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In developed countries the population of elderly people with Down syndrome expands resulting in an increasing incidence of age-related diseases, including dementia. The assessment of dementia in individuals with intellectual disability is often complicated due to large intra-individual variability in cognitive functioning prior to dementia and to lack of standardised measures to detect dementia. Structured observations of symptoms of dementia and assessment techniques tailored for people with intellectual disability are increasingly needed.

Dementia is seen earlier and more often in patients with Down syndrome (DS) than in the general population. Patients with DS develop neuropathological changes similar to the changes seen in dementia of Alzheimer's type (DAT). The literature concerning DAT in DS is reviewed. The clinical course and differential diagnoses are discussed. Before the diagnosis of DAT is made, treatable causes of dementia should be excluded.