Dawn Adams

The expression and assessment of emotions and internal states in individuals with severe or profound intellectual disabilities.

The expression of emotions and internal states by individuals with severe or profound intellectual disabilities is a comparatively under-researched area. Comprehensive or standardized methods of assessing or understanding the emotions and internal states within this population, whose ability to communicate is significantly compromised, do not exist. The literature base will be discussed and compared to that applicable to the general population. Methods of assessing broader internal states, notably depression, anxiety, and pain within severe or profound intellectual disabilities are also addressed. Finally, this review will examine methods of assessing internal states within genetic syndromes, including hunger, social anxiety, and happiness within Prader-Willi, Fragile-X and Angelman syndrome. This will allow for identification of robust methodologies used in assessing the expression of these internal states, some of which may be useful when considering how to assess emotions within individuals with intellectual disabilities.

The relationship between acquired impairments of executive function and behaviour change in adults with Down syndrome

BACKGROUND The latter stages of dementia in individuals with Down syndrome are well documented; however, earlier cognitive and behavioural changes have only recently been described. Holland et al. suggested such early signs of dementia in this population are behavioural and are similar to those seen in frontotemporal dementia, but there is, as yet, no evidence to determine whether such behavioural changes are associated with a declines in specific cognitive functions, including those associated with the frontal lobes. METHODS A longitudinal design of three time points across 16 months was used across 30 adults with Down syndrome aged 30 years and over. Measures of cognition (Neuropsychological Assessment of Dementia in Individuals with Intellectual Disabilities), receptive language (British Picture Vocabulary Scales), adaptive behaviour (Vineland Adaptive Behavior Scales), behavioural excesses and behavioural deficits (Assessment for Adults with Developmental Disabilities) and measures of executive functioning were completed at each time point. Using a data-driven method, cognitive deterioration was determined using the Reliable Change Index on performance on the Neuropsychological Assessment of Dementia in Individuals with Intellectual Disabilities across the duration of the study. Performance on the remaining measures were then compared between those with (n = 10) and those without (n = 20) cognitive deterioration. RESULTS Only individuals with cognitive deterioration showed decreases on measures of executive function and significant changes in behaviour across the duration of the study, which was not solely due to declines in memory. There were no changes between the groups on levels of adaptive behaviour. CONCLUSIONS Even in the early stages of cognitive deterioration, specific behavioural changes can be identified that are not present in those without cognitive deterioration. The differing effects of cognitive deterioration on behavioural excesses and deficits are discussed in relation to potentially differing underlying neuropathological causes.

Behavioural characteristics associated with dementia assessment referrals in adults with Down syndrome

BACKGROUND Behavioural changes associated with dementia in Down syndrome are well documented, yet little is known about the effect of such behaviours on carers and referral. By comparing the behavioural and cognitive profiles of individuals referred for a dementia assessment with those of individuals not referred, some insight can be gained into behavioural characteristics that initiate referral for specialist support or interventions. METHOD Forty-six adults with Down syndrome were divided into two groups dependent upon method of entry into the study; post-referral to a specialist service for older adults with intellectual disabilities and Down syndrome for a dementia assessment (n = 17) or after receiving information sent out to day centres and residential homes (n = 29). These groups were compared on established measures of dementia alongside two informant measures of behaviour. RESULTS Those referred for a dementia assessment evidenced scores indicative of cognitive decline on both informant and direct Neuropsychological Assessments and showed more behavioural excesses, but not deficits, and lower socialisation and coping skills than those in the comparison group. Carers of those referred for a dementia assessment reported a greater impact of behavioural excesses on staff than on the individual showing the behaviour in contrast to the comparison group. CONCLUSION The behavioural differences between those referred and the comparison group suggest that two factors are involved in the instigation of a referral for a dementia assessment: the nature of the behavioural presentation (excesses rather than deficits) and the effect of that behavioural change upon the care staff.

Age related change in social behavior in children with Angelman syndrome

We investigated the relationship between age and laughing and smiling in children with Angelman syndrome. Twenty‐four children with Angelman syndrome were exposed to three experimentally manipulated conditions: proximity only, restricted social interaction, and social interaction. Children smiled the most in the social interaction condition and the least in the proximity only condition confirming the effect of social interaction on these behaviors. There was a decline in smiling and laughing in the oldest group (13.4–15.9 years) only in the social interaction condition. This trajectory of a decline in resource soliciting behaviors with age is consistent with predictions based on kinship theory.

Brief Report: A Longitudinal Study of Excessive Smiling and Laughing in Children with Angelman Syndrome

AbstractElevated laughing and smiling is a key characteristic of the Angelman syndrome behavioral phenotype, with cross-sectional studies reporting changes with environment and age. This study compares levels of laughing and smiling in 12 participants across three experimental conditions [full social interaction (with eye contact), social interaction with no eye contact, proximity only] at two data points. No differences were noted in frequency of laughing and smiling over time in any condition. However, with age as a covariate, the frequency of laughing and smiling decreased over time in the full social interaction (with eye contact) condition only. As this is the first longitudinal study to explore these behaviors in Angelman syndrome, the results suggest a gene–environment–time interaction within the behavioral phenotype.

