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Résultat de la recherche
67 recherche sur le mot-clé 'Williams syndrome'




Addressing the Educational Needs of Children with Williams Syndrome: A Rather Neglected Area of Research? / Olympia PALIKARA in Journal of Autism and Developmental Disorders, 48-9 (September 2018)
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Titre : Addressing the Educational Needs of Children with Williams Syndrome: A Rather Neglected Area of Research? Type de document : Texte imprimé et/ou numérique Auteurs : Olympia PALIKARA, Auteur ; Maria ASHWORTH, Auteur ; J. VAN HERWEGEN, Auteur Article en page(s) : p.3256-3259 Langues : Anglais (eng) Mots-clés : Academic achievements Educational provision Interventions Transition Williams syndrome Index. décimale : PER Périodiques Résumé : Williams syndrome (WS) is a rare neurodevelopmental disorder associated with physical health problems, limitations in cognitive abilities and increased risk of mental health difficulties. This profile of complex needs may make it challenging to support children with WS in schools. Surprisingly, in the current international move for inclusion, limited research exists on the educational provision and academic achievements of children with WS, including the non-existing literature on their voices and the perspectives of key stakeholders. This letter calls for additional research on the risk and protective factors associated with the educational outcomes of these children, the perspectives of the children themselves and the development of the evidence-base about the effectiveness of education intervention programs. En ligne : http://dx.doi.org/10.1007/s10803-018-3578-x Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=368
in Journal of Autism and Developmental Disorders > 48-9 (September 2018) . - p.3256-3259[article] Addressing the Educational Needs of Children with Williams Syndrome: A Rather Neglected Area of Research? [Texte imprimé et/ou numérique] / Olympia PALIKARA, Auteur ; Maria ASHWORTH, Auteur ; J. VAN HERWEGEN, Auteur . - p.3256-3259.
Langues : Anglais (eng)
in Journal of Autism and Developmental Disorders > 48-9 (September 2018) . - p.3256-3259
Mots-clés : Academic achievements Educational provision Interventions Transition Williams syndrome Index. décimale : PER Périodiques Résumé : Williams syndrome (WS) is a rare neurodevelopmental disorder associated with physical health problems, limitations in cognitive abilities and increased risk of mental health difficulties. This profile of complex needs may make it challenging to support children with WS in schools. Surprisingly, in the current international move for inclusion, limited research exists on the educational provision and academic achievements of children with WS, including the non-existing literature on their voices and the perspectives of key stakeholders. This letter calls for additional research on the risk and protective factors associated with the educational outcomes of these children, the perspectives of the children themselves and the development of the evidence-base about the effectiveness of education intervention programs. En ligne : http://dx.doi.org/10.1007/s10803-018-3578-x Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=368 Attention to Faces in Williams Syndrome / Deborah M. RIBY in Journal of Autism and Developmental Disorders, 41-9 (September 2011)
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Titre : Attention to Faces in Williams Syndrome Type de document : Texte imprimé et/ou numérique Auteurs : Deborah M. RIBY, Auteur ; Nicola JONES, Auteur ; Philippa H. BROWN, Auteur ; Lucy J. ROBINSON, Auteur ; Stephen R. H. LANGTON, Auteur ; Vicki BRUCE, Auteur ; Leigh M. RIBY, Auteur Année de publication : 2011 Article en page(s) : p.1228-1239 Langues : Anglais (eng) Mots-clés : Williams syndrome Social attention Face perception Autism Index. décimale : PER Périodiques Résumé : Williams syndrome (WS) is associated with distinct social behaviours. One component of the WS social phenotype is atypically prolonged face fixation. This behaviour co-exists with attention difficulties. Attention is multi-faceted and may impact on gaze behaviour in several ways. Four experiments assessed (i) attention capture by faces, (ii) interference from facial stimuli, (iii) face bias, and (iv) attention disengagement. Individuals with WS were compared to typically developing participants of comparable nonverbal ability and chronological age. The first three experiments revealed no atypicality of attention to faces in WS. However, in experiment 4 there was a suggestion that individuals with WS (compared to those developing typically) found it much more time consuming to disengage from faces than objects. The results are discussed in terms of attention abnormalities and possible face disengagement difficulties in WS. En ligne : http://dx.doi.org/10.1007/s10803-010-1141-5 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=142
in Journal of Autism and Developmental Disorders > 41-9 (September 2011) . - p.1228-1239[article] Attention to Faces in Williams Syndrome [Texte imprimé et/ou numérique] / Deborah M. RIBY, Auteur ; Nicola JONES, Auteur ; Philippa H. BROWN, Auteur ; Lucy J. ROBINSON, Auteur ; Stephen R. H. LANGTON, Auteur ; Vicki BRUCE, Auteur ; Leigh M. RIBY, Auteur . - 2011 . - p.1228-1239.
