Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2017 Mar;27(1):31-45.
doi: 10.1007/s11065-017-9341-9. Epub 2017 Mar 13.

The Early Presentation of Dementia in People with Down Syndrome: a Systematic Review of Longitudinal Studies

Bianca Alexandra Lautarescu  1 , Anthony John Holland  2 , Shahid H Zaman  2
Affiliations
Review

The Early Presentation of Dementia in People with Down Syndrome: a Systematic Review of Longitudinal Studies

Bianca Alexandra Lautarescu et al. Neuropsychol Rev. 2017 Mar.

Abstract

Adults with Down syndrome (DS) are at a very high risk of developing early onset Alzheimer's disease (AD) due to trisomy of chromosome 21. AD is preceded by a prolonged prodromal "pre-clinical" phase presenting with clinical features that do not fulfil the diagnostic criteria for AD. It is important to clinically characterise this prodromal stage to help early detection of the disease as neuropathology of AD is almost universal by the fifth decade in DS. There is a lack of knowledge of the trajectory of decline associated with the onset of dementia in this population and early signs may be overlooked or misdiagnosed, negatively affecting the quality of life of those affected and the use of early pharmacological or psychosocial interventions. The objective of this systematic review is to evaluate the published literature on longitudinal data in order to identify the cognitive and behavioural changes occurring during the prodromal and early stages of AD in this population. Fifteen peer-reviewed articles met the inclusion criteria, including a total number of 831 participants, with the duration between baseline and follow up varying from 1 year to 47 years. Results suggest that, compared to the general population for which short-term (episodic) memory loss is the most common indicator associated with the onset of AD, in people with DS, executive dysfunction and Behavioural and Psychological Symptoms of Dementia (BPSD) are commonly observed during pre-clinical and early stages and may precede memory loss. The review highlights the importance of using a broad spectrum of assessments in the context of heterogeneity of symptoms. Theoretical and practical implications are discussed, as well as the need for further research.

Keywords: Ageing; Alzheimer’s disease; Dementia; Down syndrome; Longitudinal; Systematic review.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Flow chart showing the process of selecting studies to be included in systematic review
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Albert, M., & Cohen, C. (1992). The Test for Severe Impairment: an instrument for the assessment of patients with severe cognitive dysfunction. Journal of the American geriatrics society, 40(5), 449–453. - PubMed
    1. Adams D, Oliver C. The relationship between acquired impairments of executive function and behaviour change in adults with down syndrome. Journal of Intellectual Disability Research. 2010;54:393–405. doi: 10.1111/j.1365-2788.2010.01271.x. - DOI - PubMed
    1. American Psychiatric Association . Diagnostic and statistical manual of mental disorders. 5. DC: Washington; 2013.
    1. Annus, T., Wilson, L. R., Hong, Y. T., Acosta–Cabronero, J., Fryer, T. D., Cardenas–Blanco, A., ... & Menon, D. K. (2015). The pattern of amyloid accumulation in the brains of adults with down syndrome. Alzheimer's & Dementia. - PMC - PubMed
    1. Ball, S. L., Holland, A. J., Huppert, F. A., Treppner, P., Watson, P., & Hon, J. (2004). The modified CAMDEX informant interview is a valid and reliable tool for use in the diagnosis of dementia in adults with Down’s syndrome. Journal of Intellectual Disability Research, 48(6), 611–620. - PubMed