Visuo-spatial cognition in Williams syndrome: Reviewing and accounting for the strengths and weaknesses in performance

Individuals with Williams syndrome typically show relatively poor visuo-spatial abilities in comparison to stronger verbal skills. However, individuals' level of performance is not consistent across all visuo-spatial tasks. The studies assessing visuo-spatial functioning in Williams syndrome are critically reviewed, in order to provide a clear pattern of the relative difficulty of these tasks. This prompts a possible explanation of the variability in performance seen which focuses on the processing demands of some of these tasks. Individuals with Williams syndrome show an atypical processing style on tests of construction, which does not affect tests of perception

Interpretation of ambiguous situations: evidence for a dissociation between social and physical threat in Williams syndrome

There is increasing evidence that Williams syndrome (WS) is associated with elevated anxiety that is non-social in nature, including generalised anxiety and fears. To date very little research has examined the cognitive processes associated with this anxiety. In the present research, attentional bias for non-social threatening images in WS was examined using a dot-probe paradigm. Participants were 16 individuals with WS aged between 13 and 34 years and two groups of typically developing controls matched to the WS group on chronological age and attentional control ability respectively. The WS group exhibited a significant attention bias towards threatening images. In contrast, no bias was found for group matched on attentional control and a slight bias away from threat was found in the chronological age matched group. The results are contrasted with recent findings suggesting that individuals with WS do not show an attention bias for threatening faces and discussed in relation to neuroimaging research showing elevated amygdala activation in response to threatening non-social scenes in WS

Uncommon genetic syndromes and narrative production - Case Studies with Williams, Smith-Magenis and Prader- Willi Syndromes

This study compares narrative production among three syndromes with genetic microdeletions: Williams syndrome (WS), Smith-Magenis syndrome (SMS), and Prader-Willi syndrome (PWS), characterized by intellectual disabilities and relatively spared language abilities. Our objective is to study the quality of narrative production in the context of a common intellectual disability. To elicit a narrative production, the task Frog! Where Are You was used. Then, structure, process, and content of the narrative process were analysed in the three genetic disorders:WS (n52), SMS (n52), and PWS (n52). Data show evidence of an overall low narrative quality in these syndromes, despite a high variability within different measures of narrative production. Results support the hypothesis that narrative is a highly complex cognitive process and that, in a context of intellectual disability, there is no evidence of particular ‘hypernarrativity’ in these syndromes.This research was supported by the grants FEDER –

Criança, violência e saúde: desafios e questões atuais

This paper examines challenges and current issues involved in measuring exposure to different types of violence which are associated mental health problems in children and adolescents. Standardized measures suitable for epidemiological studies, selected based on their relevance in the current literature, are briefly described and commented. The assessment of child's exposure to violence may focus on a specific event (e.g., kidnapping), a specific context (e.g., war) or even of a certain type of exposure (e.g., intrafamilial physical violence). The assessment of child mental health after exposure to violence has traditionally focused on posttraumatic stress disorder (PTSD) - most frequently measured through non-diagnostic scales. However, other mental health reactions may be present and screening as well as diagnostic instruments which may be used to assess these reactions are also described. Two issues of emerging importance - the assessment of impairment and of traumatic grief in children - are also presented. Availability of culturally appropriate instruments is a crucial step towards proper identification of child mental health problems after exposure to violence.Este artigo examina os desafios e perspectivas atuais envolvidos na mensuração da exposição a diferentes tipos de violência e problemas de saúde mental em crianças e adolescentes. Instrumentos padronizados apropriados para estudos epidemiológicos, selecionados com base em sua relevância na literatura, são brevemente descritos e comentados. A avaliação de exposição à violência em crianças pode dizer respeito a um evento específico (como sequestro) ou um contexto específico (como guerra) ou mesmo um determinado tipo de exposição (como violência física intrafamiliar). A avaliação da saúde mental infantil após a exposição à violência tradicionalmente concentrou-se na avaliação do transtorno de estresse pós-traumático (TEPT) - freqüentemente avaliado através de escalas não-diagnósticas. Porém, outras reações psicológicas podem ocorrer e instrumentos que podem ser usados para avaliar estas reações também são descritos neste artigo. Dois tópicos de importância emergente - a avaliação de prejuízo funcional e do pesar traumático em crianças - são também apresentados. Instrumentos culturalmente apropriados são essenciais para a identificação de problemas de saúde mental em crianças após a exposição à violência.Division of Child and Adolescent PsychiatryUniversidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM) Departamento de PsiquiatriaUNIFESP, EPM, Depto. de PsiquiatriaSciEL

Anxiety disorders in children with Williams syndrome, their mothers, and their siblings: Implications for the etiology of anxiety disorders

This study examines the prevalence of anxiety disorders in children with Williams syndrome (WS), their sibling closest in age, and their mothers as well as the predictors of anxiety in these groups. The prevalence of anxiety disorders was assessed and compared to that in the general population. Children with WS had a significantly higher prevalence of specific phobia, generalized anxiety disorder (GAD), and separation anxiety in comparison to children in the general population. While mothers had a higher prevalence of GAD than population controls, the excess was accounted for by mothers who had onset after the birth of their WS child. The siblings had rates similar to the general population. This pattern of findings suggests the presence of a gene in the WS region whose deletion predisposes to anxiety disorders. It is also worthwhile to investigate relations between genes deleted in WS and genes previously implicated in anxiety disorders

