Hostname: page-component-848d4c4894-p2v8j Total loading time: 0.001 Render date: 2024-05-16T00:22:12.730Z Has data issue: false hasContentIssue false

Dementia in an Irish population of Down's syndrome people

Published online by Cambridge University Press:  13 June 2014

Janette F TyrRell
Affiliation:
Trinity Centre for Health Sciences, St James's Hospital, Dublin 8, Ireland
Mary P Cosgrave
Affiliation:
Mental Health and Aging, St Patrick's Hospital, James's St, Dublin 8, Ireland
Martin McLaughlin
Affiliation:
Daughters of Charity Service, St Vincent's Centre, Navan Road, Dublin 7, Ireland
Brian A Lawlor
Affiliation:
Mental Health and Aging, St Patrick's Hospital, James's St, Dublin 8, Ireland

Abstract

Objective: To determine the prevalence of dementia in an Irish sample of Down's syndrome (DS) patients and to examine the utility of a number of cognitive and functional scales in the assessment of dementia in this population.

Method: 76 DS patients diagnosed clinically (range 33–72 years; mean age 47.3 ± 8.8 years) were included in the study. The diagnosis of dementia was made on clinical grounds using DSMIIIR criteria. Cognitive and functional impairment were evaluated using the following scales; Test for Severe Impairment (TSI), Down's Syndrome Mental Status Examination (DSMSE), Daily Living Skills Questionnaire (DLSQ), and the Mental State Performance (MSP).

Results: The overall prevalence of dementia was 7.9% (95% C.I = 2.95–16.39). The presence of dementia was associated with late onset epilepsy, anticonvulsant medication and deafness. Standard cognitive tests such as the MSP showed an early ‘floor’ effect in this population. In contrast the TSI and DLSQ showed a satisfactory range of scores in these patients with moderate to severe learning disability.

Conclusions: The low prevalence of dementia in this study may be explained by the strict conservative criteria applied in the clinical diagnosis. Prospective assessment of DS patients on a longitudinal basis using decline on scales such as the TSI and DLSQ may allow more accurate diagnosis of dementia at an earlier stage in this at-risk population.

