Association Between Challenging Behaviour and Sleep Problems in Adults Enrolled in the Global Angelman Syndrome Registry.

Angelman Syndrome (AS) is a rare genetic disorder that impacts 1:20,000 people. Challenging behaviour, such as severe injurious behaviour, aggression and frequent unprovoked episodes of laughter are a significant problem among adults with AS that adversely impacts an individual's quality of life. This study, for the first time, aims understand the characteristic of challenging behaviour, its frequency, and the factors associated with it in adults with AS. Data from participants with AS (N = 37; aged 18-46 years) registered with the Global Angelman Registry, were divided into challenging behaviour and non-challenging behaviour groups based on the presence or absence of 50% of the behaviours recorded in the registry. Descriptive statistics, chi-squared and t-test analysis were conducted to assess the impact of variables on challenging behaviour. Multiple regressions were conducted to investigate the predictors of challenging behaviour. 56% of the sample presented with challenging behaviour. Disorders of arousal, self-injury, behaviour dysregulation, repetitive behaviour, and the lack of physical therapy accounted for 59% of the variance of challenging behaviour in this population. It was found that challenging behaviour was very common in this population. A significant association was found between challenging behaviour and both sleep arousal and the lack of physical therapy. Sleep arousal and the lack of physical therapy were the key factors associated with challenging behaviour in this study. Targeted interventions are needed to decrease challenging behaviour and future research should focus on sleep interventions and increased opportunities for physical therapy.

Factors associated with sleep disturbances in children and adolescents with Angelman Syndrome.

BACKGROUND: Angelman Syndrome (AS) is a rare genetic disorder characterised by hyperactivity, overexcitability, developmental delays, and lack of speech. METHODS: This study used secondary data analysis to investigate sleep disturbances in children and adolescents (n = 212) who are enrolled in the Global Angelman Syndrome Registry. Participants were divided into two groups based on the presence or absence of sleep disturbance. The cut-off score of 40 on the Sleep Disturbance Scale for Children was used to indicate the presence or absence of sleep disturbances. Sleep disturbances and their association with co-occurring conditions were examined regarding challenging behaviour, language and communication, infancy history, gastrointestinal symptoms, and epilepsy. Multiple regression was then conducted to investigate possible predictors for sleep disturbances. RESULTS: Children and adolescents with AS, with and without sleep disturbances, differed considerably regarding anxiety. Sleep disturbances were significantly associated with an ability to use spoken words and computerised communication devices, and anxiety was a predictor of sleep disturbances. CONCLUSION: Future research is necessary to replicate this novel research, and to advance the clinical treatment of sleep disturbances in children and adolescents with AS.

Adaptive living skills, sleep problems, and mental health disorders in adults with 22q11.21 deletion syndrome.

BACKGROUND: 22q11.21 deletion syndrome (22q11DS) is a neurodevelopmental syndrome caused by a microdeletion of genes at the 22q11.21 locus. It has a prevalence of 1:2000. This study investigated the prevalence of adaptive living skills, sleep problems, and mental health disorders in adults with 22q11DS and examined the relationship between these factors. METHODS: Parents with an adult son or daughter with 22q11DS completed the following: A bespoke Demographic Information Questionnaire, Sleep Questionnaire (SQ-SP), Psychopathology in Autism Checklist (PAC), and Activities of Daily Living (ADL) scale. Descriptive statistics, correlations, and one-way between groups analysis of variance (ANOVA) were conducted. RESULTS: Mental health difficulties, sleep problems, and low levels of adaptive living skills are prevalent in adults with 22q11DS. Strong positive correlations were identified between sleep problems, depression, and anxiety subscale scores and moderate negative correlations between depression, psychosis, and activities of daily living skills. CONCLUSION: Adults with 22q11DS need screening and treatment for mental health and sleep problems.

Relationships between challenging behavior and gastrointestinal symptoms, sleep problems, and internalizing and externalizing symptoms in children and adolescents with Angelman syndrome.

