Severe Levels Of Intellectual Disability Research Articles

The relationship between antiepileptic drug load and challenging behaviors in older adults with intellectual disability and epilepsy

Antiepileptic drugs (AEDs) may affect mood and behavior in people with epilepsy and intellectual disability. A high AED load, derived from AED polytherapy and/or high doses of AEDs, has been suggested to be a risk factor for behavioral side effects. Data were drawn from Wave 3 of the Intellectual Disability Supplement to the Irish Longitudinal Study on Ageing (IDS-TILDA). The Behavior Problems Inventory Short Form (BPI-S) was used to assess challenging behaviors. AED load was calculated and median AED loads obtained. Non-parametric tests and binary logistic regression were performed to determine the relationship between AED load and challenging behaviors. Of participants with a reported diagnosis of epilepsy who were taking a regular AED and had completed BPI-S (n = 142), 62.7% (n = 89) exhibited challenging behaviors. Challenging behavior was found to be more prevalent in those with more severe levels of intellectual disability (p < 0.001). Aggressive/destructive behavior and stereotyped behavior were significantly more likely in participants living in residential/campus settings. For participants with a severe/profound intellectual disability, a significantly higher median AED load was found for participants exhibiting aggressive/destructive behavior and self-injurious behavior (SIB) compared to participants not exhibiting these behaviors, indicating a high AED load may contribute to some behavioral problems in this population group. However, many factors can influence behavioral outcomes, creating difficulties in determining those that are associated and the nature of the association. Careful monitoring of AED load, together with increased vigilance for breakthrough behavioral issues is essential for dealing with these complex cases. Larger studies are needed to account for the potential confounding factors.

Prevalence of Sexual Abuse in Adults with Intellectual Disability: Systematic Review and Meta-Analysis.

This study presents the results of a systematic review on the prevalence of sexual abuse experienced in adulthood by individuals with intellectual disability. An electronic and manual search of academic journals was performed on four databases via EBSCO Host: Academic Search Complete, PsycINFO, Medline, CINAHL Full-Text. In addition, PubMed, ProQuest, and Web of Science (core collection) were searched. After an initial selection of 1037 documents, 25 articles remained for quantitative synthesis. The combined prevalence of sexual abuse in adults with intellectual disability was 32.9% (95% CI: 22.7–43.0) and sensitivity analysis revealed that the prevalence was not outweighed by a single study. Overall, the United Kingdom had the highest prevalence (r = 34.1%), and the USA had the lowest (r = 15.2%). The overall prevalence in females was lower (r = 31.8%) than that in males (r = 39.9%). Subgroup analyses revealed that prevalence of sexual abuse was higher in institutionalized individuals. The most prevalent profile of abuser is of a peer with intellectual disability. Prevalence increases from mild to severe levels of intellectual disability and decreases in profound levels. It is also more prevalent when the informant is the individual with intellectual disability than when someone else reports abuse. In sum, one in three adults with intellectual disability suffers sexual abuse in adulthood. Special attention should be paid for early detection and intervention in high risk situations.

Case study: identification of anxiety and subsequent intervention in an adolescent male with autism, severe intellectual disability and self-injurious behaviour

Introduction: Anxiety disorders are highly prevalent in individuals with autism spectrum disorder (ASD), but knowledge is limited regarding identification and treatment of these disorders in individuals with ASD and more severe levels of intellectual disability (ID). The current case study aims to explore and describe the inpatient, psychiatric assessment in an adolescent male with ASD, severe ID and self-injurious behaviour (SIB) who was diagnosed with a co-occurring anxiety disorder. The study further aims to explore the implications of this diagnosis for subsequent intervention and care, including management of SIB. Materials and methods: Case study including multimodal, psychiatric assessment and subsequent intervention. Results: Following changes in care strategies attempting to incorporate the understanding of anxiety/trauma as contributing to SIB, a reduction of SIB was observed, and this reduced frequency was maintained when the patient was discharged from the inpatient setting. Conclusions: Though no causal inferences are possible, these experiences indicate that further research is needed regarding possible associations between SIB and anxiety in individuals with ASD, including implications for treatment. Experiences from the current case further indicate that it is possible to recognize and diagnose anxiety disorder in complex cases involving ASD, severe ID, limited verbal language skills, and severe SIB.