Treating children traumatized by war and Tsunami: A comparison between exposure therapy and meditation-relaxation in North-East Sri Lanka

<p>Abstract</p> <p>Background</p> <p>The North-Eastern part of Sri Lanka had already been affected by civil war when the 2004 Tsunami wave hit the region, leading to high rates of posttraumatic stress disorder (PTSD) in children. In the acute aftermath of the Tsunami we tested the efficacy of two pragmatic short-term interventions when applied by trained local counselors.</p> <p>Methods</p> <p>A randomized treatment comparison was implemented in a refugee camp in a severely affected community. 31 children who presented with a preliminary diagnosis of PTSD were randomly assigned either to six sessions Narrative Exposure Therapy for children (KIDNET) or six sessions of meditation-relaxation (MED-RELAX). Outcome measures included severity of PTSD symptoms, level of functioning and physical health.</p> <p>Results</p> <p>In both treatment conditions, PTSD symptoms and impairment in functioning were significantly reduced at one month post-test and remained stable over time. At 6 months follow-up, recovery rates were 81% for the children in the KIDNET group and 71% for those in the MED-RELAX group. There was no significant difference between the two therapy groups in any outcome measure.</p> <p>Conclusion</p> <p>As recovery rates in the treatment groups exceeded the expected rates of natural recovery, the study provides preliminary evidence for the effectiveness of NET as well as meditation-relaxation techniques when carried out by trained local counselors for the treatment of PTSD in children in the direct aftermath of mass disasters.</p> <p>Trial registration</p> <p>ClinicalTrials.gov Identifier:NCT00820391</p

Language and sociability: insights from Williams syndrome

One of the most compelling features of Williams syndrome (WS) is the widely reported excessive sociability, accompanied by a relative proficiency in expressive language, which stands in stark contrast with significant intellectual and nonverbal impairments. It has been proposed that the unique language skills observed in WS are implicated in the strong drive to interact and communicate with others, which has been widely documented in WS. Nevertheless, this proposition has yet to be empirically examined. The present study aimed at investigating the relationship between a brain index of language processing and judgments of approachability of faces, as a proxy for sociability, in individuals with WS as contrasted to typical controls. Results revealed a significant and substantial association between the two in the WS, but not in the control group, supporting the hitherto untested notion that language use in WS might be uniquely related to their excessive social drive

Life Events, Coping, and Posttraumatic Stress Symptoms among Chinese Adolescents Exposed to 2008 Wenchuan Earthquake, China

PURPOSE: To examine the relationship between negative life events, coping styles, and symptoms of post-traumatic stress disorder (PTSD) among adolescent survivors exposed to 2008 Wenchuan Earthquake, China. METHODS: A survey was conducted in a sample of 2250 adolescent students from two schools in Dujiangyan District, a seriously damaged area, 20 kilometers away from the epicenter, 6 months after the earthquake. Participants completed a self-administered questionnaire including demographics, negative life events, coping styles, and PTSD symptoms. RESULTS: Academic pressure was the strongest predictor of adolescents' PTSD symptoms among all negative life events. Main effects of negative life events, positive coping and negative coping on PTSD symptoms were significant in both younger adolescents and older adolescents, while the moderator effects of two coping styles were found significant only within older adolescents. CONCLUSIONS: Coping may play a role to moderate the relationship between post-earthquake negative life events and PTSD symptom, but the function seems to depend on the age of participants. Psychosocial coping skills training may be important in the prevention and intervention of mental health problems in adolescent survivors of traumatic earthquake

Regional Brain Differences in Cortical Thickness, Surface Area and Subcortical Volume in Individuals with Williams Syndrome

Williams syndrome (WS) is a rare genetic neurodevelopmental disorder characterized by increased non-social anxiety, sensitivity to sounds and hypersociability. Previous studies have reported contradictory findings with regard to regional brain variation in WS, relying on only one type of morphological measure (usually volume) in each study. The present study aims to contribute to this body of literature and perhaps elucidate some of these discrepancies by examining concurrent measures of cortical thickness, surface area and subcortical volume between WS subjects and typically-developing (TD) controls. High resolution MRI scans were obtained on 31 WS subjects and 50 typically developing control subjects. We derived quantitative regional estimates of cortical thickness, cortical surface area, and subcortical volume using FreeSurfer software. We evaluated between-group ROI differences while controlling for total intracranial volume. In post-hoc exploratory analyses within the WS group, we tested for correlations between regional brain variation and Beck Anxiety Inventory scores. Consistent with our hypothesis, we detected complex patterns of between-group cortical variation, which included lower surface area in combination with greater thickness in the following cortical regions: post central gyrus, cuneus, lateral orbitofrontal cortex and lingual gyrus. Additional cortical regions showed between-group differences in one (but not both) morphological measures. Subcortical volume was lower in the basal ganglia and the hippocampus in WS versus TD controls. Exploratory correlations revealed that anxiety scores were negatively correlated with gray matter surface area in insula, OFC, rostral middle frontal, superior temporal and lingual gyrus. Our results were consistent with previous reports showing structural alterations in regions supporting the socio-affective and visuospatial impairments in WS. However, we also were able to effectively capture novel and complex patterns of cortical differences using both surface area and thickness. In addition, correlation results implicate specific brain regions in levels of anxiety in WS, consistent with previous reports investigating general anxiety disorders in the general population