21 research outputs found

    OSVRT NA KNJIGU "MEDIA NOW: UNDERSTANDING MEDIA, CULTURE, AND TECHNOLOGY" NINTH EDITION

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    Down syndrome (DS) is associated with intellectual disability and an ultra-high risk of developing dementia. Informant ratings are invaluable to assess abilities and related changes in adults with DS, particularly for those with more severe intellectual disabilities and/or cognitive decline. We previously developed the informant rated Cognitive Scale for Down Syndrome (CS-DS) to measure everyday cognitive abilities across memory, executive function, and language domains in adults with DS, finding CS-DS scores are a valid measure of general abilities, and are significantly lower for those with noticeable cognitive decline compared to those without decline. To further test the validity of the CS-DS in detecting changes associated with cognitive decline we collected longitudinal data across two time points, approximately 1.5-2 years apart, for 48 adults with DS aged 36 years and over. CS-DS total scores (78.83±23.85 vs 73.83±25.35, p=0.042) and executive function scores (46.40±13.59 vs 43.54±13.60, p=0.048) significantly decreased between the two time points, with scores in the memory domain trending towards a significant decrease (22.19±8.03 vs 20.81±8.63, p=0.064). Adults with noticeable cognitive decline at follow-up showed a trend to significantly greater change in total scores (7.81±16.41 vs 3.59±16.79, p=0.067) and significantly greater change in executive function scores (5.13±9.22 vs 1.72±9.97, p=0.028) compared to those without decline. Change in total scores showed significant correlations with change in scores from other informant measures of everyday adaptive abilities and symptoms associated with dementia, and participant assessment of general cognitive abilities (all p<0.005), while change in memory scores (R2=0.28, p=0.001) better predicted change in participant cognitive assessment scores than change in executive function (R2=0.15, p=0.016) or language (R2=0.15, p=0.018) scores. These results suggest informants may better detect changes in the executive function domain, while change in informant rated memory scores best predicts change in assessed cognitive ability. Alternatively, memory domain scores may be sensitive to changes across both early and late cognitive decline, whereas executive function domain scores are more sensitive to changes associated with later noticeable cognitive decline. Our results provide further support for the validity of the CS-DS to assess everyday cognitive abilities and to detect associated longitudinal changes in individuals with DS

    Validating the Cognitive Scale for Down Syndrome (CS-DS) to Detect Longitudinal Cognitive Decline in Adults With Down Syndrome

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    Down syndrome (DS) is associated with intellectual disability and an ultra-high risk of developing dementia. Informant ratings are invaluable to assess abilities and related changes in adults with DS, particularly for those with more severe intellectual disabilities and/or cognitive decline. We previously developed the informant rated Cognitive Scale for Down Syndrome (CS-DS) to measure everyday cognitive abilities across memory, executive function, and language domains in adults with DS, finding CS-DS scores are a valid measure of general abilities, and are significantly lower for those with noticeable cognitive decline compared to those without decline. To further test the validity of the CS-DS in detecting changes associated with cognitive decline we collected longitudinal data across two time points, approximately 1.5–2 years apart, for 48 adults with DS aged 36 years and over. CS-DS total scores (78.83 ± 23.85 vs. 73.83 ± 25.35, p = 0.042) and executive function scores (46.40 ± 13.59 vs. 43.54 ± 13.60, p = 0.048) significantly decreased between the two time points, with scores in the memory domain trending towards a significant decrease (22.19 ± 8.03 vs. 20.81 ± 8.63, p = 0.064). Adults with noticeable cognitive decline at follow-up showed a trend to significantly greater change in total scores (7.81 ± 16.41 vs. 3.59 ± 16.79, p = 0.067) and significantly greater change in executive function scores (5.13 ± 9.22 vs. 1.72 ± 9.97, p = 0.028) compared to those without decline. Change in total scores showed significant correlations with change in scores from other informant measures of everyday adaptive abilities and symptoms associated with dementia, and participant assessment of general cognitive abilities (all p &lt; 0.005), while change in memory scores (R2 = 0.28, p = 0.001) better predicted change in participant cognitive assessment scores than change in executive function (R2 = 0.15, p = 0.016) or language (R2 = 0.15, p = 0.018) scores. These results suggest informants may better detect changes in the executive function domain, while change in informant rated memory scores best predicts change in assessed cognitive ability. Alternatively, memory domain scores may be sensitive to changes across both early and late cognitive decline, whereas executive function domain scores are more sensitive to changes associated with later noticeable cognitive decline. Our results provide further support for the validity of the CS-DS to assess everyday cognitive abilities and to detect associated longitudinal changes in individuals with DS

