Hostname: page-component-586b7cd67f-g8jcs Total loading time: 0 Render date: 2024-12-02T19:27:02.720Z Has data issue: false hasContentIssue false
  • English
  • Français

The challenges of diagnosis and treatment of dementia in Down’s syndrome

Published online by Cambridge University Press:  03 February 2016

R. M. Vaughan Open the ORCID record for R. M. Vaughan [Opens in a new window] ,
C. McGee ,
S. Guerin ,
J. Tyrrell  and
P. Dodd
R. M. Vaughan*
Affiliation:
Department of Old Age Psychiatry, St Ita’s Hospital, Portrane, Co. Dublin, Ireland
C. McGee
Affiliation:
Department of Psychiatry, Our Lady’s Hospital, Navan, Co. Meath, Ireland
S. Guerin
Affiliation:
School of Psychology, Centre for Disability Studies, University College Dublin, Dublin, Ireland
J. Tyrrell
Affiliation:
St Michael’s House Intellectual Disability Service, Dublin, Ireland
P. Dodd
Affiliation:
St Michael’s House Intellectual Disability Service, Dublin, Ireland School of Medicine, Trinity College, University of Dublin, Dublin, Ireland
*
*Address for correspondence: Dr R. M. Vaughan, Department of Old Age Psychiatry, St Ita’s Hospital, Portrane, Co. Dublin, Ireland. (Email: [email protected])
Get access Rights & Permissions [Opens in a new window]

Abstract

Objectives

People with Down syndrome (DS) are at high risk for developing dementia and early diagnosis is vital in enhancing quality of life. Our aim was to compare our practice to consensus recommendations on evaluation, diagnosis and pharmacological treatment of individuals with DS who develop dementia. We also aimed to establish the average time taken to make a diagnosis of dementia and to commence pharmacotherapy, and to assess tolerability to acetylcholinesterase inhibitors.

Methods

Retrospective chart review in an exhaustive sample containing all current service users attending our service with DS and a diagnosis of dementia (n=20).

Results

The sample was 75% female and 70% had a moderate intellectual disability. The average age at diagnosis of dementia was 52.42 years old. The average time to diagnosis from first symptom was 1.13 years and the average time to commence pharmacotherapy was 0.23 years. A total of 17 patients commenced on acetylcholinesterase inhibitors, and of these seven discontinued medication due to side-effects or lack of efficacy.

Conclusions

The results on anticholinesterases add to the limited pool of data on treatment of dementia in DS. There was an identified need to improve the rates of medical, vision and hearing assessments, and prospective screening. Deficiencies in screening and diagnosis may be addressed by implementing a standardised dementia assessment pathway to include prospective screening and longitudinal assessment using easily administered scales. We highlight the importance of improving the diagnostic process, as a vital window of opportunity to commence a comprehensive care plan may be lost.

Keywords

Acetylcholinesterase inhibitorsAlzheimer’s diseasedementiaDown syndromeintellectual disability
Type
Original Research
Information
Irish Journal of Psychological Medicine , Volume 33 , Issue 3 , September 2016 , pp. 151 - 158
Copyright
© College of Psychiatrists of Ireland 2016 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