Mother–child dyadic co-regulation in children with intellectual disability: A comparison among dyads with children with chromosome 14 aberrations, Down syndrome and typical development

ABSTRACTBackground: The present study aimed at investigating mother–child dyadic co-regulation patterns in dyads with children with intellectual disability (ID).Method: 24 children paired for developmental age and vocabulary size (8 with chromosome 14 aberrations, 8 with Down syndrome, and 8 with typical development) and their mothers participated in the study. The Revised-Relational Coding Scheme was employed to assess mother–child dyadic co-regulation.Results: The dyads with children with ID appeared to have significantly fewer episodes of symmetric-patterns (i.e., situations in which mother and child share a mutual focus of attention) than those with typically developing children. In addition, the dyads with children with chromosome 14 aberrations showed the highest proportion of unengaged patterns (i.e., situations in which the partners do not interact with one another).Conclusions: A severe level of ID in combination with autistic traits, as frequently found in chromosome 14 aberrations, could lead to a less optimal mother–child interaction.

Validation of intellectual disability coding through hospital morbidity records using an intellectual disability population-based database in Western Australia

ObjectivesTo investigate how well intellectual disability (ID) can be ascertained using hospital morbidity data compared with a population-based data source.Design, setting and participantsAll children born in 1983–2010 with a hospital...

Prevalence of Dysphagia in People With Intellectual Disability: A Systematic Review.

Dysphagia (feeding and swallowing disorder) is associated with serious health complications and psychosocial sequelae. This review summarizes international research relating to the prevalence of dysphagia in people with intellectual disability. Studies published from 1990 to July 2016 were identified using Medline, Cinahl, PsycINFO, Web of Science, email requests, and cross-citations. Twenty studies were identified. Dysphagia in people with intellectual disability appears to be associated with more severe levels of intellectual disability, comorbid cerebral palsy, and motor impairments. However, further research with representative samples of people with intellectual disability using adequate methods of assessment are required in order to provide more precise prevalence estimates and clarify factors that may be associated with dysphagia in this population.

Feasibility and Reliability of Tests Measuring Health-Related Physical Fitness in Children With Moderate to Severe Levels of Intellectual Disability.

Physical fitness is an important marker for health. In this study we investigated the feasibility and reliability of health-related physical fitness tests in children with moderate to severe levels of intellectual disability. Thirty-nine children (2-18 yrs) performed tests for muscular strength and endurance, the modified 6-minute walk test (6mwt) for cardiorespiratory fitness, and body composition tests, and 30-97% of the tests were successfully completed. Short-term test-retest reliability of all tests was good (Intraclass Correlation Coefficient [ICC] > .8), long-term test-retest reliability was good for most tests (ICC > .7), but low ICCs were found for most strength tests. Measuring body composition and cardiorespiratory fitness is feasible and reliable. Measuring muscle endurance is fairly feasible and reliable.

Developmental trajectories in 22q11.2 deletion.

Chromosome 22q11.2 deletion syndrome (22q11.2DS), a neurogenetic condition, is the most common microdeletion syndrome affecting 1 in 2,000-4,000 live births and involving haploinsufficiency of ∼50 genes resulting in a multisystem disorder. Phenotypic expression is highly variable and ranges from severe life-threatening conditions to only a few associated features. Most common medical problems include: congenital heart disease, in particular conotruncal anomalies; palatal abnormalities, most frequently velopharyngeal incompetence (VPI); immunodeficiency; hypocalcemia due to hypoparathyroidism; genitourinary anomalies; severe feeding/gastrointestinal differences; and subtle dysmorphic facial features. The neurocognitive profile is also highly variable, both between individuals and during the course of development. From infancy onward, motor delays (often with hypotonia) and speech/language deficits are commonly observed. During the preschool and primary school ages, learning difficulties are very common. The majority of patients with 22q11.2DS have an intellectual level that falls in the borderline range (IQ 70-84), and about one-third have mild to moderate intellectual disability. More severe levels of intellectual disability are uncommon in children and adolescents but are more frequent in adults. Individuals with 22q11.2DS are at an increased risk for developing several psychiatric disorders including attention deficit with hyperactivity disorder (ADHD), autism spectrum disorder (ASD), anxiety and mood disorders, and psychotic disorders and schizophrenia. In this review, we will focus on the developmental phenotypic transitions regarding cognitive development in 22q11.2DS from early preschool to adulthood, and on the changing behavioral/psychiatric phenotype across age, on a background of frequently complex medical conditions.

