Objectives: Characterise the circumstances associated with death during admission of adults with Down syndrome (DS) and to identify predictors of mortality. Patients and Methods: Observational study based on data on all emergent admissions of adults with DS to hospitals of the Spanish National Health System between 1997 and 2014. We analysed…

Background: The accuracy of the National Task Group-Early Detection Screen for Dementia (NTG-EDSD) was evaluated in a sample of 185 adults with Down syndrome (DS), emphasizing 'mild cognitive impairment (MCI-DS)'. Method: Knowledgeable informants were interviewed with the NTG-EDSD, and findings were compared to an independent dementia status…

This review aimed to investigate the use of the Cambridge Neuropsychological Automated Testing Battery (CANTAB) for people at risk of cognitive impairment, especially those born with Down syndrome and those born preterm. Six databases were searched according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standards,…

Background: The CAMDEX-DS is an instrument to diagnose Alzheimer's disease (AD) in Down syndrome consisting of an informant interview and a cognitive test battery (CAMCOG-DS). Measurement properties of the German CAMDEX-DS were investigated. Method: Fifty-five adults with Down syndrome (19-58 years) participated in this observational study.…

Adults with Down syndrome are at high risk for Alzheimer's disease (AD), with most individuals developing clinical dementia by their late 60s. This increased risk for AD has been attributed, at least in part, to triplication and overexpression of the gene for amyloid precursor protein (APP) on chromosome 21, leading to elevated levels of amyloid…

Executive function (EF) decline is a consistent early sign of Alzheimer's disease (AD) among adults with Down syndrome (DS), which means that baseline measures of EF for individuals with DS are vital to allow detection of meaningful decline. We developed a framework to extract measures of three core components of EF (memory updating, inhibitory,…

Objectives: Certain heart conditions and diseases are common in Down syndrome (DS; trisomy 21), but their role in early onset dementia that is prevalent in older adults with DS has not been evaluated. To address this knowledge gap, we conducted a study of risk factors for low neurocognitive/behavioral scores obtained with a published dementia test…

Introduction: Having a diagnosis of Down syndrome (DS) is associated with intellectual disability (ID), pervasive developmental disorders and Alzheimer's dementia (AD). The association between these conditions has not been well evaluated. This paper looks to examine the current evidence pertaining to the relationship between dementia in people…

Introduction: Diagnosing dementia in people with severe/profound intellectual (and multiple) disabilities (SPI(M)D) is complex. Whereas existing dementia screening instruments as a whole are unsuitable for this population, a number of individual items may apply. Therefore, this study aimed to identify applicable items in existing dementia…

Introduction: Observable dementia symptoms are hardly studied in people with severe/profound intellectual (and multiple) disabilities (SPI(M)D). Insight in symptomatology is needed for timely signaling/diagnosis. This study aimed to identify practice-based observations of dementia symptoms in this population. Methods: Care professionals and family…

We aim to provide insight into the cause-specific mortality of older adults with intellectual disability (ID), with and without Down syndrome (DS), and compare this to the general population. Immediate and primary cause of death were collected through medical files of 1,050 older adults with ID, 5 years after the start of the Healthy Ageing and…

Background: Neuropsychological tests of memory are believed to offer the greatest sensitivity at identifying people at the risk of developing dementia. There is a paucity of standardized and appropriate neuropsychological assessments of memory for adults with an intellectual disability. This study examines how adults with an intellectual…

The following article details a piece of service development work undertaken as part of the Plymouth Down Syndrome Screening Programme. The work aimed to review the use of three measures assessing executive functioning skills used within the Programme as well as with people without Down syndrome. Three tasks assessing executive functioning (the…

Because of difficulties with neuropsychological assessments for dementia in people with learning disabilities, professionals in clinical practice have relied heavily on carer interviews, one of the most widely used being the "Dementia Questionnaire for People with Learning Disabilities" (DLD-Evenhuis et al. 2006 "Dementia…

The International Summit on Intellectual Disability and Dementia covered a range of issues related to dementia and intellectual disability, including the dearth of personal reflections of persons with intellectual disability affected by dementia. This article reflects on this deficiency and explores some of the personal perspectives gleaned from…