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Study of the deficit in planning abilities of adults with Prader-Willi Syndrome

  • Background: Prader-Willi syndrome (PWS) is a complex developmental genetic disorder associated with intellectual disability and deficits in executive functions which result in disorganisation and poor personal autonomy. Aims: This study aimed to determine impairments in planning skills of adults with PWS, in relation with their intellectual disabilities, as well as the influence of food compulsions on their performance. Methods and procedures: A modified version of the Zoo Map from the Behavioural Assessment of the Dysexecutive Syndrome was used in three groups: a group of adults with PWS in comparison with two groups both matched on chronological age, one with typical development (TD) and one with intellectual disability (ID). Outcomes and results: Compared to TD adults, both adults with PWS and ID showed increased planning time and lower raw scores on the planning task. The execution time and the number of errors were higher in the PWS group compared to the comparison groups. All three groups performed worse inBackground: Prader-Willi syndrome (PWS) is a complex developmental genetic disorder associated with intellectual disability and deficits in executive functions which result in disorganisation and poor personal autonomy. Aims: This study aimed to determine impairments in planning skills of adults with PWS, in relation with their intellectual disabilities, as well as the influence of food compulsions on their performance. Methods and procedures: A modified version of the Zoo Map from the Behavioural Assessment of the Dysexecutive Syndrome was used in three groups: a group of adults with PWS in comparison with two groups both matched on chronological age, one with typical development (TD) and one with intellectual disability (ID). Outcomes and results: Compared to TD adults, both adults with PWS and ID showed increased planning time and lower raw scores on the planning task. The execution time and the number of errors were higher in the PWS group compared to the comparison groups. All three groups performed worse in the non-food condition only for number of errors and raw score. Conclusions and implications: Planning abilities were impaired in PWS adults. Results also showed that intellectual level plays a role in participants' performance. These findings are essential to understand the difficulties of people with PWS daily life.show moreshow less

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Author details:Séverine EstivalORCiD, Johann ChevalèreGND, Virginie LaurierORCiD, Fabien Mourre, Julie Tricot, Virginie PostalORCiD
DOI:https://doi.org/10.1016/j.ridd.2021.104056
ISSN:0891-4222
ISSN:1873-3379
Pubmed ID:https://pubmed.ncbi.nlm.nih.gov/34371306
Title of parent work (English):Research in developmental disabilities
Publisher:Elsevier
Place of publishing:Amsterdam [u.a.]
Publication type:Article
Language:English
Date of first publication:2021/08/06
Publication year:2021
Release date:2024/07/11
Tag:Cognitive profile; Intellectual disability; Planning; Prader-Willi Syndrome
Volume:117
Article number:104056
Number of pages:10
Funding institution:Fondation Maladies Rares
Organizational units:Humanwissenschaftliche Fakultät / Strukturbereich Bildungswissenschaften / Department Erziehungswissenschaft
DDC classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Peer review:Referiert
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