Neonatal hypocalcemia, neonatal seizures, and intellectual disability in 22q11.2 deletion syndrome

Hypocalcemia is a common endocrinological condition in 22q11.2 deletion syndrome. Neonatal hypocalcemia may affect neurodevelopment. We hypothesized that neonatal hypocalcemia would be associated with rare, more severe forms of intellectual disability in 22q11.2 deletion syndrome. We used a logistic regression model to investigate potential predictors of intellectual disability severity, including neonatal hypocalcemia, neonatal seizures, and complex congenital heart disease, e.g., interrupted aortic arch, in 149 adults with 22q11.2 deletion syndrome. Ten subjects had moderate-to-severe intellectual disability. The model was highly significant (P < 0.0001), showing neonatal seizures (P = 0.0018) and neonatal hypocalcemia (P = 0.047) to be significant predictors of a more severe level of intellectual disability. Neonatal seizures were significantly associated with neonatal hypocalcemia in the entire sample (P < 0.0001), regardless of intellectual level. There was no evidence for the association of moderate-to-severe intellectual disability with other factors such as major structural brain malformations in this sample. The results suggest that neonatal seizures may increase the risk for more severe intellectual deficits in 22q11.2 deletion syndrome, likely mediated by neonatal hypocalcemia. Neonatal hypocalcemia often remains unrecognized until the postseizure period, when damage to neurons may already have occurred. These findings support the importance of early recognition and treatment of neonatal hypocalcemia and potentially neonatal screening for 22q11.2 deletions.

Objective assessment of sleep and sleep problems in older adults with intellectual disabilities

Little is known about sleep in older adults with intellectual disability (ID). Aim of this study was to investigate sleep and its associated factors, and to estimate the prevalence of sleep problems in this population. This study was part of the healthy aging and intellectual disabilities study. Sleep was assessed using the Actiwatch, a watch-like device that measures sleep and wakefulness based on movement activity. Participants (n=551) wore the Actiwatch at least seven days and nights continuously. Variables of interest were time in bed (TIB), sleep onset latency, total sleep time, wake after sleep onset, sleep efficiency and get-up time latency. Multivariate analyses were used to investigate factors associated with these sleep parameters. Provisional definitions were drafted to estimate the prevalence of sleep problems. Mean TIB was 630 min. Longer TIB was independently associated with higher age, more severe level of ID, living at a central facility, wheelchair dependence, female gender and depressive symptoms (adjusted R(2)=.358, F-change=8.302, p<.001). The prevalence of sleep problems was 23.9% settling problem, 63.1% night waking problem, 20.9% short sleep time, 9.3% early waking problem. 72% of the participants had at least one problem, 12.3% had three or more sleep problems. Older adults with ID lie in bed very long, and the prevalence of sleep problems is high. Further research should focus on causality of the relationships found in this study, and effects of sleep problems on health and well-being in this population.

Parental social support, coping strategies, resilience factors, stress, anxiety and depression levels in parents of children with MPS III (Sanfilippo syndrome) or children with intellectual disabilities (ID)

Mucopolysaccharidosis type III (MPS III, Sanfilippo syndrome) is a lysosomal storage disorder, caused by a deficiency in one of four enzymes involved in the catabolism of the glycosaminoglycan heparan sulphate. It is a degenerative disorder, with a progressive decline in children's intellectual and physical functioning. There is currently no cure for the disorder. To date there is a paucity of research on how this disorder impacts parents psychological functioning. Specifically, research in the area has failed to employ adequate control groups to assess if the impact of this disorder on parents psychological functioning differs from parenting a child with intellectual disability (ID). The current study examined child behaviour and parental psychological functioning in 23 parents of children with MPS III and 23 parents of children with ID. Parents completed postal questionnaires about their child's behaviour and abilities and their own psychological functioning. Parents of children with MPS III reported fewer behavioural difficulties as their child aged, more severe level of intellectual disability, and similar levels of perceived social support, coping techniques, stress, anxiety and depression levels as parents of children with ID. Both groups of parents scored above the clinical cut off for anxiety and depression. Parents of children with MPS III rated themselves as significantly less future-orientated and goal directed than parents of children with ID. Services should develop support packages for parents of children with MPS III that incorporate an understanding of the unique stressors and current-difficulty approach of this population. Future research should examine gender differences between parental psychological functioning, using mixed qualitative and quantitative approaches, and utilise matched developmental level and typically developing control groups.

Assessment of objectively measured physical activity levels in individuals with intellectual disabilities with and without Down's syndrome.

