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A neuropsychological assessment of frontal cognitive functions in Prader-Willi syndrome

Background  Prader–Willi syndrome (PWS) is associated with a characteristic behavioural phenotype whose main features are, alongside compulsive hyperphagia, deficits in social behaviour: social withdrawal, temper tantrums, perseverative speech and behaviour, mental rigidity, stereotyped behaviour, i...

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Published in:Journal of intellectual disability research 2007-05, Vol.51 (5), p.350-365
Main Authors: Jauregi, J., Arias, C., Vegas, O., Alén, F., Martinez, S., Copet, P., Thuilleaux, D.
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Arias, C.
Vegas, O.
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Copet, P.
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description Background  Prader–Willi syndrome (PWS) is associated with a characteristic behavioural phenotype whose main features are, alongside compulsive hyperphagia, deficits in social behaviour: social withdrawal, temper tantrums, perseverative speech and behaviour, mental rigidity, stereotyped behaviour, impulsiveness, etc. Similar symptoms may also be found in autistic spectrum disorders and lesional pathologies of the frontal lobe. In both cases, such symptoms have been related to dysfunctions in frontal cognitive processes such as attention, working memory and executive functions. This study uses standardized neuropsychological instruments to analyse the degree to which these processes are affected in PWS. Methods  The sample comprised 16 individuals with a genetically confirmed PWS diagnosis. Subjects’ IQ (Wechsler Adult Intelligence Scale), academic level, laterality and body mass index (BMI) were calculated. Attention, memory and executive functions were analysed using standard, widely employed neuropsychological tests. We compared the results of the sample group with the general population. Correlation analyses were carried out with IQ, academic level and BMI. Results  In all the neuropsychological measures focusing on attention, executive functions and visuoperceptual organization, the study sample scored significantly lower than the normative reference population. The scores of the tests used for measuring immediate memory were also significantly lower when trials required sequential processing, although not when they required simultaneous processing. In the memorization of a list of words, subjects showed an initial deficit which disappeared with repetition, enabling them to obtain scores similar to the reference population. No significant correlations were found with BMI, and a higher IQ or academic level did not improve scores in the majority of tests. Conclusions  The study shows a deficit in elementary frontal cognitive processes in PWS patients. This deficit may be involved in the social behaviour disorders that characterize such patients, as described in other development or frontal syndrome pathologies. However, we cannot affirm that the deficits found are specific to PWS; they could also occur in other causes of intellectual disability. Although in the study sample IQ did not correlate with frontal deficits, further research is needed to establish whether the neuropsychological alterations described form part of a cognitive phenotype for PWS. We
doi_str_mv 10.1111/j.1365-2788.2006.00883.x
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In both cases, such symptoms have been related to dysfunctions in frontal cognitive processes such as attention, working memory and executive functions. This study uses standardized neuropsychological instruments to analyse the degree to which these processes are affected in PWS. Methods  The sample comprised 16 individuals with a genetically confirmed PWS diagnosis. Subjects’ IQ (Wechsler Adult Intelligence Scale), academic level, laterality and body mass index (BMI) were calculated. Attention, memory and executive functions were analysed using standard, widely employed neuropsychological tests. We compared the results of the sample group with the general population. Correlation analyses were carried out with IQ, academic level and BMI. Results  In all the neuropsychological measures focusing on attention, executive functions and visuoperceptual organization, the study sample scored significantly lower than the normative reference population. The scores of the tests used for measuring immediate memory were also significantly lower when trials required sequential processing, although not when they required simultaneous processing. In the memorization of a list of words, subjects showed an initial deficit which disappeared with repetition, enabling them to obtain scores similar to the reference population. No