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Journal ArticleDOI

Population-based study of the prevalence and presentation of dementia in adults with Down's syndrome.

TLDR
Overall, the age-related pattern of presentation and dementia diagnoses differs from that seen in the general elderly population, however, age-specific prevalence rates of Alzheimer's disease were similar but 30–40 years earlier in life.
Abstract
BACKGROUND The reported prevalence rates of dementia in people with Down's syndrome have varied considerably across studies. The aim of this study was to investigate the extent of clinical change with age using an established diagnostic instrument in an unbiased, population-based sample of older people with Down's syndrome. METHOD Changes in memory, personality, general mental functioning and daily living skills were assessed using a modified version of the informant interview of the Cambridge Examination for Mental Disorders of the Elderly (CAMDEX). RESULTS Age-specific prevalence rates of dementia varied according to the diagnostic criteria used. Using CAMDEX criteria for Alzheimer's disease, prevalence rates increased from 3.4 to 10.3 to 40% in the 30-39, 40-49 and 50-59 age group, respectively. CONCLUSIONS Overall, the age-related pattern of presentation and dementia diagnoses differs from that seen in the general elderly population. However, age-specific prevalence rates of Alzheimer's disease were similar but 30-40 years earlier in life.

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Citations
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Journal ArticleDOI

Mechanisms of tau-induced neurodegeneration

TL;DR: Disruption of microtubules by the non-fibrillized abnormally hyperphosphorylated tau as well as its aggregation as neurofibrillary tangles probably impair axoplasmic flow and lead to slow progressive retrograde degeneration and loss of connectivity of the affected neurons.
Journal ArticleDOI

Alzheimer's Disease in Down Syndrome: Neurobiology and Risk.

TL;DR: The increase in lifespan for people with DS that has occurred over the past 100 years is described, as well as advances in the understanding of the occurrence of AD in adults with DS.
Journal ArticleDOI

Down syndrome: Cognitive and behavioral functioning across the lifespan.

TL;DR: Changes in emotional/behavioral functioning in adulthood are typically associated with neurodegeneration and individuals with DS are higher risk for dementia of the Alzheimer's type.
Journal ArticleDOI

Cognitive executive function in Down's syndrome

TL;DR: It is suggested that impaired executive function in Down's syndrome is due to abnormal development of the prefrontal cortex in Down’s syndrome.
References
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Journal ArticleDOI

CAMDEX. A standardised instrument for the diagnosis of mental disorder in the elderly with special reference to the early detection of dementia.

TL;DR: A new interview schedule for the diagnosis and measurement of dementia in the elderly is described and is acceptable to patients, has a high inter-rater reliability and the cognitive section has been shown to have high and specificity.
Journal ArticleDOI

Occurrence of neuropathological changes and dementia of Alzheimer's disease in Down's syndrome.

TL;DR: All 100 brains studied showed early arrest of brain growth and brain atrophy, a condition that may have been due to prenatal arrest of neurogenesis mainly in the granular cell layers, prenatal and postnatal arrest of synaptogenesis, and early aging.
Journal ArticleDOI

A Prospective Study of Alzheimer Disease in Down Syndrome

Florence Lai, +1 more
- 01 Aug 1989 - 
TL;DR: The prevalence of dementia in the institutionalized Down syndrome population of the study was 8% between 35 and 49 years, 55% between 50 and 59 years, and 75% of those over 60 years old and of note, 41 (84%) demented individuals with Down syndrome developed seizures.
Journal ArticleDOI

The pattern of acquisition of plaques and tangles in the brains of patients under 50 years of age with Down's syndrome.

TL;DR: By the age of 50 years, most patients are well on the way towards achieving that pattern of SP and NFT morphology and distribution that is typically seen in patients over 50 years of age with DS and in other patients in the general population with Alzheimer's disease.
Journal ArticleDOI

Operational criteria for senile dementia of Lewy body type (SDLT).

TL;DR: In this paper, a retrospective analysis of case notes of 21 autopsy patients with neuropathologically proven senile dementia of Lewy body type (SDLT) and 37 cases with neuro pathologically proven Alzheimer's disease (AD) identified a characteristic clinical syndrome in SDLT, including fluctuating cognitive impairment; psychotic features including visual and auditory hallucinations, and paranoid delusions; depressive symptoms; falling and unexplained losses of consciousness were all seen significantly more often than in AD.
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