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BACKGROUND: People living with intellectual and developmental disabilities (IDD) have suffered disproportionately in health outcomes and general well-being during the COVID-19 pandemic. There is emerging evidence of increased psychological distress. Increased strain has also fallen on clinicians managing the psychological needs of people with IDD, in the context of learning new technologies, staff shortages, reduced services and paused training opportunities. AIMS: To examine clinicians' experiences of patient care, clinical management and the impact of care delivery. METHOD: A mixed fixed-response and free-text survey comprising 28 questions covering four areas (responder demographics, clinical practice, changes to local services and clinician experiences) was developed, using the STROBE guidance. It was disseminated through an exponential snowballing technique to clinicians in seven high-income countries. Quantitative data were analysed and presented with Microsoft Excel. Qualitative data were coded and thematically analysed, and presented with in-text quotations. RESULTS: There were 139 respondents, mostly senior physicians (71%). Two-thirds reported over 10 years working in the field. Quantitative findings include increased clinician stress (77%), referrals (53%), patient distress presentations (>70%), patient isolation (73%) and carer burden (89%), and reduced patient participation in daily activities (86%). A third reported increased psychotropic prescribing. Qualitative analysis outlined changes to clinical practice, particularly the emergence and impact of telehealth. CONCLUSIONS: In the countries surveyed, the pandemic has not only had a significant impact on people with IDD, but also their carers and clinicians. A proactive, holistic international response is needed in preparedness for future public health emergencies.

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BACKGROUND: Williams syndrome (WS) is a neurogenetic syndrome characterized by a variety of medical conditions and cognitive deficits along with distinct psychiatric and behavioral characteristics. To the best of our knowledge, no studies to date have comprehensively reported the prevalence of medical, cognitive deficits, and psychiatric disorders in one cohort of people with WS in one study. OBJECTIVES: To detail the prevalence of the various clinical features of WS in a large nationwide Israeli cohort. To examine potential risk factors for attention deficit hyperactivity disorder (ADHD) in WS. METHODS: We investigated the effects of cardiovascular anomalies, intellectual quotient (IQ), and phonophobia (fear of sounds) on the likelihood of ADHD. The study included 80 participants with WS (mean age 7.76 years). Relevant medical information from medical records was obtained retrospectively. In addition, IQ testing and psychiatric assessments using structured tools were conducted. The association between ADHD and cardiovascular anomalies, IQ, and phonophobia was analyzed using a logistic regression. RESULTS: Supravalvular aortic stenosis and supravalvular pulmonary stenosis are the prevalent cardiovascular anomaly in WS. Phonophobia and ADHD are the most prevalent psychiatric diagnoses in people with WS. Phonophobia was significantly associated with the risk for ADHD in WS participants. CONCLUSIONS: Our findings regarding the type and prevalence of medical, cognitive, and psychiatric characteristics in WS correspond to results in previous publications. We also showed a potential link between phonophobia and ADHD that merits further research.

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BACKGROUND: There is little evidence to guide pharmacological treatment in adults with Down syndrome and Alzheimer's disease. Aims To investigate the effect of cholinesterase inhibitors or memantine on survival and function in adults with Down syndrome and Alzheimer's disease. METHOD: This was a naturalistic longitudinal follow-up of a clinical cohort of 310 people with Down syndrome diagnosed with Alzheimer's disease collected from specialist community services in England. RESULTS: Median survival time (5.59 years, 95% CI 4.67-6.67) for those on medication (n = 145, mainly cholinesterase inhibitors) was significantly greater than for those not prescribed medication (n = 165) (3.45 years, 95% CI 2.91-4.13, log-rank test P<0.001). Sequential assessments demonstrated an early effect in maintaining cognitive function. CONCLUSIONS: Cholinesterase inhibitors appear to offer benefit for people with Down syndrome and Alzheimer's disease that is comparable with sporadic Alzheimer's disease; a trial to test the effect of earlier treatment (prodromal Alzheimer's disease) in Down syndrome may be indicated. Declaration of interest A.S. has undertaken consulting for Ono Pharmaceuticals, outside the submitted work. Z.W. has received a consultancy fee and grant from GE Healthcare, outside the submitted work.