significant correlations were found with BMI, and a higher IQ or academic level did not improve scores in the majority of tests. Conclusions  The study shows a deficit in elementary frontal cognitive processes in PWS patients. This deficit may be involved in the social behaviour disorders that characterize such patients, as described in other development or frontal syndrome pathologies. However, we cannot affirm that the deficits found are specific to PWS; they could also occur in other causes of intellectual disability. Although in the study sample IQ did not correlate with frontal deficits, further research is needed to establish whether the neuropsychological alterations described form part of a cognitive phenotype for PWS. We believe that our understanding of the social behaviours typical of PWS may be improved by taking into consideration the cognitive functioning models of the prefrontal lobe, particularly those applied to pervasive developmental disorders.</description><identifier>ISSN: 0964-2633</identifier><identifier>EISSN: 1365-2788</identifier><identifier>DOI: 10.1111/j.1365-2788.2006.00883.x</identifier><identifier>PMID: 17391252</identifier><identifier>CODEN: JIDREN</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Publishing Ltd</publisher><subject>Achievement ; Adolescent ; Adult ; Adult and adolescent clinical studies ; Attention ; Autism ; Behavior disorders ; Behavior Patterns ; Behavior Problems ; Biological and medical sciences ; Body Composition ; Body Mass Index ; Cognition &amp; reasoning ; Cognition Disorders - diagnosis ; Cognition Disorders - epidemiology ; Cognition Disorders - physiopathology ; Cognitive Ability ; Cognitive Processes ; cognitive profile ; Comparative Analysis ; Complex syndromes ; Developmental disabilities ; Evaluation Methods ; Executive Function ; Female ; frontal functions ; Frontal Lobe - physiopathology ; Genetic disorders ; Genetics ; Humans ; Intelligence ; Intelligence Quotient ; Male ; Medical genetics ; Medical sciences ; Memory ; Memory Disorders - diagnosis ; Memory Disorders - epidemiology ; Memory Disorders - physiopathology ; Mental Retardation ; Middle Aged ; neuropsychological assessment ; Neuropsychological Tests ; Neuropsychology ; Organic mental disorders. Neuropsychology ; Patients ; Personality Traits ; Pervasive Developmental Disorders ; Phenotypes ; Prader-Willi syndrome ; Prader-Willi Syndrome - epidemiology ; Prader-Willi Syndrome - genetics ; Psychology. Psychoanalysis. Psychiatry ; Psychopathology. Psychiatry ; Semantics ; Severity of Illness Index ; Short Term Memory ; Social Behavior ; Social behaviour ; social cognition ; Symptoms (Individual Disorders) ; Wechsler Adult Intelligence Scale ; Withdrawal (Psychology)</subject><ispartof>Journal of intellectual disability research, 2007-05, Vol.51 (5), p.350-365</ispartof><rights>2007 INIST-CNRS</rights><rights>Copyright Blackwell Publishing May 2007</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c5143-4e4b8d26363946bfdd770c31bccb95fae23bdd2c7ab098d101c61e0f25d9f0f3</citedby><cites>FETCH-LOGICAL-c5143-4e4b8d26363946bfdd770c31bccb95fae23bdd2c7ab098d101c61e0f25d9f0f3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925,30999,31000</link.rule.ids><backlink>$$Uhttp://eric.ed.gov/ERICWebPortal/detail?accno=EJ756837$$DView record in ERIC$$Hfree_for_read</backlink><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&amp;idt=18633044$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/17391252$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Jauregi, J.</creatorcontrib><creatorcontrib>Arias, C.</creatorcontrib><creatorcontrib>Vegas, O.</creatorcontrib><creatorcontrib>Alén, F.</creatorcontrib><creatorcontrib>Martinez, S.</creatorcontrib><creatorcontrib>Copet, P.</creatorcontrib><creatorcontrib>Thuilleaux, D.</creatorcontrib><title>A neuropsychological assessment of frontal cognitive functions in Prader-Willi syndrome</title><title>Journal of intellectual disability research</title><addtitle>J Intellect Disabil Res</addtitle><description>Background  Prader–Willi syndrome (PWS) is associated with a characteristic behavioural phenotype whose main features are, alongside compulsive hyperphagia, deficits in social behaviour: social withdrawal, temper tantrums, perseverative speech and behaviour, mental rigidity, stereotyped behaviour, impulsiveness, etc. Similar symptoms may also be found in autistic spectrum disorders and lesional pathologies of the frontal lobe. In both cases, such symptoms have been related to dysfunctions in frontal cognitive processes such as attention, working memory and executive functions. This study uses standardized neuropsychological instruments to analyse the degree to which these processes are affected in PWS. Methods  The sample comprised 16 individuals