ObjectiveTo investigate, using accelerometers, the levels of physical activity being undertaken by individuals with intellectual disabilities with and without Down's syndrome.MethodsOne hundred and fifty two individuals with intellectual disabilities aged 12–70 years from East and South-East England. Physical activity levels in counts per minute (counts/min), steps per day (steps/day), and minutes of sedentary, light, moderate, vigorous, and moderate to vigorous physical activity (MVPA) measured with a uni-axial accelerometer (Actigraph GT1M) for seven days.ResultsNo individuals with intellectual disabilities met current physical activity recommendations. Males were more active than females. There was a trend for physical activity to decline and sedentary behaviour to increase with age, and for those with more severe levels of intellectual disability to be more sedentary and less physically active, however any relationship was not significant when adjusted for confounding variables. Participants with Down's syndrome engaged in significantly less physical activity than those with intellectual disabilities without Down's syndrome and levels of activity declined significantly with age.ConclusionsIndividuals with intellectual disabilities, especially those with Down's syndrome may be at risk of developing diseases associated with physical inactivity. There is a need for well-designed, accessible, preventive health promotion strategies and interventions designed to raise the levels of physical activity for individuals with intellectual disabilities. We propose that there are physiological reasons why individuals with Down's syndrome have particularly low levels of physical activity that also decline markedly with age.

Participation in daytime activities among people with mild or moderate intellectual disability

Community participation has been defined as performing daytime activities by people while interacting with others. Previous studies on community participation among people with intellectual disability (ID) have mainly focused on the domestic life aspect. This study investigates the variation in community participation in the domains work, social contacts and leisure activities among people with ID in the Netherlands. A number of categories of people with ID were distinguished by: (1) gender; (2) age; (3) type of education; (4) severity of ID; and (5) accommodation type. Data were gathered on 653 people with mild or moderate ID, of whom 513 by oral interviews and 140 by structured questionnaires filled in by representatives of those who could not be interviewed. Pearson chi-square tests were used to test differences between categories of people with ID in the distributions of the participation variables. Additional logistic regression analyses were conducted to correct for differences between the categories in other variables. Most people with mild or moderate ID in the Netherlands have work or other daytime activities, have social contacts and have leisure activities. However, people aged 50 years and over and people with moderate ID participate less in these domains than those under 50 years and people with mild ID. Moreover, people with ID hardly participate in activities with people without ID. High participation among people with a mild or moderate ID within the domains of work, social contact and leisure activities does not necessarily indicate a high level of interaction with the community, because the majority hardly interact with people without ID. Furthermore, older people with ID and people with a more severe level of ID seem to be more at risk for social exclusion.

Six-Minute Walk Test in Patients With Down Syndrome: Validity and Reproducibility

Vis JC, Thoonsen H, Duffels MG, de Bruin-Bon RA, Huisman SA, van Dijk AP, Hoendermis ES, Berger RM, Bouma BJ, Mulder BJ. Six-minute walk test in patients with Down syndrome: validity and reproducibility. Objectives To examine the validity of the six-minute walk test (6MWT) as a tool to evaluate functional exercise performance in patients with Down syndrome (DS). Design Comparison of the six-minute walk distance (6MWD) in 2 distinct groups of DS patients: with and without severe cardiac disease. To test reproducibility, a group of patients with DS performed the 6MWT twice. Setting Tertiary referral centers for patients with congenital heart defects and outpatient clinics for people with intellectual disabilities. Participants Adult patients with DS with (n=29) and without (n=52) severe cardiac disease categorized by cardiac echocardiography. Interventions Not applicable. Main Outcome Measure Distance walked on the 6MWT. Results The mean 6MWD in the group with severe cardiac disease was 289±104m and in the group without severe cardiac disease 280±104m ( P=.70). Older age, female sex, and severe level of intellectual disability were all found to be independently and significantly correlated with a lower 6MWD ( r=.67, P<.001). The paired 6MWD was not significantly different (310±88m vs 317±85m; P=.40) in patients who performed the 6MWT twice. The coefficient of variation was 11%. Conclusions The 6MWD between the 2 groups was not significantly different. However, the walking distance inversely correlated with the level of intellectual disability. Therefore, the 6MWT is not a valid test to examine cardiac restriction in adult patients with DS.

Day service programmes for people with a severe intellectual disability and quality of life

This study focuses on the contributions of a community and campus based day programme to the quality of life of programme participants with a severe level of intellectual disability from the perspective of parents and staff. Qualitative interviews were carried out with parents and staff to explore the contribution that the two programmes had to participants' quality of life in the 6 months since the community based programme began. Schalock and Keith's Quality of Life Questionnaire was administered to supplement the qualitative data. Results indicated that the community based programme contributed more to participants' quality of life over the preceding 6 months than the campus based programme. This study revealed that community based participants' new-found social roles, their hobbies and their work opportunities had a positive effect on their quality of life.

