented during controlled games, or imposed on participant’s natural environments, and compared to corresponding sessions when no changes were imposed. Behaviour was recorded and physiological arousal was estimated using heart rate measurements. Eight participants with PWS were compared to age and gender matched controls. Structural T1, T2 and diffusion weighted images were acquired in a Philips T3 MRI scanner. T2* weighted functional images were acquired in a block design (eight presentations of each block per participant) with alternating switching and non‐switching blocks. Results : In the single case experiments, significantly increased temper outburst related behaviours (p < 0.05) and arousal (decreased heart rate variability: p = 0.042) were shown in switching verses control challenges. Significantly increased temper outburst related behaviours were also shown when changes were imposed versus when no changes occurred (p < 0.03). Structural images suggest abnormalities in specific frontal regions in individuals with PWS and functional images revealed significant differences in switch‐related activation in individuals with PWS compared to controls. Conclusion : We propose a directional relationship between specific neural abnormalities, cognitive deficit and behaviour in PWS via particular environmental and physiological interactions.