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Neurobiol Aging. 2019 Jun;78:186-194. doi: 10.1016/j.neurobiolaging.2019.02.028. Epub 2019 Mar 11.

Frontal-subcortical behaviors during Alzheimer's disease in individuals with Down syndrome.

Author information

1
Old Age Research Group, PROTER, Department and Institute of Psychiatry, University of São Paulo School of Medicine, FMUSP, São Paulo, Brazil; Cambridge Intellectual and Developmental Disabilities Research Group, Department of Psychiatry, University of Cambridge, Cambridge, UK. Electronic address: lmfonseca@usp.br.
2
Old Age Research Group, PROTER, Department and Institute of Psychiatry, University of São Paulo School of Medicine, FMUSP, São Paulo, Brazil.
3
Laboratory of Neuroscience, LIM27, Department and Institute of Psychiatry, University of São Paulo School of Medicine, FMUSP, São Paulo, Brazil.
4
Department of Psychiatry, Federal University of São Paulo, São Paulo, Brazil.
5
Association of Parents and Friends of Individuals with Intellectual Disability of São Paulo, São Paulo, Brazil; Department of Neurology and Neurosurgery, Federal University of Sao Paulo, São Paulo, Brazil.
6
Cambridge Intellectual and Developmental Disabilities Research Group, Department of Psychiatry, University of Cambridge, Cambridge, UK.
7
Obsessive-Compulsive Spectrum Disorders Program, PROTOC, Department and Institute of Psychiatry, University of São Paulo School of Medicine, FMUSP, São Paulo, Brazil.

Abstract

There is evidence that frontal-subcortical circuits play an important role in the initial presentation of dementia in Down syndrome (DS), including changes in behavior, a decline in working memory and executive dysfunction. We evaluated 92 individuals with DS (≥30 years of age), divided into 3 groups by diagnosis-stable cognition, prodromal dementia, and Alzheimer's disease. Each individual was evaluated with an executive protocol developed for people with intellectual disabilities and was rated for behaviors related to frontal lobe dysfunction (disinhibition, executive dysfunction, and apathy) by an informant using the Frontal Systems Behavior Scale. Informant-reported behaviors related to frontal lobe dysfunction were found to correlate negatively with executive function performance. Disinhibition and executive dysfunction were associated with the clinical stage of dementia. The odds of having Alzheimer's disease increased in parallel with increases in the domain and total Frontal Systems Behavior Scale scores (p ≤ 0.5). Disinhibition, executive dysfunction and apathy should be taken into consideration during the clinical evaluation of adults with DS, and future studies should consider the intersection of neuropathology, brain connectivity, and behavior.

KEYWORDS:

Alzheimer's disease; Apathy; Dementia; Disinhibition; Down syndrome; Executive dysfunction

[Indexed for MEDLINE]

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