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Background Present day service systems evolved from the traditional model of disability intervention where the child with the disability and the family were viewed as pathological entities that needed to be fixed rather than supported. Scholars have increasingly called for a greater focus on the family in service delivery, but few studies have empirically examined the practical reality of such a shift. The present paper examines the disability-related formal service supports within the family quality of life (FQOL) framework in a sample of predominantly low-income, minority families in the USA. Methods Cross-sectional data collected from a convenience sample of 149 families using the Family Quality of Life Survey (FQOLS-2006) was analysed at the univariate, bivariate and multivariate levels. Results Over half of the families indicated that they needed more help from the service system, and the largest barrier to accessing services was a lack of information. Almost all families viewed service support as very important to their overall FQOL; however, only half of them were satisfied with the formal support that they were receiving. Less than half of the families reported having many service support opportunities and high attainment of service support, although most took high initiative in pursuing formal supports. The path model illustrated the complex inter-relationships between the six dimensions of service support. Conclusions Findings underscore the need for resources to empower families and the value of using the FQOLS-2006 to ascertain the service support needs and strengths of families. [ABSTRACT FROM AUTHOR]

Background The Taiwanese government launched a new programme in November 2004 to support adults with intellectual disabilities living in smaller facilities. This paper aims to evaluate the service outcomes of this new residential scheme over 2 years including those residents who moved from an institution and those who moved from their family. Methods A one-group repeated-measures analysis was conducted for five interviews after the adults with intellectual disabilities entered the new environment. Forty-nine adults were initially studied (T1) and 29 adults remained in the homes until the end of the study (T5). Results This study found significant improvements over the 2 years in the residents' quality of life and family contact. The results also highlight a decrease in maladaptive behaviour among the residents moving from institution and an increase in choice making and family contact among the residents moving from family. No significant changes in adaptive behaviour and community inclusion were found. Conclusion Results revealed that further policy changes and financial support including service quality assurance are required in order to improve service outcomes for adults living in the new residential scheme. [ABSTRACT FROM AUTHOR]

Background In the UK, the closure of 'long-stay' hospitals was accompanied by the development of community teams (CTs) to support people with intellectual disabilities (IDs) to live in community settings. The self-reported experiences of staff working in such teams have been neglected. Methods Focusing on a single county-wide service, comprising five multi-disciplinary and inter-agency CTs, we measured perceptions among the health care and care management Team members of (1) their personal well-being; (2) the functioning of their team; and (3) the organisation's commitment to quality, and culture. Results Almost three-quarters of the questionnaires were returned (73/101; 72%). The scores of health care practitioners and care managers were very similar: (1) the MBI scores of more than half the respondents were 'of concern'; (2) similarly, almost four in ten respondents' scores on the Vision scale of the TCI were 'of concern'; (3) the perceived commitment to quality (QIIS-II Part 2) was uncertain; and (4) the organisational culture (QIIS-II, Part 1) was viewed as primarily hierarchical. Discussion The perceived absence of a vision for the service, combined with a dominant culture viewed by its members as strongly focussed on bureaucracy and process, potentially compromises the ability of these CTs to respond proactively to the needs of people with IDs. Given the changes in legislation, policy and practice that have taken place since CTs were established, it would be timely to revisit their role and purpose. [ABSTRACT FROM AUTHOR]

Background Williams syndrome (WS) is characterised by severe deficits in visual-spatial abilities in contrast to relatively well-developed language abilities. There is very limited knowledge about visual-motor integration (VMI) in people with WS. Method Twenty-six participants with WS aged 6 to 41 years were assessed with all three tests of the Beery-VMI test, namely the VMI test, the visual perception test (VP) and the motor coordination test (MC). Their results were compared with those of 154 typically developing children (TD) aged 4 to 12. Results No influence of age on the three tested abilities was found amongst the participants with WS in comparison with the TD children. The participants with WS scored similarly to the 5-year-old TD children in all three tasks; their scores on the VMI correlated with the results on the VP and MC tests, which were similar to those of the TD children. Finally, the scores on the non-verbal intelligence test (Raven Coloured Progressive Matrices) were highly predictive of the scores in the VMI and VP tests and partially explain the variance in the MC scores. Conclusions The present study is the first to use all three tasks of the Beery-VMI test. For the TD children, the performances on the three subtests did not show the same developmental trajectory. In contrast, the participants with WS did not show the same developmental trajectory. The participants with WS exhibited poor performances on all tasks with scores comparable with the 5-year-old TD children. As high correlations between these abilities were observed, improving VP and MC could help the development of VMI, which in turn could improve visual-spatial abilities in individuals with WS. [ABSTRACT FROM AUTHOR]

