Assessment of Executive Functions in <scp>P</scp>rader–<scp>W</scp>illi Syndrome and Relationship with Intellectual Level

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

doi:10.1111/jar.12044

Cited by 19 publications

(16 citation statements)

References 50 publications

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“…Studies with direct neuropsychological assessment tools reveal deficits in verbal fluency, set shifting, and visuoperceptual organization in adults with PWS (Jauregi et al, 2007;Chevalare et al, 2013). In other work with adults, Walley and Donaldson (2005) suggest the orbitofrontal cortex, an area implicated in emotional EF, might be involved in the behavior problems seen in adults with PWS.…”

Section: Introductionmentioning

confidence: 92%

Parental Ratings of Children and Adolescents With Prader-Willi Syndrome on the Behavior Rating Inventory of Executive Function (BRIEF)

Hutchison

Pei

Leung

et al. 2015

Journal of Mental Health Research in Intellectual Disabilities

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Section: Introductionmentioning

confidence: 92%

Parental Ratings of Children and Adolescents With Prader-Willi Syndrome on the Behavior Rating Inventory of Executive Function (BRIEF)

Hutchison

Pei

Leung

et al. 2015

Journal of Mental Health Research in Intellectual Disabilities

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“…In parallel with the eating problem, PWS patients have mild to moderate intellectual disability and behavioral alterations (Ho and Dimitropoulos, 2010; Chevalere et al, 2013), including emotional outbreaks (temper tantrums) and compulsive traits (Dimitropoulos et al, 2006). Repetitive and ritualistic behaviors and difficulty with routine changes (Holland et al, 2003; Greaves et al, 2006; Dykens et al, 2011), similar to those found in autistic spectrum conditions, have also been described.…”

Section: Ot and Developmental Neurological Disordersmentioning

confidence: 99%

Ontogenesis of oxytocin pathways in the mammalian brain: late maturation and psychosocial disorders

et al. 2015

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Oxytocin (OT), the main neuropeptide of sociality, is expressed in neurons exclusively localized in the hypothalamus. During the last decade, a plethora of neuroendocrine, metabolic, autonomic and behavioral effects of OT has been reported. In the urgency to find treatments to syndromes as invalidating as autism, many clinical trials have been launched in which OT is administered to patients, including adolescents and children. However, the impact of OT on the developing brain and in particular on the embryonic and early postnatal maturation of OT neurons, has been only poorly investigated. In the present review we summarize available (although limited) literature on general features of ontogenetic transformation of the OT system, including determination, migration and differentiation of OT neurons. Next, we discuss trajectories of OT receptors (OTR) in the perinatal period. Furthermore, we provide evidence that early alterations, from birth, in the central OT system lead to severe neurodevelopmental diseases such as feeding deficit in infancy and severe defects in social behavior in adulthood, as described in Prader-Willi syndrome (PWS). Our review intends to propose a hypothesis about developmental dynamics of central OT pathways, which are essential for survival right after birth and for the acquisition of social skills later on. A better understanding of the embryonic and early postnatal maturation of the OT system may lead to better OT-based treatments in PWS or autism.

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“…Executive functioning, task switching and resistance to change in individuals with Prader-Willi syndrome Executive functions are complex cognitive processes that monitor and regulate cognition and behaviour, particularly in novel and complex situations (Collette et al 2005). Individuals with PWS show deficits in executive function, particularly in task switching (Chevalère et al 2013(Chevalère et al , 2015Copet et al 2010;Hutchison et al 2015;Jauregi et al 2007;Woodcock et al 2009b;Woodcock et al 2010). Task switching is the ability to switch mental processes so as to deal appropriately with environmental demands (Monsell 2003).…”

Section: Resultsmentioning

confidence: 99%

Rigidity in routines and the development of resistance to change in individuals with Prader–Willi syndrome

Haig

Woodcock

2017

J intellect Disabil Res

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Because task switching appears to evidence a period of high developmental sensitivity during early primary school years, we propose that this period may represent a critical time when increasing flexibility in the routines of children with PWS could limit the development of resistance to change. However, a careful balance would need to be struck, given the apparent benefit of rigid routines on current behaviour. Further work in this area is much needed.

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Assessment of Executive Functions in Prader–Willi Syndrome and Relationship with Intellectual Level

Abstract: These data suggest a deficit of executive functioning in PWS that is linked more with verbal skills than performance skills. They also suggest that the impact on executive functioning may differ according to the genotype.

Cited by 19 publications

References 50 publications

Parental Ratings of Children and Adolescents With Prader-Willi Syndrome on the Behavior Rating Inventory of Executive Function (BRIEF)

Parental Ratings of Children and Adolescents With Prader-Willi Syndrome on the Behavior Rating Inventory of Executive Function (BRIEF)

Ontogenesis of oxytocin pathways in the mammalian brain: late maturation and psychosocial disorders

Rigidity in routines and the development of resistance to change in individuals with Prader–Willi syndrome

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