There is evidence that frontal-subcortical circuits play an important role in the initial presentation of dementia in Down syndrome (DS), including changes in behaviour, a decline in working memory and executive dysfunction. We evaluated 92 individuals with DS (≥ 30 years of age), divided into three groups by diagnosis-stable cognition, prodromal dementia and Alzheimer's disease (AD). Each individual was evaluated with an executive protocol developed for people with intellectual disabilities and was rated for behaviours related to frontal lobe dysfunction (disinhibition, executive dysfunction and apathy) by an informant using the Frontal Systems Behavior Scale (FrSBe). Informant-reported behaviours 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 AD increased in parallel with increases in the domain and total FrSBe 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 behaviour.

Background The longevity of people with DS has increased substantially in recent decades. This population shows signs of premature aging in many organs and systems. The neuropathology of Alzheimer's disease (AD) is overexpressed in DS. Cognitive and functional losses, such as decline in executive function and apraxia, interfere with the sequencing of movements and the integrity of gait. There is evidence that a decrease in gait may precede other clinical symptoms of dementia in AD in the general population, but we found no studies investigating gait performance and decline in DS. The aim of this study is to characterize gait and speed performance in a sample of adults with DS divided into three groups by diagnosis: stable cognition, prodromal dementia and AD. Methods We used the Performance Oriented Mobility Assessment (POMA) to address balance, gait and the risk of falls and the Timed “Up and Go” test (TUG) to assess gait speed in a sample of 35 individuals with DS over 35 years of age. Dementia diagnosis was based on the Cambridge Examination for Mental Disorders of Older People with Down’s Syndrome and Others with Intellectual Disabilities (CAMDEX‐DS). The 1‐factor, 3‐level ANOVA model was used in the 3 groups. Results 9 participants (25.7%) had AD (mean POMA 12.0 ± 7.1; mean TUG 18.0 ±5.8), 8 (22.9%) were classified as prodromal dementia (mean POMA 19.0 ± 5.6; mean TUG 21.0± 6.1) and 18 (51.4%) were in the stable cognition group (mean POMA score 20 ± 4.5; mean TUG 17.0 ±5.5). Mean POMA scores were significant different among the groups (p<0.05) with higher impairment in the group with AD relative to those with prodromal dementia and stable cognition. We did not find difference for the TUG scores among the groups. Conclusions POMA results indicates that those with AD have worse balance, gait performance and higher risk of falls relative to those with prodromal dementia and stable cognition, suggesting that gait performance may be more affected in later stages of neurodegeneration. Gait speed does not seem to be useful differentiating the groups. Further longitudinal studies with bigger samples are needed to confirm our results.

The increasing life expectancy for people with intellectual disability is a intriguing theme that deserves attention of professionals related to this area. Although it is well known that the benefits brought by modern interventions and advances in biomedicine have increased life's longevity, it also means that during the later years of life these people often have comorbidities and a pathological aging processes. Enhancing the quality of life for this population is a unique challenge in the field of Gerontology and Intellectual Disabilities. The present work is the result of a pilot study carried out by a Service for intellectual disability and aging. The intervention focused on the participants who undergo some form of senile process through a proposal of management and temporary service adaptation which was developed based on patients´ demands. The need for social and service's adjustments for the new demands of this population is evident, and it is of special significance professional qualification and expertise in this area, as well as, the rise of information for the families and the community.