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, 449–453.Google Scholar
Aylward, EH, Burt, DB, Thorpe, LU, Lai, F, Dalton, A (1997). Diagnosis of dementia in individuals with intellectual disability. Journal of Intellectual Disability Research 41, 152–164.Google Scholar
Ball, SL, Holland, AJ, Huppert, FA, 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 syndrome. Journal of Intellectual Disability Research 48, 611–620.CrossRefGoogle Scholar
Bland, R, Hutchinson, N, Oakes, P, Yates, C (2003). Double jeopardy? Needs and services for older people who have learning disabilities. Journal of Learning Disabilities 7, 323–344.CrossRefGoogle Scholar
Burt, D, Aylward, E (2000). Test battery for the diagnosis of dementia in individuals with intellectual disability. Journal Of Intellectual Disability Research 44, 175–180.Google Scholar
Burt, DB, Loveland, KA, Chen, YW, Chuang, A, Lewis, KR, Cherry, L (1995). Ageing in adults with Down syndrome: a report from a longitudinal study. American Journal on Mental Retardation 100, 262–270.Google Scholar
Cahill, S, Clark, M, Walsh, C, O’Connell, H, Lawlor, B (2006). Dementia in primary care: the first survey of Irish general practitioners. International Journal of Geriatric Psychiatry 21, 319–324.Google Scholar
Coppus, AM, Evenhuis, HM, Verberne, GJ, Visser, FE, Oostra, BA, Eikelenboom, P, Van Duijn, CM (2008). Survival in elderly persons with Down syndrome. Journal of the American Geriatrics Society 56, 2311–2316.CrossRefGoogle ScholarPubMed
Deb, S, Braganza, J (1999). Comparison of rating scales for the diagnosis of dementia in adults with Down’s syndrome. Journal of Intellectual Disability Research 43, 400–407.Google Scholar
Dodd, K, Bhaumik, S, Benbow, SM (2009). Dementia and people with learning disabilities. Guidance on the assessment, diagnosis, treatment and support of people with learning disabilities who develop dementia, CR155. Royal College of Psychiatrists & British Psychological Society: Leicester.Google Scholar
Evenhuis, HM (1996). Further evaluation of the Dementia Questionnaire for Persons with Mental Retardation (DMR). Journal of Intellectual Disability Research 40, 369–373.CrossRefGoogle ScholarPubMed
Folstein, MF, Folstein, SE, Fanjiang, G (2001). MMSE, Mini-Mental State Examination. Psychological Assessment Resources.Google Scholar
Geyde, A (1995). Dementia Scale for Down’s Syndrome – Manual. Geyde Research & Consulting: Vancouver.Google Scholar
Hanney, M, Prasher, V, Williams, N, Jones, EL, Aarsland, D, Corbett, A, Ballard, C (2012). Memantine for dementia in adults older than 40 years with Down’s syndrome (MEADOWS): a randomised, double-blind, placebo-controlled trial. The Lancet 379, 528–536.Google Scholar
Holroyd, S, Turnbull, Q, Wolf, A (2002). What are patients and their families told about the diagnosis of dementia? Results of a family survey. International Journal of Geriatric Psychiatry 17, 218–221.Google Scholar
Kaplan, EF, Goodglass, H, Weintraub, S (1987). The Boston Naming Test: Experimental Edition. Kaplan & Goodglass: Boston.Google Scholar
Lai, F, Williams, RS (1989). A prospective study of Alzheimer disease in Down syndrome. Archives of Neurology 46, 849–853.Google Scholar
McCarron, M, McCallion, P, Reilly, E, Mulryan, N (2014). A prospective 14 year longitudinal follow up of dementia in persons with Down syndrome. Journal of Intellectual Disability Research 58, 61–70.CrossRefGoogle ScholarPubMed
Mohan, M, Carpenter, PK, Bennett, C (2009). Donepez il for dementia in people with Down syndrome. Cochrane Database of Systematic Reviews, doi: 10.1002/14651858.CD007178.pub2.Google Scholar
National Institute for Health and Care Excellence (NICE) (2006). Dementia: Supporting People with Dementia and their Carers in Health and Social Care [Cg42]. NICE: London.Google Scholar
Portelius, E, Soininen, H, Andreasson, U, Zetterberg, H, Persson, R, Karlsson, G, Mattsson, N (2014). Exploring Alzheimer molecular pathology in Down’s syndrome cerebrospinal fluid. Neurodegenerative Diseases 14, 98–106.Google Scholar
Prasher, VP, Adams, C, Holder, R (2003). Long term safety and efficacy of donepezil in the treatment of dementia in Alzheimer’s disease in adults with Down syndrome: open label study. International Journal of Geriatric Psychiatry 18, 549–551.Google Scholar
Prasher, VP, Huxley, A, Haque, MS (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, 270–278.CrossRefGoogle ScholarPubMed
Prasher, VP, Krishnan, VHR (1993). Age of onset and duration of dementia in people with Down syndrome: integration of 98 reported cases in the literature. International Journal of Geriatric Psychiatry 8, 915–922.Google Scholar
Strydom, A, Lee, LA, Jokinen, N, Shooshtari, S, Raykar, V, Torr, J, Tsiouris, JA, Courtenay, K, Bass, N, Sinnema, M, Maaskant, MA (2009). Report on the state of science on dementia in people with intellectual disabilities. International Association for the Scientific Study of Intellectual Disabilities (IASSID).Google Scholar
Strydom, A, Shooshtari, S, Lee, L (2010). Dementia in older adults with intellectual disabilities – epidemiology, presentation, and diagnosis. Journal of Policy and Practice in Intellectual Disability 7, 96–110.Google Scholar
Test, DW, Flowers, C, Hewitt, A, Solow, J (2003). Statewide study of the direct support staff workforce. Mental Retardation 41, 276–285.Google Scholar
Tiffin, J, Asher, EJ (1948). The Purdue Pegboard: norms and studies of reliability and validity. Journal of Applied Psychology 32, 234–247.Google Scholar
Tyrrell, J, Cosgrave, M, McCarron, M, McPherson, J, Calvert, J, Kelly, A, McLaughlin, M, Gill, M, Lawlor, BA (2001). Dementia in people with Down syndrome. International Journal of Geriatric Psychiatry 16, 1168–1174.Google Scholar
Tyrrell, J, Mulryan, N, Dodd, P (2014). A guidance document on dementia in persons with intellectual disability. Faculty of Intellectual Disability Psychiatry, College of Psychiatry of Ireland.Google Scholar