covid
Buscar en
Revista Médica Internacional sobre el Síndrome de Down (English Edition)
Toda la web
Inicio Revista Médica Internacional sobre el Síndrome de Down (English Edition) The usefulness of standard neuropsychological testing for adults with Down syndr...
Journal Information
Vol. 12. Issue 1.
Pages 2-7 (March 2008)
Share
Share
Download PDF
More article options
Vol. 12. Issue 1.
Pages 2-7 (March 2008)
Full text access
The usefulness of standard neuropsychological testing for adults with Down syndrome and dementia
Visits
955
M. Boada1,2,
, M. Alegret1, M. Buendia1, I. Hernández1, G. Viñas1, A. Espinosa1, S. Lara1, M. Guitart1, Ll. Tárraga1
1 Fundació ACE. Institut Català de Neurociències Aplicades
2 Hospital General Universitari Vall d'Hebron
This item has received
Article information
Abstract
Background

Subjects with Down syndrome (DS) have an increased risk of Alzheimer's disease (AD). As intellectual ability is lower in DS subjects than among the general population, it is difficult to determine whether cognition has deteriorated with age to the point of fulfilling AD diagnostic criteria. The Mini-Mental State Examination (MMSE) and the Severe Impairment Battery (SIB) are standard cognitive tests widely used to assess dementia in the general population. There are few studies using the MMSE and the SIB on subjects with DS where dementia is suspected. The aim of the present study was to analyse the appropriateness of the SIB and the MMSE in the cognitive assessment of aging subjects with DS.

Methods

The SIB and the MMSE were administered to 45 subjects with DS (16 with Alzheimer's disease and 29 without dementia), and the DMR questionnaire was given to their caregivers.

Results

DS subjects with dementia had higher impairment levels than DS subjects without dementia in their social and total DMR scores, but no significant differences were found between the two groups in the SIB and MMSE scores or in cognitive DMR performance. Overall, SIB scores correlated significantly with MMSE results, total DMR, cognitive DMR, and social DMR. MMSE performance correlated significantly with total and cognitive DMR scores as well as SIB score.

Conclusion

The SIB and the MMSE are useful assessment tools in monitoring cognitive function among subjects with DS and cognitive loss or dementia.

