Skip to main content
×
×
Home

Down's syndrome and dementia

  • Lisa R. Stanton and Rikus H. Coetzee
Abstract

Down's syndrome is the most common genetic disorder seen in clinical practice: about 94% occurs because of non-disjunction of chromosome 21 and 3–5% because of translocation. Individuals increasingly survive to middle and old age, probably because of advances in medical treatment and improved living conditions. People with Down's syndrome have an increased risk of developing Alzheimer's disease in middle age. Within ICD–10 and DSM–IV classifications there is no consensus on the diagnosis of dementia in people with learning disability. New treatments have been licensed for use in mild to moderate Alzheimer's disease (e.g. acetylcholinesterase inhibitors and memantine). The comorbid picture of Down's syndrome and Alzheimer's disease presents a unique challenge to the clinician in both diagnosis and management.

    • Send article to Kindle

      To send this article to your Kindle, first ensure no-reply@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about sending to your Kindle. Find out more about sending to your Kindle.

      Note you can select to send to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be sent to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

      Find out more about the Kindle Personal Document Service.

      Down's syndrome and dementia
      Available formats
      ×
      Send article to Dropbox

      To send this article to your Dropbox account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Dropbox.

      Down's syndrome and dementia
      Available formats
      ×
      Send article to Google Drive

      To send this article to your Google Drive account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Google Drive.

