Abstract
It is expected that the number of people with dementia will reach 66 million by 2030 and 115 million by 2050 [1]. It has also been noted that the number of cases of early onset dementia and young onset dementia (under 65 and under 45 respectively), while still infrequent are rising [2]. Overall prevalence rates of 5–7% have been reported in those over 60 [3] with prevalence rates of 2–10% for those under 65 years reported [1]. As has been widely reported, these figures are starkly different to those seen in a population of people with intellectual disabilities (ID), and in particular for those with Down syndrome (DS). Individuals with DS have a third copy of chromosome 21, trisomy 21, and this leads to 4–5 time the expression of amyloid precursor protein (APP) [4]. This overexpression of APP leads to increased amyloid deposition in the brain. This in turn leads to an increase in amyloid B deposition [5], where Amyloid B is known as a key contributor to Alzheimer’s disease (AD). A number of positron emission tomography (PET) studies have shown abnormal amyloid binding in individuals with DS [6] with abnormal binding found in those as young as 39 [7]. Thus, while these studies have shown neuropathological hallmarks prior to the fourth decade, the average age of clinical diagnosis has been found to be between 51 and 56, with an average duration of 3.5–6 years [8–10].
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Prince M, Jackson J. World Alzheimer’s report London. London: Alzheimer’s Disease International, Kings College, London; 2009.
Masellis M, Sherborn K, Neto PR, et al. Early-onset dementias: diagnostic and etiological considerations. Alzheimers Res Ther. 2013;5(Suppl 1):S7.
World Health Organization. Dementia: a public health priority. World Health Organization; 2012.
Jennings D, Seibyl J, Sabbagh M, et al. Age dependence of brain β-amyloid deposition in Down syndrome: an [18F]florbetaben PET study. Neurology. 2015;84:500–7.
Wiseman FK, Al-Janabi T, Hardy J, et al. A genetic cause of Alzheimer disease: mechanistic insights from Down syndrome. Nat Rev Neurosci. 2015;16:564–74.
Landt J, D’Abrera JC, Holland AJ, et al. Using positron emission tomography and carbon 11–labeled Pittsburgh compound B to image brain fibrillar β-amyloid in adults with Down syndrome. Safety, acceptability, and feasibility. Arch Neurol. 2011;68:890–6. doi:10.1001/archneurol.2011.36.
Annus T, Wilson LR, Hong YT, et al. The pattern of amyloid accumulation in the brains of adults with down syndrome. Alzheimers Dement. 2015;12:538–45.
McCarron M, McCallion P, Reilly E, et al. A prospective 14-year longitudinal follow-up of dementia in persons with Down syndrome. J Intellect Disabil Res. 2014;58:61–70.
Prasher VP, Krishnan VHR. Age of onset and duration of dementia in people with Down syndrome: integration of 98 reported cases in the literature. Int J Geriatr Psychiatry. 1993;8:915–22.
Lai F, Williams R. A prospective study of Alzheimer disease in Down’s syndrome. Arch Neurol. 1989;46:849–53.
Strydom A, Shooshtari S, Lee L, et al. Dementia in older adults with intellectual disabilities—epidemiology, presentation, and diagnosis. J Policy Pract Intellect Disabil. 2010;7:96–110.
Coppus AM, Evenhuis H, Verberne GJ, et al. Dementia and mortality in persons with Down’s syndrome. J Intellect Disabil Res. 2006;50:768–77.
Holland AJ, Hon J, Huppert FA, et al. A population-based study of the prevalence and presentation of dementia in adults with Down syndrome. Br J Psychiatry. 1998;172:493–8.
Tyrrell J, Cosgrave M, McCarron M, et al. Dementia in people with Down’s syndrome. Inter J Geriatr Psychiatry. 2001;16:1168–74.
Visser FE, Aldenkamp AP, van Huffelen AC, et al. Prospective study of the prevalence of Alzheimer-type dementia in institutionalized individuals with Down syndrome. Am J Ment Retard. 1997;101:400–12.
McCarron M, Mulryan N, Reilly E, et al. A prospective 20 year longitudinal follow-up of dementia in older adults with Down syndrome. J Intellect Disabil Res. 2017; doi: 10.1111/jir.12390.
Nagdee M. Dementia in intellectual disability: a review of diagnostic challenges. Afr J Psychiatry. 2011;14:194–9.
Cullen B, O’Neill B, Evans JJ, et al. A review of screening tests for cognitive impairment. J Neurol Neurosurg Psychiatry. 2007;78:790–9.
Hassiotis A, Strydom A, Allen K, et al. A memory clinic for older people with intellectual disabilities. Aging Ment Health. 2003;7:418–23.
Prasher V, Cumella S, Natarajan K, et al. Magnetic resonance imaging, Down’s syndrome and Alzheimer’s disease: research and clinical implications. J Intellect Disabil Res. 2003;47:90–100.
Prasher VP. Alzheimer’s disease and dementia in Down syndrome and intellectual disabilities. Milton Keynes: Radcliffe Publishing, Oxford; 2005.
Foreman P, Gardner I, Davis S. Multidisciplinary memory clinics: what is important to caregivers and clients? Int J Geriatr Psychiatry. 2004;19:588–9.
McCarron M, Lawlor BA. Responding to the challenges of ageing and dementia in intellectual disability in Ireland. Aging Ment Health. 2003;7:413–7.