Langues : Anglais (eng)
in Journal of Autism and Developmental Disorders > 41-9 (September 2011) . - p.1228-1239
Mots-clés : Williams syndrome Social attention Face perception Autism Index. décimale : PER Périodiques Résumé : Williams syndrome (WS) is associated with distinct social behaviours. One component of the WS social phenotype is atypically prolonged face fixation. This behaviour co-exists with attention difficulties. Attention is multi-faceted and may impact on gaze behaviour in several ways. Four experiments assessed (i) attention capture by faces, (ii) interference from facial stimuli, (iii) face bias, and (iv) attention disengagement. Individuals with WS were compared to typically developing participants of comparable nonverbal ability and chronological age. The first three experiments revealed no atypicality of attention to faces in WS. However, in experiment 4 there was a suggestion that individuals with WS (compared to those developing typically) found it much more time consuming to disengage from faces than objects. The results are discussed in terms of attention abnormalities and possible face disengagement difficulties in WS. En ligne : http://dx.doi.org/10.1007/s10803-010-1141-5 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=142 Biological determinants of the origin and development of the opposite psychological and behavioral traits associated with autism spectrum disorders and Williams syndrome / Klara VERNEROVA in Research in Autism Spectrum Disorders, 118 (October 2024)
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Titre : Biological determinants of the origin and development of the opposite psychological and behavioral traits associated with autism spectrum disorders and Williams syndrome Type de document : Texte imprimé et/ou numérique Auteurs : Klara VERNEROVA, Auteur ; Roman SOLC, Auteur Article en page(s) : 102479 Langues : Anglais (eng) Mots-clés : Williams syndrome Autism spectrum disorders Duplication 7q11.23 syndrome TFII-I genetic family Hyperserotonemia Timothy syndrome Index. décimale : PER Périodiques Résumé : Williams syndrome is a genetical disorder caused by microdeletion on chromosome 7, specifically of 7q11.23 region. Its phenotype is characterized by typical facial features, mild mental retardation, cardiovascular problems (patients often suffer from supravalvular aortic stenosis), gastrointestinal problems and endocrine abnormalities. However, Williams syndrome is best known for its unique behavioural-cognitive profile which leads to friendly, hypersocial and talkative personality. This specific neuropsychological profile is repeatedly considered to be the right opposite of the psychological profile, which is characteristic for people with autism spectrum disorder. Individuals with autism spectrum disorder often have severe impairments in social interaction, communication and they suffer from social anxieties. This contrasting cognitive-behavioural phenotype inspired many studies to examine possible biological causes, which might determine given personality features. Several results from these studies suggest that small genetical TFII-I family, which lies in the critical Williams syndrome region, could have a significant impact on the extent of personality qualities such as sociality and communication. En ligne : https://doi.org/10.1016/j.rasd.2024.102479 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=540
in Research in Autism Spectrum Disorders > 118 (October 2024) . - 102479[article] Biological determinants of the origin and development of the opposite psychological and behavioral traits associated with autism spectrum disorders and Williams syndrome [Texte imprimé et/ou numérique] / Klara VERNEROVA, Auteur ; Roman SOLC, Auteur . - 102479.