    Health comorbidities and cognitive abilities across the lifespan in Down syndrome.

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    BACKGROUND: Down syndrome (DS) is associated with variable intellectual disability and multiple health and psychiatric comorbidities. The impact of such comorbidities on cognitive outcomes is unknown. We aimed to describe patterns of physical health and psychiatric comorbidity prevalence, and receptive language ability, in DS across the lifespan, and determine relationships with cognitive outcomes. METHODS: Detailed medical histories were collected and cognitive abilities measured using standardised tests for 602 individuals with DS from England and Wales (age range 3 months to 73 years). Differences in prevalence rates between age groups and between males and females were determined using chi-squared or Fisher's exact tests. In adults, rates for psychiatric comorbidities were compared to expected population rates using standardised morbidity ratios (SMRs). Adapted ANCOVA functions were constructed to explore age and sex associations with receptive language ability across the lifespan, and regression analyses were performed to determine whether the presence of health comorbidities or physical phenotypes predicted cognitive abilities. RESULTS: Multiple comorbidities showed prevalence differences across the lifespan, though there were few sex differences. In adults, SMRs were increased in males and decreased in females with DS for schizophrenia, bipolar disorder, and anxiety. Further, SMRs were increased in both males and females with DS for dementia, autism, ADHD, and depression, with differences more pronounced in females for dementia and autism, and in males for depression. Across the lifespan, receptive language abilities increasingly deviated from age-typical levels, and males scored poorer than females. Only autism and epilepsy were associated with poorer cognitive ability in those aged 16-35 years, with no relationships for physical health comorbidities, including congenital heart defects. CONCLUSIONS: Our results indicate the prevalence of multiple comorbidities varies across the lifespan in DS, and in adults, rates for psychiatric comorbidities show different patterns for males and females relative to expected population rates. Further, most health comorbidities are not associated with poorer cognitive outcomes in DS, apart from autism and epilepsy. It is essential for clinicians to consider such differences to provide appropriate care and treatment for those with DS and to provide prognostic information relating to cognitive outcomes in those with comorbidities

    Assessment of an incentivised scheme to provide annual health checks in primary care for adults with intellectual disability: a longitudinal cohort study

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    Background People with intellectual disabilities (ID) have many comorbidities but experience inequities in access to health care. National Health Service England uses an opt-in incentive scheme to encourage annual health checks of patients with ID in primary care. We investigated whether the first 3 years of the programme had improved health care of people with ID. Methods We did a longitudinal cohort study that used data from The Health Improvement Network primary care database. We did multivariate logistic regression to assess associations between various characteristics and whether or not practices had opted in to the incentivised scheme. Findings We assessed data for 8692 patients from 222 incentivised practices and those for 918 patients in 48 non-incentivised practices. More blood tests (eg, total cholesterol, odds ratio [OR] 1·88, 95% CI 1·47–2·41, p<0·0001) general health measurements (eg, smoking status, 6·0, 4·10–8·79, p<0·0001), specific health assessments (eg, hearing, 24·0, 11·5–49·9, p<0·0001), and medication reviews (2·23, 1·68–2·97, p<0·0001) were done in incentivised than in non-incentivised practices, and more health action plans (6·15, 1·41–26·9, p=0·0156) and secondary care referrals (1·47, 1·05–2·05, p=0·0256) were made. Identification rates were higher in incentivised practices for thyroid disorder (OR 2·72, 95% CI 1·09–6·81, p=0·0323), gastrointestinal disorders (1·94, 1·03–3·65, p=0·0390), and obesity (2·49, 1·76–3·53, p<0·0001). Interpretation Targeted annual health checks for people with ID in primary care could reduce health inequities