BACKGROUND: Angelman syndrome (AS), is a rare genetic disorder. This study investigated the relationship between parent-reported comorbid symptoms including gastrointestinal symptoms, sleep problems, internalizing symptoms, and behavior problems in children and adolescents with AS. METHOD: Parents of 98 children and adolescents with AS completed the Gastrointestinal Symptom Inventory, Children's Sleep Habits Questionnaire, Child Behavior Checklist, Social Communication Questionnaire, and the Behavior Problem Inventory-Short Form. Data were analyzed using descriptive statistics, Pearson's correlation coefficients, and hierarchical multiple regressions. RESULTS: There was a high frequency of GI symptoms (99%), sleep problems (95.9%), challenging behavior (98%), internalizing symptoms (38%), and 72.4% of children and adolescents presented with ASD symptoms. Self-injurious behavior (SIB), aggressive/destructive behavior, and the frequency of stereotyped behavior positively correlated with GI symptoms and sleep problems and it was moderately negatively associated with age. Internalizing symptoms and age were positively associated with SIB. Aggression was significantly related to gender, but not the presence of ASD symptoms. CONCLUSIONS: Findings highlight the relationships between comorbid conditions. They may lead to a deeper understanding of how comorbidities present in children and adolescents with Angelman Syndrome.

Examining the Relationship Between Sleep Quality, Social Functioning, and Behavior Problems in Children with Autism Spectrum Disorder: A Systematic Review.

Over forty percent of autistic children experience poor sleep quality, and social interaction difficulties are a core characteristic of autism. However, the relationship between sleep quality and social functioning and behavior remains poorly understood. This systematic review examined the evidence concerning the impact of sleep quality on the social functioning and behavior problems in autistic children and adolescents. It also identified key related factors and evaluated how this issue has been researched to date. Seven key journals were hand-searched and five databases were systematically searched, using keywords. Titles and abstracts of 4123 items were screened against eligibility criteria by two researchers. Relevant studies were retained if they were peer-reviewed empirical papers, published in English between 2000 and 2021. Then, the full text of 97 papers was screened and if they met the eligibility criteria, their reference lists were hand-searched. Forty-six studies were included in the final review. Data were systematically extracted and two authors critically appraised the strengths and weaknesses of studies using Critical Appraisal Skills Programme tools. Key themes were identified, because a meta-analysis was not possible due to the studies' heterogeneity. The review identified that sleep quality and social functioning are associated with one another and there is a small amount of evidence that a bi-directional causal relationship may exist. Evidence suggests that several nights of suboptimal sleep duration and a lack of deep continuous sleep negatively impact externalizing and internalizing behavior. Sleep quality is also reduced by anxiety and sensory sensitivity. However, longitudinal studies with larger samples are needed to establish causality. Future research needs to examine confounding factors and to develop consensus regarding best-practice processes for the objective measurement of sleep with autistic children. Additional research also needs to further examine the consequences of poor sleep quality on internalizing behavior, and the impact of socio-cultural practices.

Gastrointestinal Symptoms in Autism Spectrum Disorder: A Systematic Review.

This systematic review aims to offer an updated understanding of the relationship between gastrointestinal symptoms (GIS) and autism spectrum disorder (ASD) in children and adolescents. The databases PsycINFO, Medline, Cinahl, and ERIC were searched using keywords, and relevant literature was hand-searched. Papers (n = 3319) were systematically screened and deemed eligible if they were empirical studies published in English since 2014 and measured the GIS of individuals with ASD who were under 18 years old. Thirty studies were included in the final review. The study findings were synthesized under eight themes, including the prevalence and nature of GIS and their relationship with developmental regression, language and communication, ASD severity, challenging behavior, comorbid psychopathology, sleep problems, and sensory issues. The review found that GIS were common and that there was contradictory evidence concerning their relationship with co-occurring conditions. It also identified evidence of some causal relationships that support the existence of the gut-immune-brain pathways. Future research needs to use large prospective designs and objective and standardized GIS measurements to provide a nuanced understanding of GIS in the context of ASD.

The Usability and Impact of a Low-Cost Pet Robot for Older Adults and People With Dementia: Qualitative Content Analysis of User Experiences and Perceptions on Consumer Websites.