Background Individuals with Williams syndrome (WS) are known to have difficulties in carrying out fine motor movements; however, a detailed behavioural profile of WS in this domain is still missing. It is also unknown how great the capacity to improve these skills with focused and extensive practice is. Method We studied initial performance and learning capacity in a sequential finger tapping (FT) task in WS and in typical development. Improvement in the FT task has been shown to be sleep dependent. WS subjects participating in the current study have also participated in earlier polysomnography studies, although not directly related to learning. Results WS participants presented with great individual variability. In addition to generally poor initial performance, learning capacity was also greatly limited in WS. We found indications that reduced sleep efficiency might contribute to this limitation. Conclusions Estimating motor learning capacity and the depth of sleep disorder in a larger sample of WS individuals might reveal important relationships between sleep and learning, and contribute to efficient intervention methods improving skill acquisition in WS. [ABSTRACT FROM AUTHOR]

Background Despite high levels of social engagement, the social competence of individuals with Williams syndrome (WS) is frequently compromised. This descriptive study explores the ability of young people with WS to learn from facial expressions when provided as a source of feedback for their actions. Method Using a novel task, the ability to interpret facial expressions and adapt behaviour after receiving feedback in the form of happy or angry faces was assessed in 12 participants with WS aged between 10 and 28 years and with a mean nonverbal mental age of 6.5 years, and in typically developing (TD) children aged between 4 and 7 years. Results Individuals with WS were able to use facial expressions as feedback in a manner commensurate with their mental age, only when other cognitive demands were low. Their performance profile differed from that of the TD children matched for mental age and from the performance profile of 4 year olds. Conclusions Possible explanations for the unique performance profile observed in the participants with WS are discussed. The results highlight the need to examine social competencies in the context of the cognitive demands characteristic of social environments. [ABSTRACT FROM AUTHOR]

Background Fragile X syndrome (FXS) is an x-linked genetic disorder that represents the most common hereditary cause of Intellectual Disability (ID). Very specific behavioural features (e.g. attention deficit hyperactivity disorder and stereotyped behaviour) are associated with FXS in adolescents and adults, yet research on temperament and behavioural characteristics in young children with FXS has been more limited and less conclusive. Method This study investigated temperament differences in young boys (3-7 years old) with FXS ( N = 26) recruited from a national FXS centre and controls ( N = 26) matched on age, gender and race. Results Compared with controls, boys with FXS exhibited less overall surgency/extraversion and effortful control. Boys with FXS also displayed significantly greater activity and shyness and less attentional focusing, inhibitory control, soothability and high intensity pleasure (tendency to enjoy intense/complex activities), relative to comparison children. A significant interaction between age and diagnosis (FXS or control) was observed for negative affectivity only. Conclusions Attention difficulties commonly found in adolescents and adults with FXS appear to also be characteristic of young boys with FXS, as reflected by lower effortful control. Age-related findings concerning negative affectivity may be particularly significant, leading to improved intervention/preventative efforts. [ABSTRACT FROM AUTHOR]

Background Substance use disorders (SUD) are associated with several neurobiological disruptions, including biases in attention and approach/avoidance behaviour. The aims of this study were to compare the strength of cognitive biases between light and problematic drinkers, to explore the role of IQ on the cognitive biases and to study the psychometric qualities of the measures. Method Participants ( N = 130) were divided into four groups based on IQ and severity of alcohol use-related problems: light ( n = 28) and problematic drinkers ( n = 25) with (sub)average IQ and light ( n = 33) and problematic drinkers ( n = 44) with mild to borderline intellectual disability (MBID). All participants performed the visual dot probe task and the approach avoidance task to measure the strength of cognitive biases. Results In contrast with the hypothesis, no cognitive biases were found in problematic drinkers. Full scale IQ nor level of craving influenced the strength of the cognitive biases in light and problematic drinkers, although IQ did influence task performance (i.e. large intra-individual, trial-to-trial variation in reaction time). The internal consistency of the visual dot probe task was good, whereas the internal consistency of the approach avoidance task was poor. Conclusion Cognitive biases seem to vary within the group of problematic drinkers as a whole. The psychometric qualities of the measures are problematic, especially in relation to the intra-individual variability in reaction time found in participants with MBID. Until the implications of this variability on the validity of implicit measures and establishing bias scores are more clear, the use of these measures in individuals with MBID calls for scrutiny. [ABSTRACT FROM AUTHOR]

