Down Syndrome - Onset Age of Dementia

Insights of Dementia in Persons With Intellectual Disability

“…When persons with ID of any etiology were screened, the dementia signs occurred as frequently in all age groups of adults [10]. However, when the dementia signs are evaluated separately in specific intellectual disability syndromes, the differences are evident as shown by our long-term follow-up studies [8,9,14,15]. Down persons start suffering from signs indicating dementia already soon after the age of 35 [8] and the risk of dementia in Williams's syndrome is similar as in general population [9,14] whereas men with Fragile-X syndrome do not appear to develop dementia at all [6].…”

“…However, when the dementia signs are evaluated separately in specific intellectual disability syndromes, the differences are evident as shown by our long-term follow-up studies [8,9,14,15]. Down persons start suffering from signs indicating dementia already soon after the age of 35 [8] and the risk of dementia in Williams's syndrome is similar as in general population [9,14] whereas men with Fragile-X syndrome do not appear to develop dementia at all [6]. Interestingly, however, cognitive aging in men with Fragile-X syndrome was found to start earlier (already before middle age) than in men in general population [15] and the course of cognition was found to be uneven in William's syndrome [14].…”

“…In Finland, the assessment of intellectual disability severity done in adolescence determines the individual's capacity for further education, and that is also when an individual's cognitive development is considered to have reached a plateau (or at least slowed down). Consequently, the test result at adolescence has been used in our long-term follow-up studies as a baseline for later testing [8][9][10].…”

“…This scale (incl. 27 items) has been used also in our studies [2,[8][9][10]. In the study group consisting of 230 persons with intellectual disability of any cause, the most common dementia symptoms were reduced selfcare skills, loss of energy, diurnal mood variation, forgetfulness and forgetting people's names [10].…”

“…For instance, stress, diet and environmental factors known to regulate the anti-aging gene Sirtuin 1 may have a role not only in the aging process but also in the induction of intellectual disability and dementia [4][5][6]. We have approached this puzzle by studying the signs indicating dementia in people with intellectual disability of varying causes [2,[7][8][9][10]. Such approach requires multidisciplinary teamwork of special units for the management of neuropsychiatric disorders of this patient group [11].…”