Skip to main content

Population-based study of the prevalence and presentation of dementia in adults with Down's syndrome

  • Anthony J. Holland (a1), Johnny Hon (a1), Felicia A. Huppert (a2), Fran Stevens (a1) and Peter Watson (a3)...

The reported prevalence rates of dementia in people with Down's syndrome have varied considerably across studies. The aim of this study was to investigate the extent of clinical change with age using an established diagnostic instrument in an unbiased, population-based sample of older people with Down's syndrome.


Changes in memory, personality, general mental functioning and daily living skills were assessed using a modified version of the informant interview of the Cambridge Examination for Mental Disorders of the Elderly (CAMDEX).


Age-specific prevalence rates of dementia varied according to the diagnostic criteria used. Using CAMDEX criteria for Alzheimer's disease, prevalence rates increased from 3.4 to 10.3 to 40% in the 30–39, 40–49 and 50–59 age group, respectively.


Overall, the age-related pattern of presentation and dementia diagnoses differs from that seen in the general elderly population. However, age-specific prevalence rates of Alzheimer's disease were similar but 30–40 years earlier in life.

Corresponding author
A.J. Holland. Section of Developmental Psychiatry, 18b Trumpington Road, Cambridge CB2 2AH
Hide All
Allsop, D. Kidd, M. Landon, M. et al (1984) Isolated senile plaque cores in Alzheimer's disease and Down's syndrome show differences in morphology. Journal of Neurology, Neurosurgery and Psychiatry, 49, 886892.
American Psychiatric Association (1994) Diagnostic and Statistical Manual of Mental Disorders (4th edn) (DSM–IV). Washington. DC: APA.
Aylward, E. H. Burt, D. B. Thorpe, L. U. et al (1997) Diagnosis of dementia in individuals with intellectual disability. Journal of Intellectual Disability Research, 41, 152164.
Crome, L. & Stern, J. (1972) Pathology of Mental Retardation. Baltimore, MD: Williams and Wilkins.
Gregory, C. A. & Hodges, J. R. (1996) Clinical features of frontal lobe dementia in comparison to Alzheimer's disease. Journal of Neural Transmitters, 47 (suppl). 103123.
Haveman, M. J. Maaskant, M. A. & Sturmans, F. (1989) Older Dutch residents of institutions, with and without Down syndrome: comparison of mortality and morbidity trends and motor/social functioning. Australia and New Zealand Journal of Developmental Disabilities, 15, 241255.
Haxby, J. V. (1989) Neuropsychological evaluation of adults with Down's syndrome: patterns of selective impairment in non-demented old adults. Journal of Mental Deficiency Research, 33, 193210.
Holland, A. J. & Oliver, C. (1995) Down's syndrome and the links with Alzheimer's disease. Journal of Neurology, Neurosurgery and Psychiatry, 59, 111114.
Kemper, T. L. (1988) Neuropathology of Down's syndrome. In The Psychobiology of Down Syndrome (ed. L. Nadel). Cambridge, MA: MIT Press.
Lai, F. & Williams, R. (1989) A prospective study of Alzheimer's disease in Down's syndrome. Archives of Neurology, 46, 849853.
Mann, D. M. A. Yates, P. O. Marcyniuk, B. et al (1986) The topography of plaques and tangles in Down's syndrome patients of different ages. Neuropathology and Applied Neurobiobgy, 12, 447457.
Mann, D. M. A. & Esiri, M. M. (1989) The pattern of acquisition of plaques and tangles in Down's syndrome patients under 50 years of age. Journal of Neurological Science, 89, 169179.
McKeith, I. G. Perry, R. H. Jabeen, S. et al (1992) Operational criteria for senile dementia of Lewy body type (SDLT). Psychological Medicine, 2, 911922.
McNemar, Q. (1955) Psychological Statistics. New York: Wiley.
Roth, M. Tym, E. Mountjoy, C. Q. et al (1986) CAMDEX – A standardised instrument for the diagnosis of mental disorder in the elderly with special reference to the early detection of dementia. British Journal of Psychiatry, 149, 698709.
Sparrow, S. S. Balla, D. A. & Cicchetti, D. V. (1984) Vineland Adaptive Behavior Scale (Interview Edition-Survey Version). Circles Pines, MN: American Guidance Association.
Thase, M. E. Tigner, R. Smeitzer, D. et al (1984) Age-related neuropsychological deficits in Down's syndrome. Biological Psychiatry, 19, 571585.
Winiewski, K. E. Winiewski, H. M. & Wen, G. Y. (1985) Occurrence of neuropathological changes and dementia of Alzheimer's disease in Down's syndrome. Annals of Neurology, 17, 278282.
World Health Organization (1992) The Tenth Revision of the International Classification of Diseases and Related Health Problems (ICD–10). Geneva: WHO.
Recommend this journal

Email your librarian or administrator to recommend adding this journal to your organisation's collection.

The British Journal of Psychiatry
  • ISSN: 0007-1250
  • EISSN: 1472-1465
  • URL: /core/journals/the-british-journal-of-psychiatry
Please enter your name
Please enter a valid email address
Who would you like to send this to? *


Altmetric attention score

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed

Population-based study of the prevalence and presentation of dementia in adults with Down's syndrome

  • Anthony J. Holland (a1), Johnny Hon (a1), Felicia A. Huppert (a2), Fran Stevens (a1) and Peter Watson (a3)...
Submit a response


No eLetters have been published for this article.


Reply to: Submit a response

Your details

Conflicting interests

Do you have any conflicting interests? *