Background Parenting stress has been consistently reported among parents of children with developmental disabilities. However, to date, no studies have investigated the impact of a molecular subtype of Angelman syndrome ( AS) on parent stress, despite distinct phenotypic differences among subtypes. Method Data for 124 families of children with three subtypes of AS: class I and II deletions ( n = 99), imprinting centre defects ( IC defects; n = 11) and paternal uniparental disomy ( UPD; n = 14) were drawn from the AS Rare Diseases Clinical Research Network ( RDCRN) database and collected from five research sites across the Unites States. The AS study at the RDCRN gathered health information to understand how the syndrome develops and how to treat it. Parents completed questionnaires on their perceived psychological stress, the severity of children's aberrant behaviour and children's sleep patterns. Children's adaptive functioning and developmental levels were clinically evaluated. Results Child-related stress reached clinical levels for 40% of parents of children with deletions, 100% for IC defects and 64.3% for UPD. Sleep difficulties were similar and elevated across subtypes. There were no differences between molecular subtypes for overall child and parent-related stress. However, results showed greater isolation and lack of perceived parenting skills for parents of children with UPD compared with deletions. Better overall cognition for children with deletions was significantly related to more child-related stress while their poorer adaptive functioning was associated with more child-related stress. For all three groups, the severity of children's inappropriate behaviour was positively related to different aspects of stress. Conclusions How parents react to stress depends, in part, on children's AS molecular subtype. Despite falling under the larger umbrella term of AS, it is important to acknowledge the unique aspects associated with children's molecular subtype. Identifying these factors can lead to tailored interventions that fit the particular needs of families of children with different AS subtypes. [ABSTRACT FROM AUTHOR]

Background Children with autism spectrum disorder ( ASD) may require interventions for communication difficulties. One type of intervention is picture communication symbols which are proposed to improve comprehension of linguistic input for children with ASD. However, atypical attention to faces and objects is widely reported across the autism spectrum for several types of stimuli. Method In this study we used eye-tracking methodology to explore fixation duration and time taken to fixate on the object and face areas within picture communication symbols. Twenty-one children with ASD were compared with typically developing matched groups. Results Children with ASD were shown to have similar fixation patterns on face and object areas compared with typically developing matched groups. Conclusions It is proposed that children with ASD attend to the images in a manner that does not differentiate them from typically developing individuals. Therefore children with and without autism have the same opportunity to encode the available information. We discuss what this may imply for interventions using picture symbols. [ABSTRACT FROM AUTHOR]

Background Modifying the consistency of food and drink is a strategy commonly used in the management of dysphagia for people with intellectual disabilities ( ID). People with ID often depend on others for the preparation of food and drink and therefore depend on those caregivers achieving the correct consistency to keep them safe and avoid discomfort during mealtimes. Clinical experience and prior research have demonstrated that although training can improve modification, carers often find modification difficult and potentially stressful and recommend additional support for carers. Fluid consistency is often modified through the addition of powdered thickener. This study investigates the efficacy of typical training and use of consistency guides, the Thickness Indicator Model ( TIM) tubes, in helping carers to modify fluids accurately. Method A 3 × 3 pre-post experimental design with a control group was employed to compare the observed accuracy of modification across three groups and at three time points (pre-intervention baseline, immediately post-training intervention and 3-10 months post-training). Sixty-two paid carers who supported people with ID were recruited to participate in the study and each was randomly allocated to one of the three groups: a control group given written guidance only, a group who received typical training and written guidance and a group who received training, written guidance and the TIM tubes. Results & Conclusions Typical training resulted in significantly greater carer accuracy in modifying fluid consistencies when compared with written guidance alone. Use of the TIM tubes also significantly improved accuracy in the modification of drinks compared with the group who modified with the aid of written guidance alone. At 3-10-month follow-up only the group who received typical training alongside the TIM tubes were significantly more accurate than the Written Guidance group. Further research is warranted to ascertain the effectiveness of the training and the utility of the TIM tubes in improving accuracy over a longer time scale and in individuals' usual living environments. [ABSTRACT FROM AUTHOR]

