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Behavioral and psychological symptoms of dementia (BPSD) have not been comprehensively studied in people with Down syndrome, despite their high risk on dementia. A novel evaluation scale was developed to identify the nature, frequency and severity of behavioral changes (83 behavioral items in 12 clinically defined sections). Central aim was to identify items that change in relation to the dementia status. Structured interviews were conducted with informants of people with Down syndrome without dementia (DS, N = 149), with questionable dementia (DS + TD, N = 65) and with diagnosed dementia (DS + AD, N = 67). Group comparisons showed a pronounced increase in frequency and severity of items about anxiety, sleep disturbances, agitation & stereotypical behavior, aggression, apathy, depressive symptoms, and, eating/drinking behavior. The proportion of individuals presenting an increase was highest in the DS + AD group and lowest in the DS group. Interestingly, among DS + TD individuals, a substantial proportion already presented increased anxiety, sleep disturbances, apathy and depressive symptoms, suggesting that these changes may be early alarm signals of dementia. The scale may contribute to a better understanding of the changes, adapting daily care/support, and providing suitable therapies to people with Down syndrome. The scale needs to be optimized based on the results and experiences. The applicability, reliability and validity require further study.

People with intellectual disabilities (IDs) are often unable to decide for themselves regarding complex medical decisions, and need assistance from representatives, often next-of-kin. People with IDs are often frail. This frailty is characterized on the one hand by health problems, on the other hand by significant limitations in both intellectual functioning and in adaptive behaviour. In this group of people, when compared to the general population, the principles of palliative care are applicable more often and over a longer period. Advance care planning should be considered not only at the end of life, but at each stage where decisions have to be made that may affect quality of life. In this article, we present three patient cases to illustrate the complexity of the process and the commitment of representatives and other key stakeholders.

Objective: To determine the level of independence and social functioning in young people with Down syndrome., Design: Cross-sectional study., Method: Via the Dutch Down Syndrome Foundation (Stichting Downsyndroom), we asked parents of children with Down syndrome born in 1992, 1993 or 1994 to complete a written questionnaire about their child. This questionnaire contained the following standardised lists: the 'Dutch social competence rating scale for people with a learning disability', the 'Child behaviour checklist' and the 'Children's social behaviour questionnaire', and additional questions on background characteristics. The results of this Dutch cohort were compared with available data on peers without Down syndrome., Results: Data from 322 young people with Down syndrome, mean age 18.4 years (range 16.8-19.9 years), were collected (response 63%). Almost 60% of participants mastered basic skills of independent functioning, such as maintaining adequate standards of personal hygiene, preparing breakfast and being able to spend at least 30 minutes at home alone. About 10% of the participants had basic skills such as cooking and paying in a shop. Nine out of ten participants had more problems with social functioning than peers without Down syndrome, mainly with social interaction, processing information and regulating their emotions. Half of the participants had clinically relevant behavioural problems., Conclusion: The results of this study show that young people with Down syndrome have limited practical and social skills, and more behavioural problems than their peers without Down syndrome. In daily life they are to a greater or lesser extent dependent on others, and need lifelong support.

As a result of increased life expectancy of persons with intellectual disabilities, the number of ageing persons with intellectual disability has increased rapidly. As a result of changed attitudes in health care, the majority of people aged 50 years or more now live in the community and make use of the standard general and specialist healthcare services. Except in people with Down syndrome, primary ageing occurs at a normal age in this population. Nevertheless, an increased age-related vulnerability can be observed, occurring earlier than in the general population. Although scientific research is still scarce, the following factors threatening health can be distinguished: functional impairments, multimorbidity, unhealthy lifestyle, incomplete detection of risk factors for cardiovascular disease, insufficient participation in population screening, missed diagnoses as a result of lack of subjective symptoms. Reliable information on health status of ageing people with an intellectual disability will become available in the next few years based on current Dutch research. Following several changes in the rules of the Dutch Healthcare Authority (NZa) it is possible for general practitioners to refer those patients with an intellectual disability living in the community to a doctor specialised in intellectual disability medicine.

Children with Down's syndrome have an increased risk of congenital anomalies and additional medical problems. These can be treated but are often seen as part of the syndrome and not as the cause of developmental retardation. Based on the current level of knowledge, specific medical tests and evaluations are recommended. The reasons for these guidelines, composed by the Down's syndrome Work Group of the Paediatric Association of the Netherlands, are illustrated by the case reports of five patients: in a 4-year-old boy, retardation improved after celiac disease had been diagnosed. In a 5-year-old boy, slow speech development was reversed after hearing loss was diagnosed. In a 2-year-old girl, development improved after a mitral valve stenosis had been corrected. In a 6-month-old boy, drinking problems were reversed when torticollis was diagnosed and a self-deflating drinking bottle was introduced. An adult man suffering from epilepsy and from self-mutilative rubbing and hitting of his eyes, regained his sight after cataract, myopia and atrophy bulbi were diagnosed. A well-balanced disclosure of the diagnosis, repeated medical screening, early motor training and social interventions combined with active parental participation, warrant optimal development. In cooperation with the Down's syndrome Parent Association, multidisciplinary medical teams have been established to offer and promote this care.