Age at death of people with an intellectual disability in Ireland

This study examined the age at death of people with an intellectual disability in Ireland. Using data from the National Intellectual Disability Database, the average age at death of 1120 people who died between 1996 and 2001 was 45.68 years, with no difference in lifespan between men and women. A more severe level of intellectual disability predicted shorter lifespan. In general, those requiring lower levels of day-to-day support and supervision had a longer lifespan. Those living in the family home had the shortest lifespan while those in hospital residential environments had the longest lifespan. Those not attending any day service had a longer lifespan than those attending a range of day services. There were also differences in age at death across different health board regions. These findings are discussed in light of their implications for service planning.

Obstacles in large‐scale epidemiological assessment of sensory impairments in a Dutch population with intellectual disabilities

A population-based epidemiological study on visual and hearing impairment was planned in a random sample of 2100 clients, drawn from a base population of 9012 users of Dutch residential and day-care intellectual disability (ID) services with the whole range of IDs. Stratification was applied for age 50 years and over and Down syndrome. Visual and hearing functions were assessed according to a standardized protocol, in cooperation with regular ophthalmologists and regional audiological centres. Anticipated obstacles in sample collection, random inclusion, informed consent, expertise of investigators, time and costs were eliminated by a careful preparation. However, inclusion and participation were incomplete. In a descriptive retrospective design, we collected data from our study files on inclusion and participation as well as reasons for non-participation, to identify unanticipated obstacles for this kind of research. Consent was obtained for 1660 clients, and 1598 clients participated in the data collection (76% of intended sample of 2100). Inclusion and participation rates were especially lower in community-based care organizations, resulting in unintentional skewing of the sample towards more severe levels of ID. Complete and reliable data to diagnose visual impairment were obtained for 1358/1598 (85%) and to diagnose hearing impairment for 1237/1598 participants (77%). Unanticipated obstacles had to do with the quality of coordination within care organizations, with characteristics of screening methods, and with collaboration with the regular health care system. Assessments of visual function were more easy to organize than were those of hearing. Based on our current experience, practical recommendations are given for future multicentre research, especially in community-based settings.

Deinstitutionalisation in Australia Part II: Results from a Long-Term Study

As part of an institutional closure programme in Queensland, Australia, 104 individuals most with moderate and severe levels of intellectual disability, who had been institutionalised long-term, were relocated to community-based group homes. Each individual was assessed 6 months prior to the relocation and then again after 1, 6, 12, 18 and 24 months of community living. Assessments involved ratings of adaptive and maladaptive behaviour, choice-making and objective life quality. The group means comparing institution to community ratings showed improvements in adaptive functioning but no significant change in maladaptive behaviour. There were also improvements in objective life quality and increased opportunities for choice-making following relocation to the community. These outcomes suggest that relocation to the community was associated with a more active and normalised lifestyle.

Prevalence of intellectual disability in Western Australia.

Record linkage of multiple sources was used to ascertain children with intellectual disability born in Western Australia between 1983 and 1992. The prevalence was 14.3 per 1000, 10.6 per 1000 for children with mild or moderate and 1.4 per 1000 for those with a severe level of intellectual disability. Prevalence was greater in males (prevalence ratio 1.6) and in children of Aboriginal mothers (prevalence ratio 2.3). Although prevalence of intellectual disability has been found to vary according to study location, method of ascertainment and criteria used, these estimates are similar to estimates from several recent studies.

Sleep problems and daytime problem behaviours in children with intellectual disability.

Sleep problems are common among children with intellectual disability (ID). The present study assessed the prevalence of severe sleep problems in a sample of children (n=286) with mild to profound ID who lived at home with their parents(s) in the Netherlands. It also explored relationships between severe sleep problems, and family and child variables. Demographic information, data on children's sleep behaviours and parent variables were collected using questionnaires. Severe settling problems, night waking and early waking were present in 4.2%, 10.8% and 4.2% of cases, respectively; 16.1% of children had at least one type of sleep problem. Children with a severe sleep problem had more severe levels of ID, used medication more often, had a greater frequency of epilepsy, were younger, had a greater frequency of cerebral palsy, and showed more daytime drowsiness and daytime napping than children without a severe sleep problem. Furthermore, children with a severe sleep problem showed more severe levels of daytime problem behaviours; for example, aggression, non-compliance and hyperactivity. The results of the present study are discussed with regard to the assessment and treatment of sleep problems in children with ID.