with a genetically confirmed PWS diagnosis. Subjects’ IQ (Wechsler Adult Intelligence Scale), academic level, laterality and body mass index (BMI) were calculated. Attention, memory and executive functions were analysed using standard, widely employed neuropsychological tests. We compared the results of the sample group with the general population. Correlation analyses were carried out with IQ, academic level and BMI. Results  In all the neuropsychological measures focusing on attention, executive functions and visuoperceptual organization, the study sample scored significantly lower than the normative reference population. The scores of the tests used for measuring immediate memory were also significantly lower when trials required sequential processing, although not when they required simultaneous processing. In the memorization of a list of words, subjects showed an initial deficit which disappeared with repetition, enabling them to obtain scores similar to the reference population. No significant correlations were found with BMI, and a higher IQ or academic level did not improve scores in the majority of tests. Conclusions  The study shows a deficit in elementary frontal cognitive processes in PWS patients. This deficit may be involved in the social behaviour disorders that characterize such patients, as described in other development or frontal syndrome pathologies. However, we cannot affirm that the deficits found are specific to PWS; they could also occur in other causes of intellectual disability. Although in the study sample IQ did not correlate with frontal deficits, further research is needed to establish whether the neuropsychological alterations described form part of a cognitive phenotype for PWS. We believe that our understanding of the social behaviours typical of PWS may be improved by taking into consideration the cognitive functioning models of the prefrontal lobe, particularly those applied to pervasive developmental disorders.</description><subject>Achievement</subject><subject>Adolescent</subject><subject>Adult</subject><subject>Adult and adolescent clinical studies</subject><subject>Attention</subject><subject>Autism</subject><subject>Behavior disorders</subject><subject>Behavior Patterns</subject><subject>Behavior Problems</subject><subject>Biological and medical sciences</subject><subject>Body Composition</subject><subject>Body Mass Index</subject><subject>Cognition &amp; reasoning</subject><subject>Cognition Disorders - diagnosis</subject><subject>Cognition Disorders - epidemiology</subject><subject>Cognition Disorders - physiopathology</subject><subject>Cognitive Ability</subject><subject>Cognitive Processes</subject><subject>cognitive profile</subject><subject>Comparative Analysis</subject><subject>Complex syndromes</subject><subject>Developmental disabilities</subject><subject>Evaluation Methods</subject><subject>Executive Function</subject><subject>Female</subject><subject>frontal functions</subject><subject>Frontal Lobe - physiopathology</subject><subject>Genetic disorders</subject><subject>Genetics</subject><subject>Humans</subject><subject>Intelligence</subject><subject>Intelligence Quotient</subject><subject>Male</subject><subject>Medical genetics</subject><subject>Medical sciences</subject><subject>Memory</subject><subject>Memory Disorders - diagnosis</subject><subject>Memory Disorders - epidemiology</subject><subject>Memory Disorders - physiopathology</subject><subject>Mental Retardation</subject><subject>Middle Aged</subject><subject>neuropsychological assessment</subject><subject>Neuropsychological Tests</subject><subject>Neuropsychology</subject><subject>Organic mental disorders. 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Psychiatry</subject><subject>Semantics</subject><subject>Severity of Illness Index</subject><subject>Short Term Memory</subject><subject>Social Behavior</subject><subject>Social behaviour</subject><subject>social cognition</subject><subject>Symptoms (Individual Disorders)</subject><subject>Wechsler Adult Intelligence Scale</subject><subject>Withdrawal (Psychology)</subject><issn>0964-2633</issn><issn>1365-2788</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2007</creationdate><recordtype>article</recordtype><sourceid>7SW</sourceid><sourceid>7QJ</sourceid><recordid>eNqNkV1v0zAUhiMEYt3gHyAUITGuEvyR2LHEzVRGt2kCNFWUO8vxx3BJ7GIn0P57HFp1EhcI39jyed6jYz9ZlkNQwrTerkuISV0g2jQlAoCUADQNLrePstmx8DibAUaqAhGMT7LTGNcgkbAiT7MTSDGDqEazbHWROz0Gv4k7-c13_t5K0eUiRh1jr92Qe5Ob4N2QbqW_d3awP3VuRicH613Mrcs_B6F0KFa262wed04F3-tn2RMjuqifH_azbPnhcjm_Km4_La7nF7eFrGGFi0pXbaPSiASzirRGKUqBxLCVsmW1ERrhVikkqWgBaxQEUBKogUG1YgYYfJa92bfdBP9j1HHgvY1Sd51w2o-RM8gYTZ9DE3n-T5ICjDCscQJf_QWu_RhcegRHqCEIIzZBzR6SwccYtOGbYHsRdhwCPiniaz6Z4JMJPinifxTxbYq-PPQf216rh-DBSQJeHwARkwwThJM2PnBNEgqqKnEv9pwOVh7Llze0Jg2eHvxuX_5lO7377_n4zfVdOqR4sY_bOOjtMS7Cd04opjVffVzw5Xv09Wq-uONf8G8_ucVW</recordid><startdate>200705</startdate><enddate>200705</enddate><creator>Jauregi, J.