Executive Functions and Prader-Willi Syndrome: Global Deficit Linked With Intellectual Level and Syndrome-Specific Associations

Chevalère, Johann; Postal, Virginie; Jauregui, J; Copet, P.; Laurier, Virginie; Thuilleaux, Denise

doi:10.1352/1944-7558-120.3.215

Cited by 30 publications

(28 citation statements)

References 40 publications

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“…PWS patients display learning difficulties especially in observational learning 46 , 47 . A deficit in executive function has been observed with impaired mental switching 30 , 48 , and impaired inhibition capacities have also been noted 49 . Concerning visual perception, some authors have argued that patient with PWS display higher skills to detect similarities which possibly explain their high performance in puzzle processing 45 .…”

Section: Rdoc Approach and Pwsmentioning

confidence: 99%

The RDoC approach for translational psychiatry: Could a genetic disorder with psychiatric symptoms help fill the matrix? the example of Prader–Willi syndrome

et al. 2020

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The Research Domain Criteria project (RDoc) proposes a new classification system based on information from several fields in order to encourage translational perspectives. Nevertheless, integrating genetic markers into this classification has remained difficult because of the lack of powerful associations between targeted genes and RDoC domains. We hypothesized that genetic diseases with psychiatric manifestations would be good models for RDoC gene investigations and would thereby extend the translational approach to involve targeted gene pathways. To explore this possibility, we reviewed the current knowledge on Prader–Willi syndrome, a genetic disorder caused by the absence of expression of some of the genes of the chromosome 15q11–13 region inherited from the father. Indeed, we found that the associations between genes of the PW locus and the modification identified in the relevant behavioral, physiological, and brain imaging studies followed the structure of the RDoC matrix and its six domains (positive valence, negative valence, social processing, cognitive systems, arousal/regulatory systems, and sensorimotor systems).

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Section: Rdoc Approach and Pwsmentioning

confidence: 99%

The RDoC approach for translational psychiatry: Could a genetic disorder with psychiatric symptoms help fill the matrix? the example of Prader–Willi syndrome

et al. 2020

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“…This difficulty with change of routine and inflexibility in PWS may be linked to a cognitive deficit in task switching [ 56 , 57 ]. Task switching requires being able to move from one task to a different task in a timely manner, an area of difficulty for individuals with PWS [ 56 , 86 ].…”

Section: Rigidity In Pwsmentioning

confidence: 99%

Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium

Schwartz

Caixàs

Dimitropoulos

et al. 2021

J Neurodevelop Disord

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Prader-Willi syndrome (PWS) is a rare neurodevelopmental genetic disorder associated with a characteristic behavioral phenotype that includes severe hyperphagia and a variety of other behavioral challenges such as temper outbursts and anxiety. These behaviors have a significant and dramatic impact on the daily functioning and quality of life for the person with PWS and their families. To date, effective therapies addressing these behavioral challenges have proven elusive, but several potential treatments are on the horizon. However, a limiting factor for treatment studies in PWS is the lack of consensus in the field regarding how to best define and measure the complex and interrelated behavioral features of this syndrome. The International PWS Clinical Trials Consortium (PWS-CTC, www.pwsctc.org) includes expert PWS scientists, clinicians, and patient advocacy organization representatives focused on facilitating clinical trials in this rare disease. To address the above gap in the field, members of the PWS-CTC “Behavior Outcomes Working Group” sought to develop a unified understanding of the key behavioral features in PWS and build a consensus regarding their definition and description. The primary focus of this paper is to present consensus definitions and descriptions of key phenotypic PWS behaviors including hyperphagia, temper outbursts, anxiety, obsessive–compulsive behaviors, rigidity, and social cognition deficits. Patient vignettes are provided to illustrate the interrelatedness and impact of these behaviors. We also review some available assessment tools as well as new instruments in development which may be useful in measuring these behavioral features in PWS.

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“…Based on the results of research that has been done, it is found that the predictive power of intellectual ability test scores on students' learning outcomes in the field of theory> students' learning outcomes in the field of practice.> This is because the learning of art in the field of theory is more the process of cognitive thinking [12] (knowledge), understanding of theories about art. This opinion is reinforced [2] by argument that, the term intellectual is defined as cognitive processes, thought processes, connecting power, ability to judge, and ability to consider.…”

Section: Resultsmentioning

confidence: 99%

Predictive Power of Intellectual Ability Test Score on Students’ Fine Art Learning Outcomes

Lubis¹,

Retnowati²,

Syawalina³

2020

Proceedings of the 3rd International Conference on Arts and Arts Education (ICAAE 2019)

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This study aims to determine the magnitude of the predictive power of intellectual ability test scores on Fine Art students' learning achievements. Intellectual ability test is part of a psychological test conducted by State Islamic Senior High School MAN 2 Padangsidimpuan as one of the selection instruments when students enroll at the school. The feedback on the intellectual ability consists of general intelligence, comprehension, spatial, and synthesis analysis, while the Fine Art students' learning achievements comprise: (1) students' theory-based learning achievements, (2) practice-based learning achievements, and (3) theory and practicebased learning achievements. This study was expo facto research. The population was all students of State Islamic Senior High School MAN 2 Padangsidimpuan class XI and the researchers employed a simple random sampling technique. The data were obtained from the results of psychological tests conducted by the Psychology Bureau Marsha Puntadewa and Fine Art students' learning achievements. The techniques used to analyze the data were a descriptive analysis, correlation, and simple linear regression. The results of the data analysis shows that the amount of the predictive power of: (1) the intellectual ability test scores on students' theory-based learning achievements is 83%, (2) the intellectual ability test scores on students' practice-based learning achievements is 75.5%, and (3) the intellectual ability test scores on students' practice and theorybased learning achievements is 85.4%.

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Executive Functions and Prader-Willi Syndrome: Global Deficit Linked With Intellectual Level and Syndrome-Specific Associations

Cited by 30 publications

References 40 publications

The RDoC approach for translational psychiatry: Could a genetic disorder with psychiatric symptoms help fill the matrix? the example of Prader–Willi syndrome

The RDoC approach for translational psychiatry: Could a genetic disorder with psychiatric symptoms help fill the matrix? the example of Prader–Willi syndrome

Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium

Predictive Power of Intellectual Ability Test Score on Students’ Fine Art Learning Outcomes

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