A 3-year follow-up study on cardiovascular disease and mortality in older people with intellectual disabilities.

BACKGROUND: With increasing longevity and a similar or increased prevalence of cardiovascular disease risk factors (as compared to the general population), people with intellectual disabilities (IDs) are at risk of developing cardiovascular disease. However, prospective studies on incidence and influencing factors of cardiovascular disease and mortality are lacking. METHODS: A three year follow-up study was undertaken to study the incidence and symptoms at presentation of myocardial accident, stroke and heart failure in older people with ID. Furthermore, the predictive value of cardiovascular disease risk factors on myocardial accident, stroke and heart failure and on all-cause mortality were studied. The baseline group consisted of the 1050 participants, aged 50 years and over, in the Dutch Healthy Ageing and Intellectual Disability (HA-ID) study. Baseline measurements were conducted between November 2008 and July 2010. Three years after baseline, medical files of 790 participants were studied. RESULTS: Cardiovascular disease (myocardial infarction, stroke and heart failure) occurred in 5.9% of the population during 3 year follow-up, and 32% of them died due to the condition. Incidence of myocardial infarction is 2.8 per 1000 personyears, for stroke 3.2 per 1000 personyears and for heart failure 12.5 per 1000 personyears. Incidence of these conditions is probably underestimated, due to atypical symptom presentation. The use of atypical antipsychotics and a history of heart failure were predictive for myocardial infarction. Heart failure was predicted by abdominal obesity, chronic kidney disease and a history of heart failure. A total of cardiovascular disease (myocardial infarction, stroke or heart failure) was predicted by abdominal obesity, a history of stroke and a history of heart failure. A low body-mass index, peripheral arterial disease, chronic kidney disease and inflammation were predictive for 3-year all-cause mortality. CONCLUSION: Incidence of cardiovascular disease in older people with ID is similar to that in the general population. A pro-active assessment and treatment of the presented cardiovascular disease risk factors may reduce cardiovascular disease and mortality in older people with ID.

Associations of symptoms of anxiety and depression with diabetes and cardiovascular risk factors in older people with intellectual disability.

BACKGROUND: Depression, anxiety, diabetes and cardiovascular risk factors are frequent health problems among older people with intellectual disability (ID). These conditions may be bidirectionally related. Depression and anxiety may have biological effects causing glucose intolerance, fat accumulation and also lifestyle changes causing metabolic syndrome. But also the effects of diabetes, metabolic syndrome and subsequent cardiovascular disease may affect mood and anxiety. This study investigated the association between depression, anxiety and diabetes and cardiovascular risk factors in older people with ID. METHODS: The healthy ageing in intellectual disability-study (HA-ID study) is a cross-sectional study among people aged 50 years and over with ID, receiving formal ID care. Screening instruments for symptoms of anxiety and depression were completed and physical examination and vena-puncture were performed to establish components of the metabolic syndrome, peripheral arterial disease and c-reactive protein. RESULTS: Of the 990 people who participated, 17% had symptoms of depression and 16% had symptoms of anxiety. Type I diabetes was present in 1%, type II diabetes in 13% of the study population. Metabolic syndrome, central obesity, hypercholesterolemia and hypertension were present in 45%, 48%, 23% and 53% respectively. In a multivariate logistic regression analysis a significant association was found between increased anxiety symptoms and diabetes only (OR 2.4, 95%CI 1.2-4.9). CONCLUSIONS: Increased anxiety symptoms and diabetes are related in older people with ID. This association may be bidirectional. No other associations of depression and anxiety symptoms with cardiovascular risk factors could be proven to be significant. Therefore, more research is needed to unravel the mechanisms of stress, mood disorders and cardiovascular disease in older people with ID. To provide comprehensive care for older people with ID, screening for diabetes and components of the metabolic syndrome in people with anxiety or mood disorders, and screening for symptoms of anxiety or depression in people with diabetes is warranted.

Chronic kidney disease in older people with intellectual disability: results of the HA-ID study.