</creator><creator>Arias, C.</creator><creator>Vegas, O.</creator><creator>Alén, F.</creator><creator>Martinez, S.</creator><creator>Copet, P.</creator><creator>Thuilleaux, D.</creator><general>Blackwell Publishing Ltd</general><general>Blackwell Publishing</general><general>Blackwell</general><general>Wiley Subscription Services, Inc</general><scope>BSCLL</scope><scope>7SW</scope><scope>BJH</scope><scope>BNH</scope><scope>BNI</scope><scope>BNJ</scope><scope>BNO</scope><scope>ERI</scope><scope>PET</scope><scope>REK</scope><scope>WWN</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7QJ</scope><scope>7X8</scope></search><sort><creationdate>200705</creationdate><title>A neuropsychological assessment of frontal cognitive functions in Prader-Willi syndrome</title><author>Jauregi, J. ; Arias, C. ; Vegas, O. ; Alén, F. ; Martinez, S. ; Copet, P. ; Thuilleaux, D.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5143-4e4b8d26363946bfdd770c31bccb95fae23bdd2c7ab098d101c61e0f25d9f0f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2007</creationdate><topic>Achievement</topic><topic>Adolescent</topic><topic>Adult</topic><topic>Adult and adolescent clinical studies</topic><topic>Attention</topic><topic>Autism</topic><topic>Behavior disorders</topic><topic>Behavior Patterns</topic><topic>Behavior Problems</topic><topic>Biological and medical sciences</topic><topic>Body Composition</topic><topic>Body Mass Index</topic><topic>Cognition &amp; reasoning</topic><topic>Cognition Disorders - diagnosis</topic><topic>Cognition Disorders - epidemiology</topic><topic>Cognition Disorders - physiopathology</topic><topic>Cognitive Ability</topic><topic>Cognitive Processes</topic><topic>cognitive profile</topic><topic>Comparative Analysis</topic><topic>Complex syndromes</topic><topic>Developmental disabilities</topic><topic>Evaluation Methods</topic><topic>Executive Function</topic><topic>Female</topic><topic>frontal functions</topic><topic>Frontal Lobe - physiopathology</topic><topic>Genetic disorders</topic><topic>Genetics</topic><topic>Humans</topic><topic>Intelligence</topic><topic>Intelligence Quotient</topic><topic>Male</topic><topic>Medical genetics</topic><topic>Medical sciences</topic><topic>Memory</topic><topic>Memory Disorders - diagnosis</topic><topic>Memory Disorders - epidemiology</topic><topic>Memory Disorders - physiopathology</topic><topic>Mental Retardation</topic><topic>Middle Aged</topic><topic>neuropsychological assessment</topic><topic>Neuropsychological Tests</topic><topic>Neuropsychology</topic><topic>Organic mental disorders. Neuropsychology</topic><topic>Patients</topic><topic>Personality Traits</topic><topic>Pervasive Developmental Disorders</topic><topic>Phenotypes</topic><topic>Prader-Willi syndrome</topic><topic>Prader-Willi Syndrome - epidemiology</topic><topic>Prader-Willi Syndrome - genetics</topic><topic>Psychology. Psychoanalysis. Psychiatry</topic><topic>Psychopathology. Psychiatry</topic><topic>Semantics</topic><topic>Severity of Illness Index</topic><topic>Short Term Memory</topic><topic>Social Behavior</topic><topic>Social behaviour</topic><topic>social cognition</topic><topic>Symptoms (Individual Disorders)</topic><topic>Wechsler Adult Intelligence Scale</topic><topic>Withdrawal (Psychology)</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Jauregi, J.</creatorcontrib><creatorcontrib>Arias, C.</creatorcontrib><creatorcontrib>Vegas, O.</creatorcontrib><creatorcontrib>Alén, F.</creatorcontrib><creatorcontrib>Martinez, S.</creatorcontrib><creatorcontrib>Copet, P.</creatorcontrib><creatorcontrib>Thuilleaux, D.</creatorcontrib><collection>Istex</collection><collection>ERIC</collection><collection>ERIC (Ovid)</collection><collection>ERIC</collection><collection>ERIC</collection><collection>ERIC (Legacy Platform)</collection><collection>ERIC( SilverPlatter )</collection><collection>ERIC</collection><collection>ERIC PlusText (Legacy Platform)</collection><collection>Education Resources Information Center (ERIC)</collection><collection>ERIC</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Applied Social Sciences Index &amp; Abstracts (ASSIA)</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of intellectual disability research</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Jauregi, J.</au><au>Arias, C.</au><au>Vegas, O.</au><au>Alén, F.</au><au>Martinez, S.</au><au>Copet, P.</au><au>Thuilleaux, D.