Hostname: page-component-78c5997874-fbnjt Total loading time: 0 Render date: 2024-11-19T14:52:24.109Z Has data issue: false hasContentIssue false

A five year follow-up study of dementia in persons with Down's syndrome: early symptoms and patterns of deterioration

Published online by Cambridge University Press:  13 June 2014

Mary P Cosgrave
Affiliation:
North Eastern Health Board, Cavan/Monaghan Mental Health Services, Cavan General Hospital, Cavan, Ireland
Janette Tyrrell
Affiliation:
Eastern Health Board, St Vincent's Hospital, Fairview, Dublin 3, Ireland
Brian A Lawlor
Affiliation:
University of Dublin, Trinity Centre for Health Sciences, St James's Hospital, Dublin 8, Ireland

Abstract

Objectives: To investigate the development of dementia over a five year follow up period in a population of females with Down's syndrome; to examine age at onset and duration of dementia in the population; to document the clinical features of dementia and to highlight scores on functional and cognitive rating scales at diagnosis of dementia and at the onset of complete dependency.

Method: A five year follow-up study of 80 female subjects on prevalence of dementia, early clinical features of dementia and patterns of scoring on rating scales at diagnosis and end-stage dementia was completed. Results: Over the five year study period the number of subjects diagnosed with dementia rose from seven (8.75%) to 35 (43.75%). Age related prevalence figures showed that dementia was more common with increasing age. The earliest recognisable symptoms of dementia were memory loss, spatial disorientation and loss of independence especially in the area of personal hygiene. These findings were confirmed by the rating scales used in the study.

Conclusions: The earliest recognisable clinical features of dementia include memory loss and increased dependency. The results of this study should facilitate earlier diagnosis of dementia in DS.

Type
Original Papers
Copyright
Copyright © Cambridge University Press 2000

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.Ellis, WG, McCulloch, JR, Corley, CL. Presenile dementia in Down's syndrome; ultrasrructural identity with Alzheimer's disease. Neurology 1974; 24: 101–6.CrossRefGoogle ScholarPubMed
2.Devenny, D, Silverman, WP, Hill, ACet al.Normal ageing in adults with Down's syndrome: a longitudinal study. J Intellect Dis Res 1996; 40: 208–21.CrossRefGoogle ScholarPubMed
3.Burt, DB, Loveland, KA, Primeaux-Hart, Set al.Dementia in adults with Down's syndrome: diagnostic challenges. Am J Ment Retard 1998; 103:130–45.2.0.CO;2>CrossRefGoogle ScholarPubMed
4.Evenhuis, HM. The natural history of dementia in Down's syndrome. Arch Neurol 1990; 47: 263–7.CrossRefGoogle ScholarPubMed
5.Lai, F, Williams, RS. A prospective study of Alzheimer's disease in Down's syndrome. Arch Neurol 1989; 46: 849–53.CrossRefGoogle Scholar
6.Visser, FE, Aldenkamp, AP, van Huffelen, ACet al.Prospective study or the prevalence of Alzheimer-type dementia in institutionalised individuals with Down's syndrome. Am J Ment Retard 1997; 101: 4: 400–12.Google ScholarPubMed
7.Prasher, VP. Age-specific prevalence, thyroid dysfunction and depressive symptomatology in adults with Down's syndrome and dementia. Int J Ger Psychiatry 1995; 10:2531.CrossRefGoogle Scholar
8.Holland, AJ, Hon, J, Huppert, Fet al.Population-based study of the prevalence and presentation of dementia in adults with Down's syndrome. Br J Psychiatry; 1998;172:493–9.CrossRefGoogle ScholarPubMed
9.Hauser, WA, Morris, M, Heston, LLet al.Seizures and myoclonus in patients with Alzheimer's disease. Neurology 1986; 36: 1226–30.CrossRefGoogle ScholarPubMed
10.McVicker, RW, Shanks, OEP, McClelland, RJ. Prevalence and associated features of epilepsy in adults with Down's syndrome. Br J Psychiatry 1994; 164: 528–32.CrossRefGoogle ScholarPubMed
11.Prasher, VP. End-stage dementia in adults with Down's syndrome. Int J Ger Psychiatry 1995; 10: 1067–9.CrossRefGoogle Scholar
12.Folstein, MF, Folstein, SE, McHugh, PH. Mini-mental state: a practical method of grading the cognitive state of patients for the clinician. J Psychiatric Res 1975; 12: 189–98.CrossRefGoogle Scholar
13.Ashford, JW, Kolm, P, Collvier, JAet al.Alzheimer patient evaluation and the Mini-Mental State: item characteristic curve analysis. J Gerontol 1989; 44:139–46.CrossRefGoogle ScholarPubMed
14.Tyrrell, J, Cosgrave, M, McLaughlin, M, Lawlor, BA. Dementia in an Irish population of Down's syndrome people. Ir J Psycholog Med 1996; 13: 51–4.CrossRefGoogle Scholar
15.World Health Organisation. The ICD-10 Classification of Mental and Behavioural Disorders. Clinical Descriptions and Diagnostic Guidelines. Geneva: WHO, 1992.Google Scholar
16.Albert, M, Cohen, C. The Test for Severe Impairment: an instrument for the assessment of patients with severe cognitive dysfunction. J Am Ger Soc 1992; 40: 449–53.CrossRefGoogle ScholarPubMed
17.Cosgrave, MP, McCarron, M, Anderson, Met al.Cognitive decline in Down's syndrome: a validity/reliability study of the Test for Severe Impairment. Am J Ment Retard 1998; 103: 193–7.2.0.CO;2>CrossRefGoogle ScholarPubMed
18.National Institute of Aging. The Daily Living Skills Questionnaire 1989.Google Scholar
19.Aylward, E, Burt, D, Thorpe, Let al.Diagnosis of dementia in individuals with intellectual disability. J Intellect Disabil Res 1997; 41: 152–64.CrossRefGoogle ScholarPubMed
20.Pary, R. Differential diagnosis of functional decline in Down's Syndrome. The Habilitative Mental Healthcare Newsletter 1992: 11: 3741.Google Scholar
21.Baird, PA, Sadovnick, AD. Life expectancv in Down's syndrome adults. Lancet 1988; 2: 1354–6.CrossRefGoogle ScholarPubMed