Background: Angelman syndrome (AS) is a rare neurogenetic disorder characterized by severe intellectual disability, little or no speech development, and movement problems. Currently, there is a...Show moreBackground: Angelman syndrome (AS) is a rare neurogenetic disorder characterized by severe intellectual disability, little or no speech development, and movement problems. Currently, there is a lack of suitable outcome measures to assess autism spectrum disorder (ASD) in children with AS, and the prevalence of ASD in AS is debated. This study aims to contribute to the development of better outcome measures by investigating the feasibility of a social eye-tracking task in children with AS. We hypothesize that 1) eye-tracking is feasible in children with AS; 2) the severity of the disability of the child can predict the feasibility; 3) children with AS show a preference for faces similar to neurotypical children, and; 4) autism symptoms correlate significantly with eye movement behavior towards faces. Methods: Data from an observational cross-sectional study of 23 children with AS was analyzed. Children were presented with an eye-tracking task that included six slides, each containing a picture of a face and four other non-social stimuli. We considered the task feasible if 1) 70% completed the task and 2) 60% of those who completed the task contributed to data of acceptable quality. Autism symptoms were measured by the Autism Diagnostic Observation Scale (ADOS-2). Results: 52.17% of participants completed the pop-out task and 80% of those provided evaluable eye-tracking data. The main reason for unsuccessful measurements was failed calibration. A logistic LASSO regression revealed that severity of disability was not a predictor of feasibility. One sample t-tests showed that participants directed their first looks more often towards faces than would be expected by chance, t(5) = 5.64, p =.002. This effect is also seen in neurotypical children. Results on whether children’s attention is also maintained on faces are mixed. Eye-movement behavior towards faces was not significantly correlated with ADOS-2 scores. Conclusion: According to our criteria, the task is not feasible since less than 70% completed it. However, in our sample, we observed that once calibration is successful, most participants completed the task and had data of acceptable quality. Therefore, we believe that eye-tracking can be a feasible measuring instrument if a better calibration method is found.Show less
Cooperation is an important part of living in a society and much research has been done to investigate the factors that promote cooperation between individuals. These studies report that...Show moreCooperation is an important part of living in a society and much research has been done to investigate the factors that promote cooperation between individuals. These studies report that cooperation could be influenced by factors like emotional facial expressions of happiness, and embarrassment, however, these are more for the adult population, and not enough for children. This study aimed to investigate the role of gender (boys and girls) as well as facial expressions of positive affect and embarrassment in the cooperative choices of children. To achieve these three hypotheses were drawn. Hypothesis one was cooperation would be higher among females than males. With the gender of the dyads randomized. Hypothesis two was, cooperation will be more when there are higher levels of positive affect. Hypothesis three was, cooperation will be more when there are higher levels of embarrassment. The participants were dyads of N = 80 children with ages ranging from 8 to 12 years old. They all performed an introduction task where half of them were asked to introduce themselves face-to-face and the other half with a black screen between them, thereby creating two visibility conditions: invisible and visible. Afterward, they played the modified version one-shot prisoner’s dilemma game. A chi-square test of independence was used to analyze hypothesis one. The results showed that there was no significant difference in cooperation between boys and girls, χ2 (1, N = 80) = .189, p = .664. A binomial logistic regression was used to analyze hypotheses two and three. The result showed that increased positive affect and embarrassment had no significant influence on the cooperative choices of children, with positive affect B=.05 SE=.18 OR = 1.06, p = .772 95% CI = [.74, 1.15] and embarrassment B=.05 SE=.07 OR = 1.05, p = .494, 95% CI = [0.91, 1.20] Therefore, it was concluded that although the results did not support the hypotheses, there may be several factors that could contribute to this, such as this study being done in real-life face-to-face settings versus the previous studies done with computer simulations or manipulations. Keywords: cooperation, emotional facial expression, positive affect, embarrassment, gender.Show less
Objective: This study examined the ability of the Short Form Informant Questionnaire of Cognitive Decline in the Elderly (SFIQCODE), to identify dementia in individuals with intellectual disability...Show moreObjective: This study examined the ability of the Short Form Informant Questionnaire of Cognitive Decline in the Elderly (SFIQCODE), to identify dementia in individuals with intellectual disability. Standard screening tools to assess for dementia, are not suitable for use with this population, more sensitive dementia screening tools are needed. This study also examined if participant levels of depression are linked to cognitive decline, and if there is a relationship between cognitive decline and difficulties performing activities of daily living (ADL). Methods: This study forms part of a longitudinal, observational study. Data gathered over two periods named Waves 3 and 4, included 740 participants over 40 years of age along with their carers who acted as respondents. ROC curve analysis was carried out to determine the sensitivity and specificity of the SFIQCODE to identify dementia in individuals with intellectual disability. Correlational analysis examined SFIQCODE scores in relation to levels of depression and binary logistic regression examined the association between SFIQCODE scores and ability to perform ADL. Results: ROC curve analysis of SFIQCODE scores in Wave 3 indicated sensitivity = 0.77 and specificity = 0.86 of the SFIQCODE to correlate with a dementia diagnosis at cut-off score of 3.3 and sensitivity = 0.60 and specificity = 0.96 at a cut-off of 4. In Wave 4, for a cut-off score of 3.3, the ROC curve analysis indicated sensitivity = 0.35 and specificity = 0.85. At a cut-off of 4, sensitivity = 0.25 and specificity = 0.94. In Wave 3 correlational analysis found r(135) = .33, p < .001 between carer reported depression and SFIQCODE scores. In Wave 4 the correlation between carer reported depression scores and SFIQCODE scores was r(50) = .26, p = .062. Logistic regression identified non-significant associations between SFIQCODE scores and ADL. Conclusions: The SFIQCODE has potential for identifying dementia in individuals with intellectual disability. The correlation between carer reported depression and SFIQCODE scores within waves suggests depression and cognitive decline may be linked in individuals with intellectual disability. No relationship was found between SFIQCODE scores and ability to perform ADL. This study identifies the importance of all-encompassing health-care for individuals with intellectual disability.Show less