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BACKGROUND: People with Down syndrome (DS) are an ultra-high risk population for Alzheimer's disease (AD). Understanding the factors associated with age of onset and survival in this population could highlight factors associated with modulation of the amyloid cascade. OBJECTIVE: This study aimed to establish the typical age at diagnosis and survival associated with AD in DS and the risk factors associated with these. METHODS: Data was obtained from the Aging with Down Syndrome and Intellectual Disabilities (ADSID) research database, consisting of data extracted from clinical records of patients seen by Community Intellectual Disability Services (CIDS) in England. Survival times when considering different risk factors were calculated. RESULTS: The mean age of diagnosis was 55.80 years, SD 6.29. Median survival time after diagnosis was 3.78 years, and median age at death was approximately 60 years. Survival time was associated with age of diagnosis, severity of intellectual disability, living status, anti-dementia medication status, and history of epilepsy. Age at diagnosis and treatment status remained predictive of survival time following adjustment. CONCLUSION: This study provides the best estimate of survival in dementia within the DS population to date, and is in keeping with previous estimates from smaller studies in the DS population. This study provides important estimates and insights into possible predictors of survival and age of diagnosis of AD in adults with DS, which will inform selection of participants for treatment trials in the future.

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BACKGROUND: Down syndrome is associated with specific cognitive deficits. Alongside this, older adults with Down syndrome are a high risk group for dementia. The Arizona Cognitive Test Battery (ACTB), a cognitive assessment battery specifically developed for use with individuals with Down syndrome, has been proposed for use as outcome measures for clinical trials in this population. It has not been validated in older adults with Down syndrome. This study aims to assess the use and validity of the ACTB in older adults with Down syndrome. METHODS: Participants with Down syndrome aged 45 and over were assessed using the ACTB, standard tabletop tests and informant ratings. RESULTS: Assessment outcomes of 49 participants were analysed. Of these, 19 (39%) had a diagnosis of dementia or possible dementia. Most participants were able to attempt most of the tasks, although some tasks had high floor effects (including CANTAB Intra-Extra Dimensional shift stages completed and Modified Dots Task). Of the ACTB tasks, statistically significant differences were observed between the dementia and no dementia groups on CANTAB Simple Reaction Time median latency, NEPSY Visuomotor Precision-Car and Motorbike and CANTAB Paired Associates Learning stages completed. No significant differences were observed for CANTAB Intra-Extra Dimensional Shift, Modified Dots Task, Finger Sequencing, NEPSY Visuomotor precision-Train and Car and CANTAB Paired Associates Learning first trial memory score. Several of the tasks in the ACTB can be used in older adults with Down syndrome and have mild to moderate concurrent validity when compared to tabletop tests and informant ratings, although this varies on a test by test basis. CONCLUSIONS: Overall, scores for a number of tests in the ACTB were similar when comparing dementia and no dementia groups of older adults with Down syndrome, suggesting that it would not be an appropriate outcome measure of cognitive function for clinical trials of dementia treatments without further modification and validation.

