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Síndrome de Down

y demencia

According to the National Institute on Aging, frontotemporal disorders (FTD), sometimes called frontotemporal dementia, are the result of damage to neurons in the frontal and temporal lobes of the brain. Many possible symptoms can result, including unusual behaviors, emotional problems, trouble communicating, difficulty with work, or difficulty with walking. FTD is rare and tends to occur at a younger age than other forms of dementia. Roughly 60% of people with FTD are 45 to 64 years old.

FTD is progressive, meaning symptoms get worse over time. In the early stages, people may have just one symptom. As the disease progresses, other symptoms appear as more parts of the brain are affected. It is difficult to predict how long someone with FTD will live. Some people live more than 10 years after diagnosis, while others live less than two years after they are diagnosed.

There is currently no cure for FTD, and no treatments slow or stop the progression of the disease, but there are ways to help manage the symptoms.  The National Institute on Aging has a helpful page for care partners/caregivers and FTD. (Click on image to the right)

There are three types of frontotemporal disorders (FTD): behavioral variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), and movement disorders.  For more detail and symptoms, see the site at the National Institute on Aging.



To watch a brief video on frontotemporal dementia

from the Alzheimer's Society (UK) click on image ...

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Image courtesy NIA

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Frontotemporal Disorders: Information for Patients, Families, and Caregivers

The National Institute for Neurological Disorders and Stroke designed this booklet to help people with frontotemporal disorders and their families learn more about these conditions and resources for coping. The publication provides detailed information about the three major types of frontotemporal disorders: progressive behavior/ personality decline (such as Pick's disease), progressive language decline (including primary progressive aphasia), and progressive motor decline. Common symptoms, causes, and diagnosis are discussed. Information about the treatment and management of these disorders, with practical advice for both people with frontotemporal disorders and their caregivers, is provided.

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Frontotemporal Dementia and Intellectual Disability

Frontal Lobe Degeneration in Adults with Down Syndrome and Alzheimer's Disease: A Review

Fonseca L, M, Yokomizo J, E, Bottino C, M, Fuentes D., Dement Geriatr Cogn Disord 2016;41:123-136. doi: 10.1159/000442941

There is a proven link between Down syndrome and the early development of the neuropathological features of Alzheimer's disease (AD). Changes in the personality and behavior of adults with Down syndrome might indicate the early stages of dementia or of frontotemporal lobar degeneration. The objective of this study was to investigate the executive functions and changes in behavior associated with frontal lobe degeneration in individuals with Down syndrome who develop AD. We conducted a systematic review selecting studies employing cognitive assessments. We identified few studies using objective measurements to determine whether cognitive aspects associated with the frontal lobe correlate with dementia in this population. We observed a tendency toward such correlations. There is a need for further studies in which objective measures of cognitive and behavioral factors are evaluated together with data related to brain function and morphology.

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The relationship between acquired impairments of executive function and behaviour change in adults with Down syndrome

Adams, D., & Oliver, C. Journal of Intellectual Disability Research, 2010 May, 54(5), 393-405.

The latter stages of dementia in individuals with Down syndrome are well documented; however, earlier cognitive and behavioral changes have only recently been described. Holland et al. suggested such early signs of dementia in this population are behavioral and are similar to those seen in frontotemporal dementia, but there is, as yet, no evidence to determine whether such behavioral changes are associated with a declines in specific cognitive functions, including those associated with the frontal lobes. A longitudinal design of three time points across 16 months was used across 30 adults with Down syndrome aged 30 years and over. Measures of cognition (Neuropsychological Assessment of Dementia in Individuals with Intellectual Disabilities), receptive language (British Picture Vocabulary Scales), adaptive behavior (Vineland Adaptive Behavior Scales), behavioral excesses and behavioral deficits (Assessment for Adults with Developmental Disabilities) and measures of executive functioning were completed at each time point. Using a data-driven method, cognitive deterioration was determined using the Reliable Change Index on performance on the Neuropsychological Assessment of Dementia in Individuals with Intellectual Disabilities across the duration of the study. Performance on the remaining measures were then compared between those with (n=10) and those without (n=20) cognitive deterioration.  Only individuals with cognitive deterioration showed decreases on measures of executive function and significant changes in behavior across the duration of the study, which was not solely due to declines in memory. There were no changes between the groups on levels of adaptive behavior.  Even in the early stages of cognitive deterioration, specific behavioral changes can be identified that are not present in those without cognitive deterioration. The differing effects of cognitive deterioration on behavioral excesses and deficits are discussed in relation to potentially differing underlying neuropathological causes.

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