BACKGROUND: Worldwide, populations are aging exponentially. Older adults and people with dementia are especially at risk of social isolation and loneliness. Social robots, including robotic pets, have had positive impacts on older adults and people with dementia by providing companionship, improving mood, reducing agitation, and facilitating social interaction. Nevertheless, the issue of affordability can hinder technology access. The Joy for All (JfA) robotic pets have showed promise as examples of low-cost alternatives. However, there has been no research that investigated the usability and impact of such low-cost robotic pets based on perceptions and experiences of its use with older adults and people with dementia. OBJECTIVE: The aim of our study was to explore the usability and impact of the JfA robotic cat, as an example of a low-cost robot, based on perceptions and experiences of using the JfA cat for older adults and people with dementia. METHODS: We used a novel methodology of analyzing a large volume of information that was uploaded by reviewers of the JfA cat onto online consumer review sites. Data were collected from 15 consumer websites. This provided a total of 2445 reviews. Next, all reviews were screened. A total of 1327 reviews that contained information about use of the JfA cat for older adults or people with dementia were included for analysis. These were reviews that contained terms relating to "older adults," "dementia," and "institutional care" and were published in the English language. Descriptive statistics was used to characterize available demographic information, and textual data were qualitatively analyzed using inductive content analysis. RESULTS: Most reviews were derived from consumer sites in the United States, and most reviewers were family members of users (ie, older adults and people with dementia). Based on the qualitative content analysis, 5 key themes were generated: prior expectations, perceptions, meaningful activities, impacts, and practicalities. Reviewers had prior expectations of the JfA cat, which included circumstantial reasons that prompted them to purchase this technology. Their perceptions evolved after using the technology, where most reported positive perceptions about their appearance and interactivity. The use of the robot provided opportunities for users to care for it and incorporate it into their routine. Finally, reviewers also shared information about the impacts of device and practicalities related to its use. CONCLUSIONS: This study provides useful knowledge about the usability and impact of a low-cost pet robot, based on experiences and perceptions of its use. These findings can help researchers, robot developers, and clinicians understand the viability of using low-cost robotic pets to benefit older adults and people with dementia. Future research should consider evaluating design preferences for robotic pets, and compare the effects of low-cost robotic pets with other more technologically advanced robotic pets.

Attention-Deficit/Hyperactivity Disorder Symptoms, Gastrointestinal Symptoms, Sleep Problems, Challenging Behavior, Adaptive Behavior, and Quality of Life in Children and Adolescents with Autism Spectrum Disorder.

This study investigated the relationship between sleep, gastrointestinal symptoms, challenging behavior, adaptive behavior, and quality of life between children and adolescents with autism spectrum disorder (ASD), with and without attention-deficit/hyperactivity disorder (AD/HD) symptoms. Parents of 118 children and adolescents with ASD completed the Conners Early Childhood Rating Scale-Parent Short Form or the Conners 3-Parent Short Form, Children's Sleep Habits Questionnaire, Gastrointestinal Symptom Inventory, Behavior Problems Inventory-Short Form, Pediatric Quality of Life Inventory and the Vineland Adaptive Behavior Scales, Second Edition. The ASD group and the ASD with AD/HD groups differed significantly in sleep problems, gastrointestinal symptoms, and quality of life. Regressions indicated that AD/HD symptoms accounted for a small proportion of the variance for the differences in sleep problems and quality of life. AD/HD symptoms contribute to the complex needs of individuals with ASD. Research is necessary to investigate how these symptoms exacerbate comorbidities.

The Co-Occurrence of Autism Spectrum Disorder and Cerebral Palsy and Associated Comorbid Conditions in Children and Adolescents.

BACKGROUND: Comorbidity is the co-occurrence of two or more disorders in the same person. AIM: This study investigated the frequency of comorbid conditions, in children and adolescents, with autism spectrum disorder (ASD), cerebral palsy (CP), and a comorbid diagnosis of ASD and CP. METHOD: Ninety-six children and adolescents with ASD, CP, and both ASD and CP aged between 4 and 18 years participated in this study. Parents completed the Gastrointestinal Symptom Inventory, Children's Sleep Habits Questionnaire, Child Behavior Checklist, Social Communication Questionnaire, and the Vineland Adaptive Behavior Scales. RESULTS: Results of ANOVA analyses revealed significant group differences in sleep problems, social communication difficulties, and adaptive behavior. Regression analysis found that the presence of an intellectual disability significantly predicted levels of adaptive behavior. CONCLUSION: This research demonstrated the importance of studying comorbidities in children and adolescents with CP alone, ASD alone, and combined ASD and CP.

An examination of adaptive behavior and functional outcomes in adults with 22q11.2 deletion syndrome: A parental perspective.