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><ericid>EJ756837</ericid><atitle>A neuropsychological assessment of frontal cognitive functions in Prader-Willi syndrome</atitle><jtitle>Journal of intellectual disability research</jtitle><addtitle>J Intellect Disabil Res</addtitle><date>2007-05</date><risdate>2007</risdate><volume>51</volume><issue>5</issue><spage>350</spage><epage>365</epage><pages>350-365</pages><issn>0964-2633</issn><eissn>1365-2788</eissn><coden>JIDREN</coden><abstract>Background  Prader–Willi syndrome (PWS) is associated with a characteristic behavioural phenotype whose main features are, alongside compulsive hyperphagia, deficits in social behaviour: social withdrawal, temper tantrums, perseverative speech and behaviour, mental rigidity, stereotyped behaviour, impulsiveness, etc. Similar symptoms may also be found in autistic spectrum disorders and lesional pathologies of the frontal lobe. In both cases, such symptoms have been related to dysfunctions in frontal cognitive processes such as attention, working memory and executive functions. This study uses standardized neuropsychological instruments to analyse the degree to which these processes are affected in PWS. Methods  The sample comprised 16 individuals with a genetically confirmed PWS diagnosis. Subjects’ IQ (Wechsler Adult Intelligence Scale), academic level, laterality and body mass index (BMI) were calculated. Attention, memory and executive functions were analysed using standard, widely employed neuropsychological tests. We compared the results of the sample group with the general population. Correlation analyses were carried out with IQ, academic level and BMI. Results  In all the neuropsychological measures focusing on attention, executive functions and visuoperceptual organization, the study sample scored significantly lower than the normative reference population. The scores of the tests used for measuring immediate memory were also significantly lower when trials required sequential processing, although not when they required simultaneous processing. In the memorization of a list of words, subjects showed an initial deficit which disappeared with repetition, enabling them to obtain scores similar to the reference population. No significant correlations were found with BMI, and a higher IQ or academic level did not improve scores in the majority of tests. Conclusions  The study shows a deficit in elementary frontal cognitive processes in PWS patients. This deficit may be involved in the social behaviour disorders that characterize such patients, as described in other development or frontal syndrome pathologies. However, we cannot affirm that the deficits found are specific to PWS; they could also occur in other causes of intellectual disability. Although in the study sample IQ did not correlate with frontal deficits, further research is needed to establish whether the neuropsychological alterations described form part of a cognitive phenotype for PWS. We believe that our understanding of the social behaviours typical of PWS may be improved by taking into consideration the cognitive functioning models of the prefrontal lobe, particularly those applied to pervasive developmental disorders.</abstract><cop>Oxford, UK</cop><pub>Blackwell Publishing Ltd</pub><pmid>17391252</pmid><doi>10.1111/j.1365-2788.2006.00883.x</doi><tpages>16</tpages></addata></record>
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source Applied Social Sciences Index & Abstracts (ASSIA); Wiley-Blackwell Read & Publish Collection; ERIC
subjects Achievement
Adolescent
Adult
Adult and adolescent clinical studies
Attention
Autism
Behavior disorders
Behavior Patterns
Behavior Problems
Biological and medical sciences
Body Composition
Body Mass Index
Cognition & reasoning
Cognition Disorders - diagnosis
Cognition Disorders - epidemiology
Cognition Disorders - physiopathology
Cognitive Ability
Cognitive Processes
cognitive profile
Comparative Analysis
Complex syndromes
Developmental disabilities
Evaluation Methods
Executive Function
Female
frontal functions
Frontal Lobe - physiopathology
Genetic disorders
Genetics
Humans
Intelligence
Intelligence Quotient
Male
Medical genetics
Medical sciences
Memory
Memory Disorders - diagnosis
Memory Disorders - epidemiology
Memory Disorders - physiopathology
Mental Retardation
Middle Aged
neuropsychological assessment
Neuropsychological Tests
Neuropsychology
Organic mental disorders. Neuropsychology
Patients
Personality Traits
Pervasive Developmental Disorders
Phenotypes
Prader-Willi syndrome
Prader-Willi Syndrome - epidemiology
Prader-Willi Syndrome - genetics
Psychology. Psychoanalysis. Psychiatry
Psychopathology. Psychiatry
Semantics
Severity of Illness Index
Short Term Memory
Social Behavior
Social behaviour
social cognition
Symptoms (Individual Disorders)
Wechsler Adult Intelligence Scale
Withdrawal (Psychology)
title A neuropsychological assessment of frontal cognitive functions in Prader-Willi syndrome
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