Keywords:
Dementia
Cognition
Alzheimer's disease
Down syndrome
Full text is only aviable in PDF
Bibliography
[1.]
American Psychiatric Association.
DSM-IV-TR. Breviario: Criterios diagnósticos.
Masson, (2003),
[2.]
D.A. Devenny, W.P. Silverman, A.L. Hill, E. Jenkins, E.A. Sersen, K.E. Wisniewski.
Normal ageing in adults with Down's syndrome: a longitudinal study.
J Intellect Disabil Res, 40 (1996), pp. 208-221
[3.]
J.P. Kresslak, S.F. Nagata, I. Lott, O. Nalcioglu.
Magnetic resonance imaging analysis of age-related changes in the brains of individuals with Down's syndrome.
Neurology, 44 (1994), pp. 1039-1045
[4.]
I.T. Lott, E. Head.
Down syndrome and Alzheimer's disease: a link between development and aging.
Ment Retard Dev Disabil Res Rev, 7 (2001), pp. 172-178
[5.]
J. Flórez.
Neurodegeneración y aportaciones terapéuticas.
Biogerontología, pp. 379-401
[6.]
A.J. Holland, C. Oliver.
Down syndrome and the links with Alzheimer disease.
J Neurol Neurosurg Psychiatry, 59 (1995), pp. 111-114
[7.]
M. Margallo-Lana, C.M. Morris, A.M. Gibson, et al.
Influence of the amyloid precursor protein locus on dementia in Down syndrome.
Neurology, 62 (2004), pp. 1996-1998
[8.]
B. Chicoine, D. McGuire.
Longevity of a woman with Down syndrome: a case study.
[9.]
G. McKhann, D. Drachman, M. Folstein, R. Katzman, D. Price, E.M. Stadlan.
Clinical diagnosis of Alzheimer's disease: report of NINCDSADRDA Work Group.
Neurology, 34 (1984), pp. 939-944
[10.]
J.C. Morris, A. Heyman, R.C. Mohs, et al.
The consortium to establish a registry for Alzheimer's disease (CERAD). Part I. Clinical and neuropsychological assessment of Alzheimer's disease.
Neurology, 39 (1989), pp. 1159-1165
[11.]
Clinical diagnosis and management of Alzheimer's disease,
[12.]
H. Potter.
Review and hypothesis: Alzheimer's disease and Down syndrome-chromosome 21 nondisjunction may underlie both disorders.
Am J Hum Genet, 48 (1991), pp. 1192-2000
[13.]
A. Goate, M.C. Chartier-Harlin, M. Mullan, et al.
Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease.
Nature, 349 (1991), pp. 704-706
[14.]
M.C. Chartier-Harlin, M. Parfitt, S. Legrain, et al.
Apolipoprotein E, epsilon 4 allele as a major risk factor for sporadic early and late-onset forms of Alzheimer's disease: analisys of the 19q13.2 chromosomal region.
Hum Mol Genet, 3 (1994), pp. 569-574
[15.]
K. Beyreuther, P. Pollwein, G. Multhaup, et al.
Regulation and expression of the Alzheimer's beta/A4 amyloid protein precurson in health, disease and Down's syndrome.
Ann NY Acad Sci, 695 (1993), pp. 91-102
[16.]
K.L. Goodison, I.M. Parhad, C.L. White, A.A. Sima, A.W. Clark.
Neuronal and glial gene expression in neocortex of Down's syndrome and Alzheimer's disease.
J Neuropathol Exp Neurol, 52 (1993), pp. 192-198
[17.]
C.L. Van Broeckhoven.
Molecular genetics of Alzheimer disease: identification of genes and gene mutations.
Eur Neurol, 35 (1995), pp. 8-19
[18.]
S. Deb, J. Braganza.
Comparison of rating scales for the diagnosis of dementia in adults with Down's syndrome.
J Intellect Disabil Res, 43 (1999), pp. 400-407
[19.]
S.A. Cooper, V.P. Prasher.
Maladaptive behaviours and symptoms of dementia in adults with Down's syndrome compared with adults with intellectual disability of other aetiologies.
J Intellect Disabil Res, 42 (1998), pp. 293-300
[20.]
E.H. Ayward, D.B. Burt, L.U. Thorpe, F. Lai, A. Dalton.
Diagnosis of dementia in individuals with intellectual disability.
J Intellect Disabil Res, 41 (1997), pp. 152-164
[21.]
H.M. Evenhuis, M.M.F. Kengen, H.A.L. Eurlings.
Dementia Questionnaire for Mentally Retarded Persons.
Hooge Burch, (1990),
[22.]
H.M. Evenhuis.
Evaluation of a screening instrument for dementia in ageing mentally retarded persons.
J Intellect Disabil Res, 36 (1992), pp. 337-347
[23.]
H.M. Evenhuis.
Further evaluation of the dementia questionnaire for persons with mental retardation (DMR).
J Intellect Disabil Res, 40 (1996), pp. 369-373
[24.]
M.F. Folstein, S.E. Folstein, P.R. McHugh.
Minimental state: a practical method for grading the cognitive state of patients for the clinican.
J Psychiatr Res, 12 (1975), pp. 189-198
[25.]
A.L. Politoff, R.P. Stadter, N. Monson, P. Hass.
Cognition- related EEG abnormalities in nondemented Down syndrome subjects.
Dementia, 7 (1996), pp. 69-75
[26.]
J. Hon, F.A. Huppert, A.J. Holland, P. Watson.
Neuropsychological assessment of older adults with Down's syndrome: an epidemiological study using the Cambridge Cognitive Examination (CAMCOG).
Br J Clin Psychol, 38 (1999), pp. 155-165
[27.]
J. Saxton, K.L. McGonigle, A. Swihart, F. Boller.
The Severe Impairment Battery.
Thames Valley Test Company, (1993),
[28.]
K.V. Wild, J.A. Kaye.
The rate of progression of Alzheimer's disease in the later stages: evidence from the Severe Impairment Battery.
JINS, 4 (1998), pp. 512-516
[29.]
P. Witts, S. Elders.
The Severe Impairment Battery: assessing cognitive ability in adults with Down syndrome.
Br J Clin Psychol, 37 (1998), pp. 213-216
[30.]
V.P. Prasher, A. Huxley, M.S. Haque, Down syndrome Ageing Study Group.
A 24-week, double-blind, placebo-controlled trial of donepezil in patients with Down syndrome and Alzheimer's disease-pilot study.
Int J Geriatr Psychiatry, 17 (2002), pp. 270-278
[31.]
S.J. Krinsky-McHale, D.A. Devenny, W.P. Silverman.
Changes in explicit memory associated with early dementia in adults with Down's syndrome.
J Intellect Disabil Res, 46 (2002), pp. 198-208
[32.]
M. Boada-Rovira, I. Hernández-Ruiz, S. Badenas-Homiar, M. Buendia-Torras, L. Tárraga-Mestre.
Estudio clinicoterapéutico de la demencia en las personas con síndrome de Down y eficacia del donepecilo en esta población.
Rev Neurol, 41 (2005), pp. 129-136
[33.]
F.E. Visser, A.P. Aldenkamp, A.C. Van Huffelen.
Early signs of dementia checklist.
Am J Ment Retard, 101 (1997), pp. 400-412
[34.]
J. Llinas-Regla, M. Lozano-Gallego, O.L. Lopez, et al.
Validation of the spanish version of the Severe Impairment Battery.
Neurología, 10 (1995), pp. 14-18
[35.]
V.P. Prasher.
Dementia questionnaire for person with mental retardation (DMR): modified criteria for adults with Down syndrome.
J Appl Res Intellect Disabil, 10 (1997), pp. 54-60
[36.]
M. Panisset, M. Roudier, J. Saxton, F. Boller.
Severe Impairment Battery. A neuropsychological test for severely demented patients.
Arch Neurol, 51 (1994), pp. 41-45
[37.]
M.D. Lezak.
Neuropsychological assessment.
Oxford University Press, (1995),
[38.]
R. Blesa, M. Pujol, M. Aguilar.
Clinical valicity of thee ‘Mini-mental state’ for Spanish spearking communities.
Neuropsychologia, 39 (2001), pp. 1150-1157
[39.]
V.P. Prasher, A. Filer.
Behavioural disturbance in people with Down's syndrome and dementia.
J Intellect Disabil Res, 39 (1995), pp. 432-436
[40.]
A. Strydom, A. Hassiotis.
Diagnostic instruments for dementia in older people with intellectual disability in clinical practice.
Aging Ment Health, 7 (2003), pp. 431-437

This paper received the 10Th Ramon Trias Fargas Award for Research on Down Syndrome (10th edition).

Copyright © 2008. Fundació Catalana Síndrome Down
Download PDF
Article options