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OBJECTIVE: Dementia is a common clinical presentation among older adults with Down syndrome. The presentation of dementia in Down syndrome differs compared with typical Alzheimer's disease. The performance of manualised dementia criteria in the International Classification of Diseases (ICD)-10 and Diagnostic and Statistical Manual of Mental Disorders-IV-Text Revision (DSM-IV-TR) is uncertain in this population.We aimed to determine the concurrent validity and reliability of clinicians' diagnoses of dementia against ICD-10 and DSM-IV-TR diagnoses. Validity of clinical diagnoses were also explored by establishing the stability of diagnoses over time. METHODS: We used clinical data from memory assessments of 85 people with Down syndrome, of whom 64 (75.3%) had a diagnosis of dementia. The cases of dementia were presented to expert raters who rated the case as dementia or no dementia using ICD-10 and DSM-IV-TR criteria and their own clinical judgement. RESULTS: We found that clinician's judgement corresponded best with clinically diagnosed cases of dementia, identifying 84.4% cases of clinically diagnosed dementia at the time of diagnosis. ICD-10 criteria identified 70.3% cases, and DSM-IV-TR criteria identified 56.3% cases at the time of clinically diagnosed dementia. Over time, the proportion of cases meeting ICD-10 or DSM-IV-TR diagnoses increased, suggesting that experienced clinicians used their clinical knowledge of dementia presentation in Down syndrome to diagnose the disorder at an earlier stage than would have been possible had they relied on the classic description contained in the diagnostic systems. CONCLUSIONS: Clinical diagnosis of dementia in Down syndrome is valid and reliable and can be used as the standard against which new criteria such as the DSM-5 are measured.

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INTRODUCTION: Adults with intellectual disabilities have higher rates of mental ill-health and problem behaviors than the general population. METHOD: In this study, we present data on trends in challenging behavior in residential care centers in Israel from 1998 to 2008 and further data on trends in employment of psychiatrists from 1998 to 2009 and psychotropic medication use from 1998 to 2008. Data was collected from annual questionnaires sent out to all residential care centers in Israel, from the Office of the Medical Director, Division for Intellectual and Developmental Disabilities, Ministry of Social Affairs and Social Services. RESULTS: Rates of challenging behaviors in people with intellectual disabilities living in residential care centers in Israel continues to rise. Alongside this, trends in regular psychotropic medication use also continues to increase. CONCLUSION: Consideration of biological, psychological, social, and environmental factors in the assessment and management of people with intellectual disabilities and challenging behaviors is important. This is best conducted using a multidisciplinary approach, which may include psychiatric assessment. Non-pharmacological interventions should always be considered either alongside, or instead of medication.

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BACKGROUND: It is well established that people with intellectual disabilities are at higher risk of developing mental illnesses. This study aimed to assess the need for a specialized service for people (children and adults) with intellectual disabilities and mental health problems living in Israel. METHODS: Our research question was: is there a need for a specialist mental health service for people with intellectual disabilities living in Israel and, if so, what type of service would be most appropriate? We conducted a qualitative study using semi-structured interviews with 14 major stakeholders to identify key themes in response to our research question. The data were coded and themes were identified. RESULTS: Participants were generally not satisfied with current mental health care for people with intellectual disabilities and there was a general agreement that services are in need of improvement. We identified three major themes from the data. These were: current services, future services, and ways to facilitate change. CONCLUSION: We hope that our findings will be instrumental in shaping the ongoing debate about the best form of delivery of services to this population in Israel. Specifically, we suggest the development of a more specialized system, with the formation of multidisciplinary regional assessment and treatment units in parallel with improved relevant training for all mental health workers and the possibility of referral to specialized teams in more complex cases.

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PURPOSE OF REVIEW: Improvements in health and social care for people with intellectual disability have led to a dramatic increase in the life expectancy of this population, resulting in a large and growing number of older adults with intellectual disability. They are at risk of age-related mental disorders such as dementia and continue to present with high rates of mental illness. RECENT FINDINGS: Recent research with older adults with intellectual disability has included physical health issues that may affect mental well being, and biological and clinical features of dementia in people with Down syndrome. Two key clinical trials of memantine and antioxidants for dementia in Down syndrome showed that these treatment options were ineffective in the short term. Other research focussed on environmental and psychosocial issues (including carer issues) and end-of-life care. SUMMARY: Improved mental well being requires effective management of mental illness and co-morbid physical health problems as well as consideration of environmental and social issues. Biological research has identified potential treatments for age-related decline, which has led to the development of medication trials. Despite disappointing results, two recent medication trials showed that randomized controlled trials are feasible in older people with intellectual disabilities - a group who are often excluded from trials.

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