Behavioural features of Italian infants and young adults with Williams–Beuren syndrome

Gagliardi, Chiara; Martelli, S.; Tavano, Alessandro; Borgatti, Renato

doi:10.1111/j.1365-2788.2010.01376.x

Cited by 11 publications

(12 citation statements)

References 62 publications

(135 reference statements)

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“…To overcome the limitation of some previous studies on ID, deriving psychiatric diagnosis only from questionnaires, the diagnosis was made by the psychiatric examination of an expert clinician interviewing patients and patients' caregivers [1,9,19,20,30]. The choice to interview patients with ID was based on studies indicating that adults with ID are reliable reporters of their psychological and emotional states [9,31].…”

Section: Every Participant Lived With Their Own Familiesmentioning

confidence: 99%

“…Previous studies conducted in these two genetic syndromes independently documented variable percentage of psychiatric disorders, anxiety and behavior problems [1,9,[13][14][15][16][17][18][19][20].…”

Section: Introductionmentioning

confidence: 99%

“…Individuals with WS usually display high sociability, excessive empathy (which may be inappropriate), anxiety, preoccupations and fears, impulsivity, inattention, sadness and depression, generalized anxiety disorder, phobias, and hyperactivity disorder -ADHD - [9,20,21]. Individuals with DS suffer from externalizing disorders and social problems as well as anxiety and obsessive-compulsive disorder, but rarely display depression [22][23][24][25][26].…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Detecting Psychiatric Profile in Genetic Syndromes: A Comparison of Down Syndrome and Williams Syndrome

Vicari¹,

Costanzo²,

Arm³

et al. 2016

J Genet Syndr Gene Ther

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The occurrence and co-occurrence of psychiatric disorders have been more frequently reported in people with Intellectual Disability (ID) than in the general population. The present study was aimed at verifying whether the psychiatric profile of individuals with ID is just a consequence of ID or derives from a specific genotype. The psychiatric profile of 112 individuals with Down syndrome (DS) and 85 with Williams syndrome (WS) was examined. The interactions between psychiatric symptom clusters and the effect of age were also investigated.Participants with WS had higher rates of psychiatric disorders, and, specifically, of Anxiety disorders and Psychosis than DS. However, the psychiatric profile changed by age, since Anxiety disorder was higher in individuals with WS compared to DS in young age, while Psychosis in old age. A relation between the occurrence of disorders, as Anxiety disorder and Mood Disorder, was found only in participants with WS. Moreover, distinct Anxiety and Behavior Disorder subtypes emerged between groups.Results indicate that the genetic etiology of ID differently affects the psychiatric characteristics of the groups and suggest the importance of a targeted psychiatric care for individuals with WS and DS.

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Section: Every Participant Lived With Their Own Familiesmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Detecting Psychiatric Profile in Genetic Syndromes: A Comparison of Down Syndrome and Williams Syndrome

Vicari¹,

Costanzo²,

Arm³

et al. 2016

J Genet Syndr Gene Ther

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“…Outro problema que se associa à síndrome é a elevada incidência de transtornos psiquiátricos sendo os mais incidentes as fobias específicas, Transtorno de Ansiedade e o Transtorno de Déficit de Atenção e Hiperatividade (TDAH) (DYKENS, 2003, LEYFER et al, 2006MARTENS;WILSON;REUTENS, 2008;GAGLIARDI et al, 2011;TEIXEIRA et al, 2012). Pessoas com SW, que têm comorbidade com TDAH, apresentam padrões comportamentais de hiperatividade e impulsividade semelhantes aos observados em crianças com desenvolvimento típico que também apresentam o transtorno.…”

Section: Competências Escolares E Sociais Relato De Pesquisaunclassified

Competências escolares e sociais em crianças e adolescentes com Síndrome de Williams

Hayashiuchi

Segin²,

Schwartzman

et al. 2012

Rev. bras. educ. espec.

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RESUMO: a Síndrome de Williams (SW) é uma doença genética e neurocomportamental causada por uma deleção hemizigótica de múltiplos genes na região cromossômica 7q11-23. Caracteriza-se por alterações cognitivas e comportamentais que interferem no ajustamento psicossocial. O objetivo do estudo foi verificar indicadores comportamentais de habilidades nas áreas social, escolar e de realização de atividades de um grupo de crianças e adolescentes com SW e sinais de desatenção e hiperatividade. A amostra foi composta por 22 crianças e adolescentes com diagnóstico clínico e genético de SW entre sete e 18 anos, média de idade 11,6 (desvio padrão 3,7) e suas respectivas mães. Os instrumentos de coleta de dados foram a Escala de Inteligência Wechsler para Crianças, o Inventário dos Comportamentos de Crianças e Adolescentes de seis a 18 anos e um Questionário que avaliou presença de sinais de desatenção e hiperatividade baseado nos critérios clínicos para Transtorno de Déficit de Atenção e Hiperatividade do Manual de Classificação Estatística dos Transtornos Mentais da Associação Americana de Psiquiatria. Os principais resultados apontaram para um elevado número de sinais de desatenção e hiperatividade (90% do total da amostra pontuaram positivamente nesses sinais). Também foram verificados prejuízos graves nas habilidades e competências de desempenho escolar do grupo, diferentemente dos resultados positivos obtidos nas escalas de socialização e prática de esportes. Conclui-se que os resultados positivos encontrados na área social podem agir como fatores protetores para o desenvolvimento de problemas afetivos como isolamento, tristeza, sentimentos de solidão e baixa autoestima. PALAVRAS-CHAVE:Educação Especial. Competência Social. Desatenção. Hiperatividade. ABSTRACT:Williams Syndrome (WS) is a genetic and neurobehavioral disease caused by a hemizygous deletion of multiples genes in the chromosome region 7q11-23. It is characterized by cognitive and behavioral changes that interfere in psychosocial adjustment. The objective of the study was to verify behavioral indicators of competence in social, educational and performance activities in a group of children and adolescents with WS and signs of inattention and hyperactivity. The sample was composed of 22 children and adolescents with WS clinical and genetic diagnoses between 7 and 18 years, average age 11.6 (standard deviation 3.7) and their mothers.

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“…For example, a few studies have examined the potential relation between intellectual ability and anxiety, with no significant associations found (Dodd & Porter, 2011a;Gagliardi et al, 2011;Leyfer et al, 2006;Leyfer et al, 2009;Perez-Garcia et al, 2011;Porter et al, 2009). In a study on sensory modulation in WS, John and Mervis (2010) used cluster analysis to identify two clusters of children with WS (low sensory-modulation impairment, high sensorymodulation impairment) and examined differences in other domains of functioning as a function of cluster.…”

Section: Anxiety In Individuals With Williams Syndromementioning

confidence: 99%

Anxiety in children with Williams syndrome : association with negative reactivity, self-regulation, and sensory modulation.

Crawford-Zelli

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Behavioural features of Italian infants and young adults with Williams–Beuren syndrome

Cited by 11 publications

References 62 publications

Detecting Psychiatric Profile in Genetic Syndromes: A Comparison of Down Syndrome and Williams Syndrome

Detecting Psychiatric Profile in Genetic Syndromes: A Comparison of Down Syndrome and Williams Syndrome

Competências escolares e sociais em crianças e adolescentes com Síndrome de Williams

Anxiety in children with Williams syndrome : association with negative reactivity, self-regulation, and sensory modulation.

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