Measurement tools for mental health problems and mental well-being in people with severe or profound intellectual disabilities : a systematic review

Mental health problems affect people with intellectual disabilities (ID) at rates similar to or in excess of the non-ID population. People with severe ID are likely to have persistent mental health problems. In this systematic review (PROSPERO 2015:CRD42015024469), we identify and evaluate the methodological quality of available measures of mental health problems or well-being in individuals with severe or profound ID. Electronic searches of ten databases identified relevant publications. Two reviewers independently reviewed titles and abstracts of retrieved records (n=41,232) and full-text articles (n=573). Data were extracted and the quality of included papers was appraised. Thirty-two papers reporting on 12 measures were included. Nine measures addressed a broad spectrum of mental health problems, and were largely observational. One physiological measure of well-being was included. The Aberrant Behavior Checklist, Diagnostic Assessment for the Severely Handicapped Scale-II and Mood, Interest and Pleasure Questionnaire are reliable measures in this population. However, the psychometric properties of six other measures were only considered within a single study - indicating a lack of research replication. Few mental health measures are available for people with severe or profound ID, particularly lacking are tools measuring well-being. Assessment methods that do not rely on proxy reports should be explored further.

Anxiety-related symptomatology in young children on the autism spectrum:

Anxiety symptomatology is frequently reported in autistic children, and the prevalence of anxiety disorder is estimated at around 40%. However, most studies have focused upon children of age 8 years or above, so little is known about early signs of anxiety in younger children with autism. This study sought to describe anxiety-related symptomatology in 95 5- to 6-year-old autistic children using the Anxiety Scale for Children with Autism Spectrum Disorder. Wide variability was found in levels of symptomatology with the most frequently reported items within the ‘uncertainty’ subscale and the least frequently reported items in the ‘anxious arousal’ subscale. Comparisons of those with scores less than or greater than 70 on adaptive behaviour suggests some influence of ability on presentation of anxiety-related symptomatology.

School-related anxiety symptomatology in a community sample of primary-school-aged children on the autism spectrum

Although it is recognized that the prevalence of anxiety is elevated in children with autism spectrum disorder, there has been very limited research exploring such anxiety in school contexts. As a result, there is limited detailed information for teachers or educators on how anxiety in autism may present in the school setting for children on the autism spectrum. The aims of this study were to (a) report the profile of results on a measure of school anxiety in a community sample of children on the autism spectrum, (b) investigate whether scores on this measure differed with child variables or enrollment into a mainstream or special school, and (c) document the level of agreement between teacher-reported and parent-reported anxiety symptoms. Teachers of 92 children aged 5-12 completed a questionnaire pack including the School Anxiety Scale-Teacher Rating (SAS-TR). Elevated levels of anxiety (above the SAS-TR total anxiety clinical cut-off) were noted in 21.7% of the sample, with a larger proportion of children scoring above the generalized anxiety cut-off (27.2%) than the social anxiety cut-off (14.1%). Older participants (U = 744, p = .02, η2 = 0.06) and those attending mainstream schools (U = 661, p = .02, η2 = 0.06) had significantly higher scores on the generalized, but not the social, anxiety subscales, with effect sizes suggesting a medium effect. The results highlight the need for further, more detailed research into the presentation and impact of school anxiety in children with autism attending both mainstream and special schools.

Brief Report: Repetitive Behaviour Profiles in Williams Syndrome: Cross Syndrome Comparisons with Prader-Willi and Down Syndromes.

This study describes the profile of repetitive behaviour in individuals with Williams syndrome, utilising cross-syndrome comparisons with people with Prader–Willi and Down syndromes. The Repetitive Behaviour Questionnaire was administered to caregivers of adults with Williams (n = 96), Prader–Willi (n = 103) and Down (n = 78) syndromes. There were few group differences, although participants with Williams syndrome were more likely to show body stereotypies. Individuals with Williams syndrome also showed more hoarding and less tidying behaviours than those with Down syndrome. IQ and adaptive ability were negatively associated with repetitive questioning in people with Williams syndrome. The profile of repetitive behaviour amongst individuals with Williams syndrome was similar to the comparison syndromes. The cognitive mechanisms underlying these behaviours in genetic syndromes warrant further investigation.

Parent descriptions of the presentation and management of anxiousness in children on the autism spectrum

The complex interaction between anxiety and autism has led to debate about the presentation of anxiety in individuals on the spectrum and questions about the extent to which traditional checklists assess the entire range of symptomatology. Moreover, studies to date have not explored how the presentation of anxiety may differ between settings. Through a combination of open-ended questions, closed questions and standardised measures, parents of 173 children (aged 6–13) on the autism spectrum provided (1) descriptors of their child’s anxiety at home, school and in the community and (2) strategies used to reduce their child’s anxiety in each setting. Over half (52.6%) felt their child was anxious at home, 77.6% at school and 76.2% in the community. Parents reported differing presentations of anxiety between settings, with the majority of descriptions relating to observable, behavioural changes (e.g. hides/shuts down, repetitive behaviours) rather than cognitive or physiological signs. Parents also reported using different strategies across settings. The use of open-ended questions allowed the identification of signs of anxiety not explored within traditional questionnaires and highlighted the potential for signs to vary across settings. This knowledge is critical to inform the development or adaptations of anxiety measures and interventions.