Dementia in Older Adults With Intellectual Disabilities-Epidemiology, Presentation, and Diagnosis

Strydom, André; Shooshtari, Shahin; Lee, Lynette; Raykar, Veena; Torr, Jennifer Jayne; Tsiouris, John A.; Jokinen, Nancy; Courtenay, Ken; Bass, Nick; Sinnema, Margie; Maaskant, Marian A.

doi:10.1111/j.1741-1130.2010.00253.x

Cited by 119 publications

(121 citation statements)

References 77 publications

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“…Another feature of AD-DS is the more-frequent and earlier appearance of neurological symptoms such as gait disturbance and seizures 19 when compared with LOAD. Although heterogeneous, seizures associated with AD-DS often initially present with myoclonic jerks before progressing to tonic-clonic seizures and later to non-epileptic myoclonus with cerebellar signs; electroencephalograms show diffuse slowing and spike-and-wave patterns [48][49][50] .…”

Section: Clinical Features Of Ad-dsmentioning

confidence: 99%

“…As indicated above, AD is now common in adults with DS who are over the age of 40 years and, like other genetic forms of EOAD, develops two to three decades earlier in individuals with DS than in the general population. Data describing the prevalence of AD-DS vary between studies because of diagnostic issues, such as the presence of variable premorbid deficits, and survey methodology 19 . However, the prevalence of AD in people who have DS is <5% under the age of 40 (REF.…”

Section: Prevalence Of Ad-dsmentioning

confidence: 99%

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A genetic cause of Alzheimer disease: mechanistic insights from Down syndrome

et al. 2015

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Down syndrome, which arises in individuals carrying an extra copy of chromosome 21, is associated with a greatly increased risk of early-onset Alzheimer disease. It is thought that this risk Europe PMC Funders Author ManuscriptsEurope PMC Funders Author Manuscripts is conferred by the presence of three copies of the gene encoding amyloid precursor protein (APP) -an Alzheimer disease risk factor -although the possession of extra copies of other chromosome 21 genes may also play a part. Further study of the mechanisms underlying the development of Alzheimer disease in people with Down syndrome could provide insights into the mechanisms that cause dementia in the general population.Down syndrome (DS) is a complex, highly variable disorder that arises from trisomy of chromosome 21. It was one of the first chromosomal disorders to be identified 1 and occurs with an incidence of approximately 1 in 800 births 2 . Its prevalence within a given population is influenced by infant mortality rates, access to health care, termination rates, average maternal age 3 and life expectancy. Indeed, despite the increased availability of prenatal diagnosis and access to the option of termination, the global prevalence of DS is rising because of improvements in life expectancy: the number of adults with DS aged over 40 years has doubled in northern Europe since 1990 and, in the United Kingdom, one-third of the estimated 40,000 people with DS are thought to be over 40 years of age 4 .DS is the most common form of intellectual disability. In addition to the features that are found in everyone with the disorder, such as the characteristic facial dysmorphology, there are many DS-associated phenotypes that have variable penetrance and severity. For example, approximately 40% of individuals with DS have heart malformations (usually atrioventricular septal defects) 5 . A key feature of DS is a striking propensity to develop early-onset Alzheimer disease (EOAD). Complete trisomy of chromosome 21 universally causes the development of amyloid plaques and neurofibrillary tangles (NFTs), which are typical characteristics of AD brain pathology, by the age of 40, and approximately twothirds of individuals with DS develop dementia by the age of 60 (REFS 6,7). However, rates of dementia do not reach 100%, even in older individuals, suggesting that some individuals with DS are protected from the onset of AD (FIG. 1).All of the features of DS arise because of aberrant dosages of coding and/or non-coding sequences present on chromosome 21. Among these sequences, the gene encoding amyloid precursor protein (APP) is thought to have a key role in the pathology of AD. The additional copy of APP may drive the development of AD in individuals with DS (AD-DS) by increasing the levels of amyloid-β (Aβ), a cleavage product of APP that misfolds and accumulates in the brain in people with AD. Consistent with this hypothesis, individuals with small internal chromosome 21 duplications that result in three copies of APP -a rare familial trait known as duplic...