Background A number of jurisdictions have instituted legislation requiring an independent person to be present during police interviews with vulnerable people. In Victoria, Australia, a group of volunteers known as Independent Third Persons help to fulfil this role with people who present with cognitive impairment arising from their mental illness or disability. This study sought to explore the perspectives of the Independent Third Person volunteers on police identification of and responses to people with intellectual disability (ID). Methods All registered Independent Third Person volunteers across the State of Victoria in Australia were identified and sent a postal survey on their experiences and confidence in performing their role, their perceptions of police competency, and the challenges they faced working at this interface. Results Of the 207 Independent Third Persons identified, 94 (45%) completed and returned the survey. Participants reported that despite being overly reliant on previous police contacts and cues relating to communication difficulties, they viewed police as generally competent in their ability to identify people with ID. They also considered themselves confident in performing their own roles at this interface, albeit more so at the perfunctory aspects of the role and less so with the emotional aspects of supporting the person being interviewed. Conclusions Police are seen as competent at identifying those with cognitive deficits and seeking appropriate supports for the person with ID in the interview context. More specialised training for police members is recommended in communicating with people with IDs. Volunteers working at this interface require additional support and training in helping to meet the emotional needs of those being interviewed. [ABSTRACT FROM AUTHOR]

Background People with developmental disabilities are at high risk for a limited participation in leisure activities. The aim of this study was to investigate the participation in, preference for and interest in leisure activities of young and adults with developmental disabilities, and to examine the factors associated with leisure activity. Methods A cross-sectional design was used with a convenience sample of 237 people aged 17 to 65, living in the community. Leisure participation was assessed with the Spanish version of Leisure Assessment Inventory. Percentages were calculated by types of activity, and repeated measures anovas were used to analyse the differences between types of activities, and mixed anovas to analyse the factors that explain differences in leisure activity participation, preference and interest. Results Leisure social activities and recreation activities at home were mostly solitary and passive in nature and were identified as those being most commonly engaged in. Respondents expressed preference for more social and physical activity, and they were interested in trying out a large number of physical activities. Age and type of schooling determine participation in leisure activity. The results underscore the differences in leisure activity participation, preference and interest depending on the severity of the disability. Conclusions The findings reveal interesting patterns of participation in leisure activities from the viewpoint of youngsters and adults with developmental disabilities. Leisure participation among people with developmental disabilities is likely to be more affected by environmental factors than by personal factors. [ABSTRACT FROM AUTHOR]

Background Individuals with an intellectual disability (ID) continue to experience major obstacles towards social, educational and vocational integration. Negative attitudes toward persons with ID has remained relevant over time and has led to discrimination and stigma. Objective The present study describes the development of a new questionnaire for tapping into the general population's attitudes toward individuals with ID and addresses its psychometric properties. Methods Adopting a multidimensional perspective, the Attitudes Toward Intellectual Disability Questionnaire (ATTID) was developed from a series of previously validated instruments and principles from the Montreal Declaration on Intellectual Disability (2004). The ATTID was administered by phone to 1605 randomly selected adult men and women, stratified by region in the Province of Quebec, Canada. Results The ATTID yielded a five-factor structure overlapping the tri-partite model of attitudes. The cognitive component was represented by two factors: knowledge of capacity and rights and knowledge of causes of ID. The affective component tapped into two factors: discomfort and sensitivity/compassion. Finally, the behavioural component emerged as a single factor. The ATTID had good internal consistency with Cronbach's alpha coefficients ranging from 0.59 to 0.89 for the five factors and of 0.92 for the overall questionnaire. Test-retest reliability yielded correlations from 0.62 to 0.83 for the five factors. Conclusion The ATTID can be used to measure attitudes among different populations and allows comparisons over time within the same population as a function of various intervention strategies for de-stigmatising ID. [ABSTRACT FROM AUTHOR]

Background Genetically Williams syndrome (WS) promises to provide essential insight into the pathophysiology of cortical development because its ∼28 deleted genes are crucial for cortical neuronal migration and maturation. Phenotypically, WS is one of the most puzzling childhood neurodevelopmental disorders affecting most intellectual deficiencies (i.e. low-moderate intelligence quotient, visuospatial deficits) yet relatively preserving what is uniquely human (i.e. language and social-emotional cognition). Therefore, WS provides a privileged setting for investigating the relationship between genes, brain and the consequent complex human behaviour. Methods We used in vivo anatomical magnetic resonance imaging analysing cortical surface-based morphometry, (i.e. surface area , cortical volume , cortical thickness , gyrification index ) and cortical complexity , which is of particular relevance to the WS genotype-phenotype relationship in 22 children (2.27-14.6 years) to compare whole hemisphere and lobar surface-based morphometry between WS ( n = 10) and gender/age matched normal controls healthy controls ( n = 12). Results Compared to healthy controls, WS children had a (1) relatively preserved Cth; (2) significantly reduced SA and CV; (3) significantly increased GI mostly in the parietal lobe; and (4) decreased CC specifically in the frontal and parietal lobes. Conclusion Our findings are then discussed with reference to the Rakic radial-unit hypothesis of cortical development, arguing that WS gene deletions may spare Cth yet affecting the number of founder cells/columns/radial units, hence decreasing the SA and CV. In essence, cortical brain structure in WS may be shaped by gene-dosage abnormalities. [ABSTRACT FROM AUTHOR]