Langues : Anglais (eng)
in Research in Autism Spectrum Disorders > 118 (October 2024) . - 102479
Mots-clés : Williams syndrome Autism spectrum disorders Duplication 7q11.23 syndrome TFII-I genetic family Hyperserotonemia Timothy syndrome Index. décimale : PER Périodiques Résumé : Williams syndrome is a genetical disorder caused by microdeletion on chromosome 7, specifically of 7q11.23 region. Its phenotype is characterized by typical facial features, mild mental retardation, cardiovascular problems (patients often suffer from supravalvular aortic stenosis), gastrointestinal problems and endocrine abnormalities. However, Williams syndrome is best known for its unique behavioural-cognitive profile which leads to friendly, hypersocial and talkative personality. This specific neuropsychological profile is repeatedly considered to be the right opposite of the psychological profile, which is characteristic for people with autism spectrum disorder. Individuals with autism spectrum disorder often have severe impairments in social interaction, communication and they suffer from social anxieties. This contrasting cognitive-behavioural phenotype inspired many studies to examine possible biological causes, which might determine given personality features. Several results from these studies suggest that small genetical TFII-I family, which lies in the critical Williams syndrome region, could have a significant impact on the extent of personality qualities such as sociality and communication. En ligne : https://doi.org/10.1016/j.rasd.2024.102479 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=540 Interpretation of Ambiguous Situations: Evidence for a Dissociation Between Social and Physical Threat in Williams Syndrome / Helen F. DODD in Journal of Autism and Developmental Disorders, 41-3 (March 2011)
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Titre : Interpretation of Ambiguous Situations: Evidence for a Dissociation Between Social and Physical Threat in Williams Syndrome Type de document : Texte imprimé et/ou numérique Auteurs : Helen F. DODD, Auteur ; Melanie A. PORTER, Auteur Année de publication : 2011 Article en page(s) : p.266-274 Langues : Anglais (eng) Mots-clés : Interpretation bias Cognitive bias Williams syndrome Anxiety Developmental disorders Index. décimale : PER Périodiques Résumé : Williams syndrome (WS) is associated with an unusual profile of anxiety, characterised by increased rates of non-social anxiety but not social anxiety (Dodd and Porter, J Ment Health Res Intellect Disabil 2(2):89–109, 2009). The present research examines whether this profile of anxiety is associated with an interpretation bias for ambiguous physical, but not social, situations. Sixteen participants with WS, aged 13–34 years, and two groups of typically developing controls matched to the WS group on chronological age (CA) and mental age (MA), participated. Consistent with the profile of anxiety reported in WS, the WS group were significantly more likely to interpret an ambiguous physical situation as threatening than both control groups. However, no between-group differences were found on the ambiguous social situations. En ligne : http://dx.doi.org/10.1007/s10803-010-1048-1 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=118
in Journal of Autism and Developmental Disorders > 41-3 (March 2011) . - p.266-274[article] Interpretation of Ambiguous Situations: Evidence for a Dissociation Between Social and Physical Threat in Williams Syndrome [Texte imprimé et/ou numérique] / Helen F. DODD, Auteur ; Melanie A. PORTER, Auteur . - 2011 . - p.266-274.
Langues : Anglais (eng)
in Journal of Autism and Developmental Disorders > 41-3 (March 2011) . - p.266-274
Mots-clés : Interpretation bias Cognitive bias Williams syndrome Anxiety Developmental disorders Index. décimale : PER Périodiques Résumé : Williams syndrome (WS) is associated with an unusual profile of anxiety, characterised by increased rates of non-social anxiety but not social anxiety (Dodd and Porter, J Ment Health Res Intellect Disabil 2(2):89–109, 2009). The present research examines whether this profile of anxiety is associated with an interpretation bias for ambiguous physical, but not social, situations. Sixteen participants with WS, aged 13–34 years, and two groups of typically developing controls matched to the WS group on chronological age (CA) and mental age (MA), participated. Consistent with the profile of anxiety reported in WS, the WS group were significantly more likely to interpret an ambiguous physical situation as threatening than both control groups. However, no between-group differences were found on the ambiguous social situations. En ligne : http://dx.doi.org/10.1007/s10803-010-1048-1 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=118 Neuron density is decreased in the prefrontal cortex in Williams syndrome / Caroline HORTON LEW in Autism Research, 10-1 (January 2017)
