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Normal ageing in adults with Down's syndrome: a
longitudinal study
D. A. Devenny,' W. P. Silverman.' A. L. Hill.^ E. Jenkins,' E. A. Sersen' & K. E. Wisniewski'
I New York State University for Basic Research in Developmental Disabilities, and
1 The Third Foundation, Staten Island, New York, USA
Abstract
The ubiquitous presence of the neuropathoiogy of
Alzheimer disease (AD) in individuals with Down's
syndrome{DS)over40yearsofagesuggests that this
group of people will exhibit a high prevalence of
dementia of the Alzheimer type (DAT) as they age. The
present study indicates that there is a clear discrepancy
between the presumed presence of AD neuropathoiogy
and the clinical expression of DAT among older people
with DS. In the first 6 years of a longitudinal study, the
present authors compared 91 adults (31 -63 years of
age) with DS and mild or moderate mental retardation to
64adults(3l-76yearsofage) with other forms of mental
retardation (MR) on yearly measures of mental status,
short- and long-term memory, speeded psychomotor
function, and visuospatial organization. The results
indicated that, over repeated testing on the verbal
long-term memory test, younger participants with DS
showed small increases in their scores, while older
participants with DS showed very slight decreases.
Overall performance scores on this test and a speeded
psychomotor task were poorer for both diagnostic
groups in individuals aged SO years and older. The
magnitude and type of these selective changes in
performance were consistent with performance profiles
Correspondence: Dartynne A. Devenny PhD, Department of
Psychology, NYS Institute for Basic Research. 1050 Foresi Hill
Road, Siaten Island, NY 10314, USA.
1996 Blackwell Science Ltd
observed in older healthy adults without mental
retardation on tests measuring similar cognitive
functions. Only four out of the 91 people with DS in the
present sample showed changes in funaioning that have
led to a diagnosis of possible DAT. and in these
individuals, alternative causes of performance declines
were concurrently present (e.g, thyroid dysfunction).
These findings indicate that some age-associated changes
in funaioning are related to 'normal' but probably
precocious ageing among adults with DS. Furthermore,
these findings suggest that adults with DS and mild or
moderate mental retardation may be at lower risk for
dementia during their fourth and fifth decades of life than
previous studies have suggested.
Introduction
An extensive body of literature documents that
people with Down's syndrome (DS) are more likely
to show declines in ftinctioning after reaching 50
years of age than are adults with mental retardation
(MR) from other aetiologies (Jervis 1948; Olson &
Shaw 1969; Dalton et al. 1974; Wisniewski et al.
1978; Thase 1982; Thase et al. 1984; Zigman et al.
1993). These declines have been attributed to the
presence of Alzheimer disease (AD) neuropathoiogy
which has been found in virtually all cases over 40
years of age that have come to autopsy (for review
see Mann 1993). At the same time, there is a lack of
concordance between the number of individuals
 Journal of Intellectual Disability Research
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D A Devenny et al. * Normal ageing in adults with Down's syndrome
years of age over 6 years, and have found that, with
only four exceptions, these individuals have
essentially maintained their initial performance
levels. Clearly, had dementia been characteristic of
these individuals, systematic declines in test scores
should have been observed. In fact, subjects with
DS in theirfiftiesdid show relatively lower levels of
performance on measures of recent memory and a
time-constrained coding test, but their overall
performance profiles were suggestive of precocious
but 'normal' ageing. Thus, there is a real
discrepancy between the prevalence of clinical and
neuropathological features of AD in adults with DS.
This indicates that there is no compelling
justification for assuming that members of this
population are experiencing DAT unless clinicians
employ detailed and multidiscipHnary evaluations,
and have ruled out all possible alternative
explanations for observed clinical symptoms.
Acknowledgements
The authors wish to express their thanks to Ausma
Rabe, Diana Burt, Daisy Edmundson, Maureen
Marlow and Ellen Zimmerli for their critical and
editorial comments on the manuscript. We also wish
to thank the following agencies that have supported
and encouraged our studies: AHRC New York
League Workshops in Manhattan and the Bronx;
ARC of Westchester; Brooklyn Guild for
Exceptional Children and Conklin Residence;
Catholic Charities Brooklyn Diocese; Pathfinder
Village; and Wassaic Developmental Center. This
research was supported in part by funds from New
York State Office of Mental Retardation and
Developmental Disabilities, and NIH grants Poi
HD 22634 and Por AG 11531 to H.M.W.
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