Abstract
Down Syndrome (DS) is characterised by premature aging and an accelerated decline of cognitive functions in the vast majority of cases. As the life expectancy of DS persons is rapidly increasing, this decline is becoming a dramatic health problem. The aim of this study was to thoroughly evaluate a group of 67 non-demented persons with DS of different ages (11 to 66 years), from a neuropsychological, neuropsychiatric and psychomotor point of view in order to evaluate in a cross-sectional study the age-related adaptive and neuropsychological features, and to possibly identify early signs predictive of cognitive decline. The main finding of this study is that both neuropsychological functions and adaptive skills are lower in adult DS persons over 40 years old, compared to younger ones. In particular, language and short memory skills, frontal lobe functions, visuo-spatial abilities and adaptive behaviour appear to be the more affected domains. A growing deficit in verbal comprehension, along with social isolation, loss of interest and greater fatigue in daily tasks, are the main features found in older, non demented DS persons evaluated in our study. It is proposed that these signs can be alarm bells for incipient dementia, and that neuro-cognitive rehabilitation and psycho-pharmacological interventions must start as soon as the fourth decade (or even earlier) in DS persons, i.e. at an age where interventions can have the greatest efficacy.
Highlights
Down Syndrome (DS) is the most common known genetic cause of moderate to severe intellectual disability, with a prevalence of around 14 per 10,000 live births [1, 2]
Ten DS persons were excluded from further neuropsychological evaluation because of the presence of features of dementia, or other severe psychiatric features according to DSM IV criteria [16] or other adverse clinical conditions that could interfere with the evaluation
Our study clearly indicates that language skills in DS persons are affected in lexical and morpho-syntactic aspects, both in comprehension and in expression, and such characteristics are more pronounced in DS adults
Summary
Down Syndrome (DS) is the most common known genetic cause of moderate to severe intellectual disability, with a prevalence of around 14 per 10,000 live births [1, 2]. The life expectancy for DS subjects has dramatically changed in the last decades, increasing from 12 in 1949 to nearly 60 years of age today [3, 4], and it is believed that it will increase in the future [5]. This implies the occurrence of an unprecedented phenomenon of aging in the DS population, which in Italy is estimated to be about 49,000 persons [6]. Dementia is present in 55% of DS adults in their sixth decade [12], preceded by changes in language skills [13, 14] and in frontal lobe functions [15] such as executive functions [13]
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