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Section: Clinical Features Of Ad-dsmentioning

confidence: 99%

Section: Prevalence Of Ad-dsmentioning

confidence: 99%

A genetic cause of Alzheimer disease: mechanistic insights from Down syndrome

et al. 2015

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“…Dementia in ID is frequently associated with co-morbidities including psychiatric presentations, for example depression, and behavioral problems (8). Some of these psychiatric co-morbidities including problem behavior may be noted to improve with the prescription of dementia medication listed above.…”

Section: Management Of Behavioral and Psychiatric Symptoms Associatedmentioning

confidence: 99%

“…Nevertheless, there are increasing numbers of older adults with ID who may require considerable care inputs. The average annual cost of care for older individuals with ID in the UK is approaching £50,000 ($71,952; €54,034 at 2013 rates for purchasing power parity), most of which is accounted for by personal care rather than health care costs, with mental health problems being an important predictor of costs) (8). Appropriate treatment and management of behavioral and psychiatric symptoms may therefore help to reduce the burden on caregivers as well as the cost of care.…”

Section: Introductionmentioning

confidence: 99%

Pharmacological Management of Behavioral and Psychiatric Symptoms in Older Adults with Intellectual Disability

Eady

Courtenay²,

Strydom³

2015

Drugs Aging

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Abstract:Given medical and social advances, the life expectancy of individuals with intellectual disability (ID) has increased dramatically, leading to a generation of older individuals with such disabilities. This review focuses on the pharmacological treatment of behavioral and psychiatric symptoms and disorders in older persons with ID. Older adults with ID often present with medical co-morbidities and mental health issues. Medication management of behavioral and psychiatric problems is complicated by a higher risk for adverse events, lack of decision-making capacity, and complex care networks. Some studies have shown that persons with ID and co-morbid mental disorders are undertreated in comparison to individuals with similar disorders in the general population, resulting in poorer outcomes.However, older adults with ID are also at risk of polypharmacy and older age is a risk factor for development of side-effects. A general principle is that medication treatment for psychiatric disorders in older persons with ID should be started at low dosages and be increased cautiously while monitoring response and side-effects. The use of psychotropic drugs for older persons with ID and behavioral problems remains controversial, particularly in those with dementia. Key points:• The growing population of older persons with ID has an increased risk for aging related health issues such as dementia, on a background of lifelong disabilities and physical health and psychiatric co-morbidities. Management of psychiatric symptoms in older persons with ID• Medication management of behavioral and psychiatric problems may be complicated by a higher risk for adverse events, lack of decision-making capacity, and complex care networks.• Medication management of specific conditions is however beneficial, but in general, medication should be started at low doses, with careful titration against response, and regular monitoring for side-effects.

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“…12 Wiseman et al 13 reported that approximately 5% to 15% of people with DS aged 40 to 49 years and more than 30% of those aged 50 to 59 years experience a significant decline in their cognition, indicating dementia. McCarron et al 14 reported that 68% to 80% of people with DS have developed dementia by the age of 65 years.…”

Section: Introductionmentioning

confidence: 99%

Ageing in individuals with intellectual disability: issues and concerns in Hong Kong

2018

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Dementia in Older Adults With Intellectual Disabilities-Epidemiology, Presentation, and Diagnosis

Abstract: Background: As life expectancy of people with intellectual disabilities (ID) extends into older

Cited by 119 publications

References 77 publications

A genetic cause of Alzheimer disease: mechanistic insights from Down syndrome

A genetic cause of Alzheimer disease: mechanistic insights from Down syndrome

Pharmacological Management of Behavioral and Psychiatric Symptoms in Older Adults with Intellectual Disability

Ageing in individuals with intellectual disability: issues and concerns in Hong Kong

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