22q11.2 Deletion Syndrome (22q11DS) is a genetic syndrome caused by a chromosomal microdeletion. It affects approximately 1 in 850-992 pregnancies, and its clinical manifestations include congenital heart disease, gastrointestinal symptoms, and psychiatric illnesses. The study examined the relationship between adaptive behavior and functional outcomes, educational attainment, employment, and independent living, and whether age, gender, intellectual disability, presence of psychiatric disorder, and close friendships could predict levels of adaptive behavior. Parents of adults with 22q11DS (n = 101; 48 male and 54 female) completed the Waisman Activities of Daily Living Scale, demographic details, and questions elicited employment, education, and relationships status. Analysis conducted in SPSS, included descriptive statistics, measures of association, Analysis of Variance, logistic and linear regressions. Differences in levels of overall adaptive behavior were found regarding employment and living status, but not in educational attainment. Having close friendships was associated with adaptive behavior as well as the likelihood of living independently. Further research is needed, ideally using prospective designs and purposive sampling strategies. This needs to examine how social and communication deficits impact relationship building and how they are affected by the clinical manifestations of 22q11DS. It also needs to focus on how different social structures interface with levels of adaptive behavior.

Examining the Utility of the DESCARTE Model for Case Study Research.

The DESCARTE model aims to guide health care researchers on the design, conduct, and reporting of case studies. The current article critically appraises the utility of the model and provides an example of its application to a multiple case study that examined the effect of MARIO, a social robot, on the resilience of people with dementia. The DESCARTE model is explained, and its strengths and the challenges encountered during its application are discussed. DESCARTE provides a structured framework that promotes quality in case study research by requiring the researcher to articulate philosophical cohesiveness, ethical considerations, reflexivity, and the strategies used during data integration and throughout data analysis. However, the model does not prompt the development of research questions or methods to promote rigor and reflexivity throughout the case study. A task list and additional guiding questions are provided to address these deficits and to improve the utility of the DESCARTE model. [Research in Gerontological Nursing, 14(2), 60-68.].

The Perceptions of People with Dementia and Key Stakeholders Regarding the Use and Impact of the Social Robot MARIO.

People with dementia often experience loneliness and social isolation. This can result in increased cognitive decline which, in turn, has a negative impact on quality of life. This paper explores the use of the social robot, MARIO, with older people living with dementia as a way of addressing these issues. A descriptive qualitative study was conducted to explore the perceptions and experiences of the use and impact of MARIO. The research took place in the UK, Italy and Ireland. Semi-structured interviews were held in each location with people with dementia (n = 38), relatives/carers (n = 28), formal carers (n = 28) and managers (n = 13). The data was analyzed using qualitative content analysis. The findings revealed that despite challenges in relation to voice recognition and the practicalities of conducting research involving robots in real-life settings, most participants were positive about MARIO. Through the robot's user-led design and personalized applications, MARIO provided a point of interest, social activities, and cognitive engagement increased. However, some formal carers and managers voiced concern that robots might replace care staff.

Fostering the Resilience of People With Dementia: A Narrative Literature Review.

Background: Resilience is a process through which people use resources to adapt to adversity. Interventions aiming to support resilience in people with dementia have been developed. However, the optimal content, structure and impact of these interventions is unclear. This literature review explores the factors through which interventions foster resilience in people with dementia and examines their efficacy. Methods: Eight databases were searched systematically, for literature published from 2000 to 2019. Following the removal of duplicate articles, the titles and abstracts of 6,749 articles were screened. Articles were selected if they: reported empirical studies in English; focused on resilience; involved people with dementia and psychosocial interventions. The full text of 53 articles were examined and three studies, reported in six papers, were included in the final review. Data were systematically extracted, and two authors critiqued the studies using the Critical Appraisal Skills Programme check lists. The studies were examined to determine how resilience was defined and operationalized and their findings were synthesized using the theoretical resilience framework. Results: Five interventions aiming to foster resilience were identified: Dementia Advisors; Peer Support Network Services; Visual Arts Enrichment Activities; Memory Makers; and Early-Stage and Beyond Community Activities. All studies defined resilience as a process and most involved people with mild dementia who had family carers. The interventions impacted resilience by reducing the adversity of stigma and social isolation; increasing personal and social resources, providing stigma-free space and reciprocal support. Interventions empowered people with dementia, increasing their self-esteem and self-worth. Resilience can be fostered both during, and after interventions. However, the efficacy of interventions could not be determined because the research designs utilized did not measure efficacy. Conclusions: Interventions need facilitators to ensure they are strength-based, person-centered and they enable reciprocal social interactions. Future research needs to develop interventions that aim to foster the resilience of people with dementia who lack family carers and/or have more advanced dementia through meaningful activities that are identified by people with dementia as important to their resilience. Robust methodologies, including randomized controlled trials should be used to measure effectiveness and explore the impact of interventions regarding the: interplay between individual and community resources; the importance of reciprocity; and temporal aspects of resilience.