McCreary BD, Fotheringham J, Holden J, et al. Experiences in an Alzheimer clinic for persons with Down syndrome. In: Berg JM, Karlinsky H, Holland AJ, editors. Alzheimer disease, Down syndrome and their relationship. Oxford: Oxford University Press; 1993. p. 115–31.
Chicoine B, McGuire D, Rubin SS. Specialty clinic perspectives. In: Janicki MP, Dalton AJ, editors. Dementia, aging and intellectual disabilities: a handbook. Castletown: Hamilton Printing; 1999. p. 278–93.
Cahill S, Moore V, Pierce M. Memory clinics in Ireland. Dublin: DSIDC; 2011.
Burt DB, Aylward EH. Test battery for the diagnosis of dementia in individuals with intellectual disability. J Intellect Disabil Res. 2000;44:175–80.
Moran JA, Rafii MS, Keller SM, et al. The National Task Group on intellectual disabilities and dementia practices consensus recommendations for the evaluation and management of dementia in adults with intellectual disabilities. Mayo Clin Proc. 2013;88:831–40.
Appollonio I, Gori C, Riva GP, et al. Cognitive assessment of severe dementia: the test for severe impairment (TSI). Arch Gerontol Geriatr. 2001;7(Suppl):25–31.
Albert M, Cohen C. The test for severe impairment: an instrument for the assessment of patients with severe cognitive dysfunction. J Am Geriatr Soc. 1992;40:449–53.
Foldi NS, Majerovitz SD, Sheikh K, et al. The test for severe impairment: validity with the dementia rating scale and utility as a longitudinal measure. Clin Neuropsychol. 1999;13:22–9.
Mattis S. Dementia rating scale: professional manual. Odessa: Psycho Assess Resources; 1988.
Mack W, Freed D, Williams BW, et al. Boston naming test: shortened version for use in Alzheimer’s disease. J Gerontol Psychol Sci. 1992;47:154–8.
Jacobs DM, Albert SM, Sano M, et al. Assessment of cognition in advanced AD: the test for severe impairment. Neurology. 1999;52:1689–91.
Stern Y, Sano M, Paulson J, et al. Modified mini-mental state examination: validity and reliability. Neurology. 1987;37(S1):179. Abstract.
Appollonio I, Gori C, Riva G, et al. Assessing early to late stage dementia: the TSI and BANS-S scales in the nursing home. Int J Geriatr Psychiatry. 2005;20:1138–45.
Volicer L, Hurley AC, Lathi DC, et al. Measurement of severity in advanced Alzheimer’s disease. J Gerontol. 1994;49:M223–6.
Tyrrell JF, Cosgrave MP, McLaughlin M, et al. Dementia in an Irish population of Down’s syndrome people. Ir J Psychol Med. 1996;13:51–4.
Cosgrave MP, McCarron M, Anderson M, et al. Cognitive decline in Down syndrome: a validity/reliability study of the test for severe impairment. Am J Ment Retard. 1998;103:193–7.
Haxby JV. Neuropsychological evaluation of adults with Down’s syndrome: patterns of selective impairment in non-demented old adults. J Ment Defic Res. 1989;33:193–210.
Cosgrave MP. Clinical and biological aspects of dementia in Down’s syndrome. MD thesis, shelf mark 5643. Dublin: University of Dublin, Trinity College Dublin; 2000.
Visser FE, Kuilman M. Dementia symptoms in Down’s syndrome in a residentially treated group of mentally handicapped. Ned Tijdschr Geneeskd, Nederlands. 1990;134:1141–5.
National Institute of Aging, Laboratory of Neurosciences. The daily living skills questionnaire. National Institute of Aging US; 1989.
Pary R. Differential diagnosis of functional decline in Down’s syndrome. Habilitative Ment Healthc Newslett. 1992;11:37–41.
Evenhuis HM, Eurlings HAL, Kengen MMF. Dementia questionnaire for mentally retarded persons (DMR): for diagnosis of dementia in mentally retarded people-1990. Zwammerdam: Hooge Burch Institute for Mentally Retarded People; 1990.
Evenhuis HM, Kengen MMF, Eurlings HAL. Dementia questionnaire for people with learning disabilities, Amsterdam: Harcourt Test Publisher. 2006. https://www.pearsonclinical.com.au/products/view/113.
Aylward E, Burt D, Thorpe LU, et al. Diagnosis of dementia in individuals with intellectual disability. J Intellect Disabil Res. 1997;41:152–64.
Stern RG, Mohs RC, Bierer LM, et al. Deterioration on the blessed test in Alzheimer’s disease: longitudinal data and their implication for clinical trials and identification of subtypes. Psychol Res. 1992;42:101–10.
Seltzer GB. Modified fuld object memory evaluation. Madison: Waisman Centre, University of Wisconsin; 1997.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2018 Springer International Publishing AG
About this chapter
Cite this chapter
McCarron, M. et al. (2018). The Test for Severe Impairment. In: Prasher, V. (eds) Neuropsychological Assessments of Dementia in Down Syndrome and Intellectual Disabilities. Springer, Cham. https://doi.org/10.1007/978-3-319-61720-6_8
Download citation
DOI: https://doi.org/10.1007/978-3-319-61720-6_8
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-61719-0
Online ISBN: 978-3-319-61720-6
eBook Packages: MedicineMedicine (R0)