      Down's syndrome and dementia
      Available formats
      ×
Copyright
References
Hide All
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 Geriatric Society, 40, 449453.
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.
Aylward, E. H., Li, Q., Honeycutt, N. A. et al (1999) MRI volumes of the hippocampus and amygdala in adults with Down's syndrome with and without dementia. American Journal of Psychiatry, 156, 564568.
Bouman, W. P. & Pinner, G. (2002) Use of atypical antipsychotic drugs in old age psychiatry. Advances in Psychiatric Treatment, 8, 4958.
Burt, D. B. & Aylward, E. H. (2000) Test battery for the diagnosis of dementia in individuals with intellectual disability. Journal of Intellectual Disability Research, 44, 175180.
Cooper, S. A. & Prasher, V. P. (1998) Maladaptive behaviours and symptoms of dementia in adults with Down's syndrome compared with adults with intellectual disabilities of other aetiologies. Journal of Intellectual Disability Research, 42, 293300.
Cosgrave, M. P., Tyrrell, J., McCarron, M. et al (2000) A five year follow-up study of dementia in persons with Down's syndrome: early symptoms and patterns of deterioration. Irish Journal of Psychological Medicine, 17, 511.
Evenhuis, H. M. (1990) The natural history of dementia in Down's syndrome. Archives of Neurology, 47, 263267.
Evenhuis, H. M. (1996) Further evaluation of the Dementia Questionnaire for Persons with Mental Retardation (DMR). Journal of Intellectual Disability Research, 40, 369373.
Fraser, B. (1999) Psychopharmacology and people with learning disability. Advances in Psychiatric Treatment, 5, 471477.
Gedye, A. (1995) Dementia Scale for Down's Syndrome. Manual. Vancouver, BC: Gedye Research and Consulting.
Hemingway-Eltomey, J. M. & Lerner, A. J. (1999) Adverse effects of donepezil in treating Alzheimer's disease associated with Down's syndrome. American Journal of Psychiatry, 156, 1470.
Holland, A. J., Hon, J., Huppert, F. A. et al (1998) Population-based study of the prevalence and presentation of dementia in adults with Down's syndrome. British Journal of Psychiatry, 172, 493498.
Holland, A. J., Hon, J., Huppert, A. et al (2000) Incidence and course of dementia in people with Down's syndrome: findings from a population-based study. Journal of Intellectual Disability Research, 44, 138146.
Kishnani, P. S., Spiridigliozzi, G. A., Heller, J. H. et al (2001) Donepezil for Down's syndrome (comment). American Journal of Psychiatry, 158, 143.
Lawlor, B. A., McCarron, M., Wilson, G. et al (2001) Temporal lobe-oriented CT scanning and dementia in Down's syndrome. International Journal of Geriatric Psychiatry, 16, 427429.
Mann, D. M., Iwatsubo, T., Ihara, Y. et al (1996) Predominant deposition of amyloid-beta 42(43) in plaques in cases of Alzheimer's disease and hereditary cerebral haemorrhage associated with mutations in the APP gene. American Journal of Pathology, 148, 12571266.
Moss, M. B., Albert, M. S., Butters, N. et al (1986) Differential patterns of memory loss among patients with Alzheimer's disease, Huntington's disease, and alcoholic Korsakoff's syndrome. Archives of Neurology, 43, 239246.
Oliver, C., Crayton, L., Holland, A. et al (1998) A four year prospective study of age-related cognitive change in adults with Down's syndrome. Psychological Medicine, 28, 13651377.
Prasher, V. P., Huxley, A. & Haque, M. S. (2002) A 24-week, double-blind, placebo-controlled trial of donepezil in patients with Down syndrome and Alzheimer's disease. Pilot study. International Journal of Geriatric Psychiatry, 17, 270278.
Rabe, A., Wisniewski, K. E., Schupf, N. et al (1990) Relationship of Down's syndrome to Alzheimer's disease. In Application of Basic Neuroscience to Child Psychiatry (eds Deutsch, S. I., Weizman, A. & Weizman, R.) pp. 325340. New York: Plenum Press.
Reiss, S., Levitan, G. W. & Szyszko, J. (1982) Emotional disturbance and mental retardation: diagnostic overshadowing. American Journal of Mental Deficiency, 86, 567574.
Royal College of Psychiatrists (2001) DC–LD [Diagnostic Criteria for Psychiatric Disorders for Use with Adults with Learning Disabilities/Mental Retardation] (Occasional Paper OP48). London: Gaskell.
Schupf, N. (2002) Genetic and host factors for dementia in Down's syndrome. British Journal of Psychiatry, 180, 405410.
Schupf, N., Kapell, D., Nightingale, B. et al (2001) Specificity of the fivefold increase in Alzheimer's disease in mothers of adults with Down syndrome. Neurology, 57, 979984.
Seltzer, G. B., Schupf, N. & Wu, H. S. (2001) A prospective study of menopause in women with Down syndrome. Journal of Intellectual Disability Research, 45, 17.
Simard, M. & Van Reekum, R. (2001) Dementia with Lewy bodies in Down's syndrome. International Journal of Geriatric Psychiatry, 16, 311320.
Temple, V., Jozsvai, E., Konstantareas, M. M. et al (2001) Alzheimer dementia in Down's syndrome: the relevance of cognitive ability. Journal of Intellectual Disability Research, 45, 4755.
Tyrell, J., Cosgrave, M., McCarron, M. et al (2001) Dementia in people with Down's syndrome. International Journal of Geriatric Psychiatry, 16, 11681174.
Visser, F. E., Aldenkamp, A. P., Van Huffelen, A. C. et al (1997) Prospective study of the prevalence of Alzheimer-type dementia in institutionalized individuals with Down syndrome. American Journal on Mental Retardation, 101, 400412.
Wisniewski, K. E., Wisniewski, 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.
Recommend this journal

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

BJPsych Advances
  • ISSN: 1355-5146
  • EISSN: 1472-1481
  • URL: /core/journals/bjpsych-advances
Please enter your name
Please enter a valid email address
Who would you like to send this to? *
×

Metrics

Altmetric attention score

Full text views

Total number of HTML views: 0
Total number of PDF views: 19 *
Loading metrics...

Abstract views

Total abstract views: 269 *
Loading metrics...

* Views captured on Cambridge Core between 2nd January 2018 - 25th May 2018. This data will be updated every 24 hours.

Down's syndrome and dementia

  • Lisa R. Stanton and Rikus H. Coetzee
Submit a response

eLetters

No eLetters have been published for this article.

×

Reply to: Submit a response


Your details


Conflicting interests

Do you have any conflicting interests? *