Evaluation of a Companion Robot for Individuals With Dementia: Quantitative Findings of the MARIO Project in an Irish Residential Care Setting.

The current study focuses on the short-term effect of MARIO, a social robot, on quality of life, depression, and perceived social support in persons with dementia (PWD) and evaluates their acceptability of MARIO. Ten PWD in one nursing home took part in a 4-week pilot study, where each participant had up to 12 sessions with MARIO. Sessions comprised engagement in music, news, reminiscence, games, and calendar applications. Standardized questionnaires were administered before and after the 4-week period. Participants had a sustained interest in MARIO during their interactions and an acceptance of MARIO's appearance, sound, and applications. Consequently, participants spent more time socially engaged. No statistically significant differences were found in quality of life, depression, and perceived social support. PWD can engage with a social robot in a real-world nursing home. Future research should incorporate a larger sample and longer intervention period. [Journal of Gerontological Nursing, 45(7), 36-45.].

MARIO Project: Validation and Evidence of Service Robots for Older People with Dementia.

BACKGROUND: In the EU funded MARIO project, specific technological tools are adopted for the people living with dementia (PLWD). In the final stage of the project, a validation of the MARIO companion robot was performed from August to October 2017. OBJECTIVE: The aims of the present study are: 1) to illustrate the key results and evidence obtained in the final evaluation phase of the project across the three different pilot sites; 2) to assess the engagement dimensions of the PLWD who interacted with the MARIO robot; and 3) to assess the acceptability and efficacy of the MARIO companion robot on clinical, cognitive, neuropsychiatric, affective and social aspects, resilience, quality of life in PLWD, and burden level of the caregivers. METHODS: 38 people (M = 14; F = 24) with Alzheimer's disease were screened for eligibility and all were included. The following tests were administered Pre and Post interactions with MARIO: Observational Measurement of Engagement (OME), Mini-Mental State Examination (MMSE), Clock Drawing Test (CDT), Frontal Assessment Battery (FAB), Neuropsychiatric Inventory (NPI), Cornell Scale for Depression in Dementia (CSDD), Multidimensional Scale of Perceived Social Support (MSPSS), 14-item Resilience Scale (RS-14), Quality of Life in Alzheimer's Disease (QOL-AD), Caregiver Burden Inventory (CBI), Tinetti Balance Assessment (TBA), and Comprehensive Geriatric Assessment (CGA) was carried out. RESULTS: In Post-MARIO interactions, significant improvements were observed in RS-14 (p = 0.020).Considering the age of the people, PLWD with 68-76 years perceived that they had major social support (MSPSS Total: p = 0.016) and friends to support them (MSPSS Fri: p = 0.014). Indeed, the younger people (55-67 years) were less depressed (CSDD: p = 0.033), and more resilient (RS-14: p = 0.003). The people aged 77-85 years perceived they had major family support (MSPSS Fam: p = 0.018). The participants were gender and education matched without any statistically significant difference. CONCLUSION: MARIO may be a useful tool in mitigating depression and loneliness, while enhancing social connectedness, resilience, and overall quality of life for people with dementia.

Interacting with Dementia: The MARIO Approach.

MARIO is a companion robot that aims to help people with dementia (PWD) to battle isolation and loneliness by enabling them to stay socially active by providing a number of applications focused on hobbies (music, movies, etc), staying engaged with communities (reading headlines, reading local twitter feeds etc.) and staying connected with family and friends (telephoning them, reading their news from twitter, etc.). This paper presents the results from the initial trials of MARIO interacting with PWD involving a limited set of applications. It confirms some of the challenges hypothesized at the outset of the study and provides guidelines for future development work.

Investigating the Effect of Social Robot Embodiment.

The experiment described in this paper is an early assessment to identify if the embodiment of a verbal and visual user interaction system in a robot is more effective in people with dementia than when using the same system in a simple laptop. This study provides input for the robot's design.