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Titre : Neuron density is decreased in the prefrontal cortex in Williams syndrome Type de document : Texte imprimé et/ou numérique Auteurs : Caroline HORTON LEW, Auteur ; Chelsea BROWN, Auteur ; Ursula BELLUGI, Auteur ; Katerina SEMENDEFERI, Auteur Article en page(s) : p.99-112 Langues : Anglais (eng) Mots-clés : frontal pole design-based stereology cytoarchitecture Williams syndrome prefrontal cortex neuron density Index. décimale : PER Périodiques Résumé : Williams Syndrome (WS) is a rare neurodevelopmental disorder associated with a hemideletion in chromosome 7, which manifests a distinct behavioral phenotype characterized by a hyperaffiliative social drive, in striking contrast to the social avoidance behaviors that are common in Autism Spectrum Disorder (ASD). MRI studies have observed structural and functional abnormalities in WS cortex, including the prefrontal cortex (PFC), a region implicated in social cognition. This study utilizes the Bellugi Williams Syndrome Brain Collection, a unique resource that comprises the largest WS postmortem brain collection in existence, and is the first to quantitatively examine WS PFC cytoarchitecture. We measured neuron density in layers II/III and V/VI of five cortical areas: PFC areas BA 10 and BA 11, primary motor BA 4, primary somatosensory BA 3, and visual area BA 18 in six matched pairs of WS and typically developing (TD) controls. Neuron density in PFC was lower in WS relative to TD, with layers V/VI demonstrating the largest decrease in density, reaching statistical significance in BA 10. In contrast, BA 3 and BA 18 demonstrated a higher density in WS compared to TD, although this difference was not statistically significant. Neuron density in BA 4 was similar in WS and TD. While other cortical areas were altered in WS, prefrontal areas appeared to be most affected. Neuron density is also altered in the PFC of individuals with ASD. Together these findings suggest that the PFC is targeted in neurodevelopmental disorders associated with sociobehavioral alterations. En ligne : http://dx.doi.org/10.1002/aur.1677 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=303
in Autism Research > 10-1 (January 2017) . - p.99-112[article] Neuron density is decreased in the prefrontal cortex in Williams syndrome [Texte imprimé et/ou numérique] / Caroline HORTON LEW, Auteur ; Chelsea BROWN, Auteur ; Ursula BELLUGI, Auteur ; Katerina SEMENDEFERI, Auteur . - p.99-112.
Langues : Anglais (eng)
in Autism Research > 10-1 (January 2017) . - p.99-112
Mots-clés : frontal pole design-based stereology cytoarchitecture Williams syndrome prefrontal cortex neuron density Index. décimale : PER Périodiques Résumé : Williams Syndrome (WS) is a rare neurodevelopmental disorder associated with a hemideletion in chromosome 7, which manifests a distinct behavioral phenotype characterized by a hyperaffiliative social drive, in striking contrast to the social avoidance behaviors that are common in Autism Spectrum Disorder (ASD). MRI studies have observed structural and functional abnormalities in WS cortex, including the prefrontal cortex (PFC), a region implicated in social cognition. This study utilizes the Bellugi Williams Syndrome Brain Collection, a unique resource that comprises the largest WS postmortem brain collection in existence, and is the first to quantitatively examine WS PFC cytoarchitecture. We measured neuron density in layers II/III and V/VI of five cortical areas: PFC areas BA 10 and BA 11, primary motor BA 4, primary somatosensory BA 3, and visual area BA 18 in six matched pairs of WS and typically developing (TD) controls. Neuron density in PFC was lower in WS relative to TD, with layers V/VI demonstrating the largest decrease in density, reaching statistical significance in BA 10. In contrast, BA 3 and BA 18 demonstrated a higher density in WS compared to TD, although this difference was not statistically significant. Neuron density in BA 4 was similar in WS and TD. While other cortical areas were altered in WS, prefrontal areas appeared to be most affected. Neuron density is also altered in the PFC of individuals with ASD. Together these findings suggest that the PFC is targeted in neurodevelopmental disorders associated with sociobehavioral alterations. En ligne : http://dx.doi.org/10.1002/aur.1677 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=303 Patterns of Sensitivity to Emotion in Children with Williams Syndrome and Autism: Relations Between Autonomic Nervous System Reactivity and Social Functioning / Anna JÄRVINEN in Journal of Autism and Developmental Disorders, 45-8 (August 2015)
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PermalinkBrief Report: Evaluating the Effectiveness of Music to Obtain More Accurate Blood Pressure Readings in Children with Williams Syndrome / Jennifer R. WALTON in Journal of Autism and Developmental Disorders, 52-2 (February 2022)
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PermalinkA Distance-Delivered Social Skills Program for Young Adults with Williams Syndrome: Evaluating Feasibility and Preliminary Efficacy / Marisa H. FISHER in Journal of Autism and Developmental Disorders, 52-12 (December 2022)
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PermalinkEnhanced Memory for Vocal Melodies in Autism Spectrum Disorder and Williams Syndrome / M. W. WEISS in Autism Research, 14-6 (June 2021)
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PermalinkEpigenetic aging in Williams syndrome / Satoshi OKAZAKI in Journal of Child Psychology and Psychiatry, 63-12 (December 2022)
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