Background The diagnostic validity of autism spectrum disorder (ASD) based on Diagnostic and Statistical Manual of Mental Disorders (DSM) has been challenged in Down syndrome (DS), because of the high prevalence of cognitive impairments in this population. Therefore, we attempted to validate DSM-based diagnoses via an unbiased categorisation of participants with a DSM-independent behavioural instrument. Methods Based on scores on the Aberrant Behaviour Checklist - Community, we performed sequential factor (four DS-relevant factors: Autism-Like Behaviour, Disruptive Behaviour, Hyperactivity, Self-Injury) and cluster analyses on a 293-participant paediatric DS clinic cohort. The four resulting clusters were compared with DSM-delineated groups: DS + ASD, DS + None (no DSM diagnosis), DS + DBD (disruptive behaviour disorder) and DS + SMD (stereotypic movement disorder), the latter two as comparison groups. Results Two clusters were identified with DS + ASD: Cluster 1 (35.1%) with higher disruptive behaviour and Cluster 4 (48.2%) with more severe autistic behaviour and higher percentage of late onset ASD. The majority of participants in DS + None (71.9%) and DS + DBD (87.5%) were classified into Cluster 2 and 3, respectively, while participants in DS + SMD were relatively evenly distributed throughout the four clusters. Conclusions Our unbiased, DSM-independent analyses, using a rating scale specifically designed for individuals with severe intellectual disability, demonstrated that DSM-based criteria of ASD are applicable to DS individuals despite their cognitive impairments. Two DS + ASD clusters were identified and supported the existence of at least two subtypes of ASD in DS, which deserve further characterisation. Despite the prominence of stereotypic behaviour in DS, the SMD diagnosis was not identified by cluster analysis, suggesting that high-level stereotypy is distributed throughout DS. Further supporting DSM diagnoses, typically behaving DS participants were easily distinguished as a group from those with maladaptive behaviours. [ABSTRACT FROM AUTHOR]

Background Despite progress in the process of deinstitutionalisation, very little is known about the health conditions of people with intellectual disability (PWID) who live in large institutions and PWID living in small residential services, family homes or independent living within the community. Furthermore, there are no international comparison studies at European level of the health status and health risk factors of PWID living in fully staffed residential services with formal support and care compared with those living in unstaffed family homes or independent houses with no formal support. Methods A total of 1269 persons with ID and/or their proxy respondents were recruited and face-to-face interviewed in 14 EU countries with the P15, a multinational assessment battery for collecting data on health indicators relevant to PWID. Participants were grouped according to their living arrangements, availability of formal support and stage of deinstitutionalisation. Results Obesity and sedentary lifestyle along with a number of illnesses such as epilepsy, mental disorders, allergies or constipation were highly prevalent among PWID. A significantly higher presence of myocardial infarctions, chronic bronchitis, osteoporosis and gastric or duodenal ulcers was found among participants in countries considered to be at the early stage of deinstitutionalisation. Regardless of deinstitutionalisation stage, important deficits in variables related to such medical health promotion measures as vaccinations, cancer screenings and medical checks were found in family homes and independent living arrangements. Age, number of people living in the same home or number of places in residential services, presence of affective symptoms and obesity require further attention as they seem to be related to an increase in the number of illnesses suffered by PWID. Discussion Particular illnesses were found to be highly prevalent in PWID. There were important differences between different living arrangements depending on the level of formal support available and the stage of deinstitutionalisation. PWID are in need of tailored primary health programs that guarantee their access to quality health and health promotion and the preventative health actions of vaccination programs, systematic health checks, specific screenings and nutritional controls. Extensive national health surveys and epidemiological studies of PWID in the EC member states are urgently needed in order to reduce increased morbidity rates among this population. [ABSTRACT FROM AUTHOR]