    Ageing in Down syndrome: Exploring executive functioning and frontal cortical activity using functional near infrared spectroscopy

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    Adults with Down syndrome (DS) are at exceptionally high risk of developing early-onset dementia due to near universal Alzheimer’s disease (AD) neuropathology from age 35. Executive functioning (EF) decline may be an early dementia marker in DS. However, a paucity of functional neuroimaging in DS research limits our understanding of brain function and its relationship with EF performance at all ages. This knowledge gap may reflect difficulties administering neuroimaging protocols using restrictive modalities such as functional magnetic resonance imaging (fMRI). Functional near infrared spectroscopy (fNIRS) is an emerging cortical neuroimaging modality that is less restrictive than fMRI, thus may offer new opportunities for neuroimaging in people with DS. Four studies examined the feasibility of using fNIRS to measure EF-related frontal cortical activity in adults with DS. In study 1, 5 adults with DS and 5 carers discussed fNIRS alongside facilitators and barriers to neuroimaging research participation. Studies 2 (n=12 adults without DS) and 3 (n=9 adults with DS) assessed initial feasibility with four EF tasks: a go / no-go (GNG), a verbal fluency (VFT), a dimensional change card sort (DCCS) and a novel computerised picture-Stroop task. Study 4 examined task performance and haemodynamic responses in the GNG, VFT and Stroop tasks in 46 adults with DS, aged 18-59. 94% of participants with DS completed an fNIRS scan. The GNG, VFT and Stroop were widely accessible, with the latter two tasks showing consistent haemodynamic responses across studies. Peak oxy-haemoglobin concentration during Stroop interference in left inferior frontal / superior temporal regions decreased significantly with increasing age, and this task showed potential sensitivity to performance decline in early dementia stages. fNIRS is an extremely well-tolerated neuroimaging technique, which when combined with sensitive EF measures, can be used to examine age-related changes in brain function in people with DS

    The Association between Physical Activity and CAMDEX-DS Changes Prior to the Onset of Alzheimer’s Disease in Down Syndrome

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    Background: People with Down syndrome are at ultra-high risk of developing Alzheimer’s dementia. At present, there are no preventative or curative treatments. Evidence from sporadic Alzheimer’s disease literature suggests that lifestyle factors including physical activity may help maintain cognitive and functional skills and reduce dementia risk. Our study aimed to explore the association between regular exercise undertaken by participants with Down syndrome and changes in dementia-related domains of cognition and function. This was to consider whether physical activity may be a protective measure to delay cognitive decline and dementia in Down syndrome. Methods: Demographic, lifestyle, and health information was collected at baseline and at a two year follow up from 214 adults with Down syndrome without dementia, who also underwent assessment using the Cambridge Examination for Mental Disorders of Older People with Down Syndrome and Others with Intellectual Disabilities (CAMDEX-DS) and genetic analysis. Logistic regression models were used to examine the potential associations between decline in CAMDEX-DS domains and exercise whilst controlling for key variables. Results: At baseline, engaging in moderate intensity exercise was associated with a 47% reduced risk of everyday skills decline and engaging in high intensity exercise was associated with a 62% reduced risk of decline in personality and behaviour. At follow-up, high levels of exercise were associated with an 87% reduced risk of decline in personality and behaviour. Moderate intensity exercise at baseline was associated with a 62% reduction in risk of decline during the follow-up period in memory and orientation. Discussion: Based on our data it appears that regular moderate and high intensity exercise could reduce the risk of clinically detectable decline in a Down syndrome population with possible long-term benefits. People with Down syndrome may engage in less physical activity than their peers, and barriers remain which can prevent people with Down syndrome engaging in exercise. Our work highlights how important it is that people with Down syndrome are supported to be physically active, and to promote exercise as part of a healthy ageing plan. Clinical trials in this area would be justified to determine if engaging in exercise can lead to realistic improvements in maintaining functioning and delaying dementia onset in Down syndrome and to help develop guidance in this area
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