TY  - JOUR
A1  - Estival, Séverine
A1  - Chevalère, Johann
A1  - Laurier, Virginie
A1  - Mourre, Fabien
A1  - Tricot, Julie
A1  - Postal, Virginie
T1  - Study of the deficit in planning abilities of adults with Prader-Willi Syndrome
JF  - Research in developmental disabilities
N2  - 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 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.
KW  - Cognitive profile
KW  - Intellectual disability
KW  - Planning
KW  - Prader-Willi Syndrome
Y1  - 2021
U6  - https://doi.org/10.1016/j.ridd.2021.104056
SN  - 0891-4222
SN  - 1873-3379
VL  - 117
PB  - Elsevier
CY  - Amsterdam [u.a.]
ER  -