Type
Original Papers
Copyright
Copyright © Cambridge University Press 1996

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

1.Heller, J. Human chromosome aberrations as related to physical and mental dysfunction. J Hered 1969; 60: 239–48.CrossRefGoogle ScholarPubMed
2.Heston, LL. Alzheimer's disease and Down's syndrome: new insights. Ann of Intern Med 1985; 103: 566–78.Google Scholar
3.Yatham, L, McHale, P, Kinsella, A. Down's Syndrome and its association with Alzheimer's disease. Acta Psychiatr Scand 1988; 77: 3841.CrossRefGoogle ScholarPubMed
4.Schupf, N, Kapell, D, Lee, JH, Ottman, R, Mayeux, R. Increased risk of Alzheimer's Disease in mothers of adults with Down's syndrome. Lancet 1994; 344: 353–6.CrossRefGoogle ScholarPubMed
5.Karlinsky, H. Alzheimer's disease in Down's syndrome. J Am Ger Soc 1986; 34: 728–34.CrossRefGoogle ScholarPubMed
6.Mann, DMA. Alzheimer's disease and Down's syndrome. Histopath 1988; 13:125–37.CrossRefGoogle ScholarPubMed
7.Winiewski, K, Dalton, A, Crapper McLachlan, DR, Wen, GY, Winiewski, HM. Alzheimer's disease in Down's syndrome: clinicopathological studies. Neurology 1985;35:957–61.CrossRefGoogle Scholar
8.Rumble, M, Retallack, R, Hilbich, C, ét al. Amyloid A4 protein and its precursors in Down's syndrome and Alzheimer's disease. N Engl J Med 1989; 320: 1446–52.CrossRefGoogle ScholarPubMed
9.Thase, ME, Liss, L, Smeltzer, D, Maloon, J. Clinical evaluation of dementia in Down's syndrome. J Ment Defic Res 1982; 26: 239–44.Google ScholarPubMed
10.Dalton, AJ, Crapper McLachlan, DR. Clinical expression of Alzheimer's disease in Down's syndrome. Psych Clin North Am 1986; 4: 659–70.CrossRefGoogle Scholar
11.Fenner, ME, Hewitt, KE, Torpy, DH. Down's syndrome: intellectual and behavioural functioning during adulthood. J Ment Defic Res 1987; 31: 241–9.Google ScholarPubMed
12.Evenhuis, HM. The natural history of dementia in Down's syndrome. Arch Neurol 1990; 47: 263–7.CrossRefGoogle ScholarPubMed
13.Lai, F, Williams, RS. A prospective study of Alzheimer's disease in Down's syndrome. Arch Neurol 1989; 46: 849–53.CrossRefGoogle Scholar
14.Oliver, C, Holland, AJ. Down's syndrome and Alzheimer's disease: a review. Psych Med 1986; 16:307–22.CrossRefGoogle ScholarPubMed
15.Prasher, VP, Krishnan, VHR. Age of onset and duration of dementia in people with Down's syndrome. Int J Ger Psychiat 1993; 8:915–22.CrossRefGoogle Scholar
16.Haxby, JV. Neuropsychological evaluation of adults with Down's syndrome; patterns of selective impairment in nondemented old adults. J Ment Defic Res 1989; 33:193–210.Google ScholarPubMed
17.Brugge, KL, Nichols, SL, Salmon, DP, et al.Cognitive impairment in adults with Down's syndrome. Neurology 1994; 44: 232–8.CrossRefGoogle ScholarPubMed
18.Tangye, SR. The EEG and the incidence of epilepsy in Down's syndrome. J Ment Defic Res 1979; 23: 1724.Google ScholarPubMed
19.Blackwood, DHR, St Clair, DM, Muir, WJ, Oliver, CJ, Dickens, P. The development of Alzheimer's disease in Down's syndrome assessed by auditory event-related potentials. J Ment Defic Res 1988; 32:439–53.Google ScholarPubMed
20.Schapiro, MB, Azari, NP, Grady, CL, Haxby, JV, Horwitz, B. Down's syndrome: differentiating mental retardation and dementia, in Nadel, L, CJ, Epstein, editors. Down's syndrome and Alzheimer disease. New York: Wiley-Liss, 1992: 103–22.Google Scholar
21.Aylward, E, Burt, D, Thorpe, L, Lai, F, Dalton, A. Diagnosis of dementia in individuals with intellectual disability. Washington: American Association on Mental Retardation 1995.Google Scholar
22.International League against Epilepsy: commission on classification and terminology. Proposal for revised clinical and electroencephalographic classification of epileptic seizures. Epilepsia 1981; 22:489–501.CrossRefGoogle Scholar
23.Pary, R. Differential diagnosis of functional decline in Down's syndrome. The habilitative mental health newsletter 1992; 6: 3741.Google Scholar
24.Keane, V, O'Neill, D, Walsh, JB, Coakley, D, Coffey, V, Mulcahy, M. Difficulties in diagnosing Alzheimer's disease in Down's Syndrome, in O'Neill, D, ed. Carers, professionals and Alzheimer's disease. London: John Libbey, 1991:315–21.Google Scholar
25.Warren, AC, Holroyd, S, Folstein, MF. Major depression in Down's syndrome. Br J Psych 1989; 155: 202–5.CrossRefGoogle ScholarPubMed
26.Albert, M, Cohen, C. The test for severe impairment: an instrument for the assessment of patient with severe cognitive dysfunction. J Am Ger So. 1992; 40:449–53.CrossRefGoogle ScholarPubMed
27.Prasher, VP, Corbett, JA. Onset of seizures as a poor indicator of longevity in people with Down's syndrome and dementia. Int J Ger Psychiat 1993; 8:923–7.CrossRefGoogle Scholar
28.McVicker, R, Shanks, O, McClelland, R. Prevalence and associated features of epilepsy in adults with Down's syndrome. Br J Psychiat 1994; 164: 528–32.CrossRefGoogle ScholarPubMed
29.Soininen, H, Partanen, J, Jousmaki, V, et al.Age-related cognitive decline and EEG slowing in Down's syndrome as a model of Alzheimer's disease. Neuroscience 1993; 53:5763.CrossRefGoogle Scholar