With increasing longevity and cardiovascular events, chronic kidney disease may also become a significant problem in older people with intellectual disability (ID). We studied prevalence and associations of chronic kidney disease as part of the Healthy Ageing and Intellectual Disability (HA-ID) study, a large Dutch cross-sectional study among people with ID aged 50 years and over, using creatinine and cystatin-C measurement in plasma. Glomerular filtration rate (GFR) was calculated using the Modification of Diet in Renal Disease (MDRD) and Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equations. Equations based on creatinine (as the MDRD equation) may underestimate kidney dysfunction in people with sarcopenia, because low muscle mass leads to a low creatinine production. Therefore, also prevalence of chronic kidney disease was studied in the sarcopenic group, using different GFR equations. Prevalence of chronic kidney disease, among 635 participants, was 15.3%, which equals prevalence in the general Dutch population. In the group of participants with sarcopenia (n=82), the CKD-EPI equation based on creatinine and cystatin-C gave a higher prevalence of chronic kidney disease than did the MDRD equation, but confidence intervals were very wide. Chronic kidney disease was associated with higher age, Down syndrome, obesity, hypercholesterolemia and hypothyroid disease. GFR should be measured in all older people with ID and polypharmacy, and in older people with ID and Down syndrome as part of the regular health checks. Moreover, if sarcopenia is present and information on GFR is required, this should not be measured based on creatinine only, but additional measures, such as cystatin-C, should be taken into account.

Peripheral arterial disease in older people with intellectual disability in The Netherlands using the ankle-brachial index: results of the HA-ID study.

Older people with an intellectual disability (ID) have been shown to have similar to increased cardiovascular risks as compared to the general population. Peripheral arterial disease (PAD), atherosclerosis distal from the aortic bifurcation, is associated with increased cardiovascular morbidity and mortality. The prevalence of PAD has not been investigated in this population. Therefore, the aim of the present study was to determine the prevalence of PAD in older people with ID in The Netherlands, the rate of prior diagnoses, and correlations with participant characteristics, and to compare the prevalence with PAD in the general Dutch population. 771 people aged 50 years and over participated in ankle-brachial index (ABI) measurement as part of a multi-centre cross-sectional study (HA-ID study). PAD was defined as an ABI<0.9. After excluding those, who met the exclusion criteria, 629 participants remained. PAD was present in 20.7% of the participants and 97% had not been diagnosed before. People with higher age, smokers and people who lived in central settings, walked with support and were more dependent in activities of daily living were more at risk of PAD. Prevalence of PAD is higher than in the general population (17.4% of 562 eligible participants with ID, as compared to 8.1% of 917 Dutch participants of the PANDORA study, a pan-European study into the prevalence of PAD) through all age groups. Because the high prevalence of PAD implies a serious health risk for older people with ID, we recommend that ankle-brachial index measurement is to be routinely performed as part of the cardiovascular risk management in this group.

Cardiovascular risk factors (diabetes, hypertension, hypercholesterolemia and metabolic syndrome) in older people with intellectual disability: results of the HA-ID study.

Hypertension, diabetes, hypercholesterolemia and the metabolic syndrome are important risk factors for cardiovascular disease (CVD). In older people with intellectual disability (ID), CVD is a substantial morbidity risk. The aims of the present study, which was part of the Healthy Ageing in Intellectual Disability (HA-ID) study, were (1) to determine the prevalence of CVD risk factors in older people with ID and to compare this with the prevalence in the same-aged general population, (2) to determine how many risk factors had not been previously diagnosed, and (3) to identify correlates of CVD risk factors (gender, age, level of ID, Down syndrome, independent living, activities of daily living, mobility, instrumental activities of daily living, physical activity, use of atypical antipsychotics, central obesity), using logistic regression analyses. In this cross-sectional study, 980 people with borderline to profound ID participated. Hypertension (53%), diabetes (14%) and metabolic syndrome (45%) were present similarly as in the general Dutch population. Hypercholesterolemia was present less often (23%). Fifty percent of the people with hypertension had not been previously diagnosed with this condition. Percentages for diabetes, hypercholesterolemia, and the metabolic syndrome were 45, 46 and 94 respectively. People who were more at risk for CVD risk factors were women, older people, people with obesity, people who lived more independently and people who were able to do groceries or prepare a meal independently. Policy on prevention, detection and treatment of CVD risk factors is urgently needed.

Overweight and obesity in older people with intellectual disability.

Overweight and obesity are major health problems associated with increased cardiovascular disease risk, which is not sufficiently studied in people with intellectual disability yet. The present study was part of the Healthy Ageing in Intellectual Disability (HA-ID) study. The aim of this study was to establish (1) the prevalence of overweight, obesity and body fat percentage in older people with intellectual disability (ID) through measurement of Body Mass Index (BMI), waist circumference, waist-to-hip ratio (WHR) and skin fold thickness, and compare this with prevalence of overweight and obesity in the general population, and (2) the association of overweight and obesity with participant and treatment characteristics (gender, age, level of ID, Down syndrome, autism, independent living, smoking, (instrumental) activities of daily living ((I)ADL), physical activity and use of atypical antipsychotic medication) using regression analyses. In this cross-sectional study 945 persons, aged 50 and over with borderline to profound ID, living in central settings, in community settings and independently were included. Overweight and obesity were highly prevalent, with more obesity (26%) than in the general Dutch older population (10%) as measured by BMI, and 46-48% obesity as measured by waist circumference and WHR respectively. Women, people with Down syndrome, higher age, less severe ID, autism, people who are able to eat independently, preparing meals and doing groceries independently, people with physical inactivity and use of atypical antipsychotics were significantly more at risk of being overweight or obese. This merits specific actions by policy makers and clinical practice to improve health outcomes.

Physical conditions and challenging behaviour in people with intellectual disability: a systematic review.

BACKGROUND: Challenging behaviour is a major problem among people with intellectual disabilities. Physical factors may be an important cause. The aim of the present systematic review was to determine the physical conditions associated with challenging behaviour. METHODS: A literature search was conducted in PubMed and the Cochrane systematic review database for empirical studies published between 1990 and 2008. The quality of all the studies that met the inclusion criteria was assessed using the SIGN-50 methodology checklists. RESULTS: The search identified 45 studies, which looked at general medical conditions, motor impairment, epilepsy, sensory impairment, gastrointestinal disease, sleep disorders, dementia and others. There were four high-quality observational studies, seven well-conducted observational studies, 21 observational studies of low methodological quality and 13 non-analytical studies. There were significant and independent associations between challenging behaviours and urinary incontinence, pain related to cerebral palsy and chronic sleep problems, and between self-injurious behaviour and visual impairment. No association was found with hearing impairment, bowel incontinence, mobility impairment or epilepsy. Many other physical conditions were not addressed at all. CONCLUSION: Medical conditions can play a role in challenging behaviour, and this should be evaluated in the clinical setting. So far, the level of evidence is generally low, and longitudinal studies are completely lacking. We recommend a systematic approach to research examining the role of physical conditions in challenging behaviour, the ultimate aim being to establish a basis for the development of clinical guidelines.

Behavioural phenotype in Börjeson-Forssman-Lehmann syndrome.

BACKGROUND: Börjeson-Forssman-Lehmann syndrome (BFLs) is an X-linked inherited disorder characterised by unusual facial features, abnormal fat distribution and intellectual disability. As many genetically determined disorders are characterised not only by physical features but also by specific behaviour, we studied whether a specific behavioural phenotype exists in BFLs. METHODS: We studied in detail the behaviour of four molecularly proven BFLs patients, and reviewed available literature on BFLs specifically for behavioural characteristics. RESULTS: Behaviour in persons with BFLs is in general friendly, but can be challenging with externalising and thrill-seeking features. Social skills are good. However, variation among patients is wide. Three patients from a single family showed expressed hypersexual behaviour. This was not present in other patients. CONCLUSION: In BFLs a specific behavioural phenotype exists and in behaviour general is challenging besides a friendly habit. Within single families more problematic behaviour may occur. Further behavioural and molecular analysis of a larger group of patients is warranted to determine whether a genotype-behavioural phenotype correlation exists.

[Autism and catatonia: successful treatment using lorazepam. A case study]. / Autisme en katatonie. Een succesvolle behandeling met lorazepam.

A 24-year-old man who was mentally retarded and had an autistic disorder, developed mutism and motor symptoms. He was diagnosed with catatonia and was treated successfully with lorazepam. Additionally, we review the literature about the diagnosis and treatment of catatonia in patients with autism; in such cases accurate diagnosis is vital but is complicated by overlapping symptoms.