Cognitive Executive Function in Down's Syndrome

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British Journal of Clinical Psychology (2006), 45, 517

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Cognitive executive function in Downs syndrome

John Rowe1*, Anthony Lavender2 and Vicky Turk3
1
City and Hackney Teaching Primary Care NHS Trust and City and East London
Mental Health NHS Trust, London, UK
2
Centre for Applied Social and Psychological Development, Salomons, Canterbury
Christ Church University, Kent, UK
3
Oxleas NHS Trust, Kent, UK
Objectives. The study investigated executive function in adults with Downs
syndrome.
Design. Participants with Downs syndrome (N 26) were compared with
non-Down syndrome learning disabled participants (N 26).
Method. The two groups performance on a range of tests of executive function
were compared. Groups were matched on age and a measure of vocabulary.
Results. The Downs syndrome group performed at a significantly lower level on a
number of tests of executive function.
Conclusion. It is suggested that impaired executive function in Downs syndrome is
due to abnormal development of the prefrontal cortex in Downs syndrome. Tests of
executive function may be sensitive to cognitive changes with the onset of dementia in
people with Downs syndrome. Longitudinal studies examining changes in executive
function in people with Downs syndrome are recommended.
In Downs syndrome (DS) there is a significantly increased risk for dementia of the
Alzheimer type (DAT) with the onset being at an earlier age. However, because of
pre-existing cognitive impairment, people with DS do not generally reach the level of
cognitive ability against which decline due to dementia can easily be measured. There is
a recognized need for methods for detecting the early cognitive changes with DAT onset
in DS (Das & Mishra, 1995; Devenny et al., 1996; Haxby, 1989; Holland & Oliver, 1995;
Schapiro, Haxby & Grady 1992). The pre-morbid assessment of people with DS order to
establish individual baselines of cognitive and adaptive functioning, against which
future decline due to possible DAT onset can be measured, is recommended (Burt &
Aylward, 1998; Burt & Aylward, 2000; NHS Executive and Social Service Inspectorate,
2001; Oliver, 1998; Turk, Dodd, & Christmas, 2000). Early detection enables timely care
planning, and consideration of interventions such as cholinergic therapy.
* Correspondence should be addressed to John Rowe, Learning Difficulties Service, St Leonards Primary Care Centre, Nuttall St,
London N1 5LZ, UK (e-mail: john.rowe@chpct.nhs.uk).
DOI:10.1348/014466505X29594

John Rowe et al.
Early diagnosis, via cognitive assessment, may be aided by the identification of

cognitive domains of relative weakness in DS. Holland, Hon, Huppert, and Stevens
(2000) and Holland, Hon, Huppert, Stevens, and Watson (1998) hypothesize that
functions served by neural substrates affected by early DS neuropathology, either
directly or through being the target of projections from affected areas, will be sensitive
to further insult due to lack of reserve capacity. That is, they will be unable to sustain
further decrement without the effects being manifest.
A survey by Holland et al. (2000) of prevalence rates of dementia in people with DS
in a UK health district found that a number of cases, particularly in younger age bands,
met diagnostic criteria for frontal-type dementia (Gregory & Hodges, 1996). They argue
that the presentation and course of dementia in DS reflects, or is affected by, pre-existing
abnormalities in brain development, specifically hypoplasia of the frontal cortex. In DS,
the frontal cortex, limbic system, and brain stem, generally show reduction in volume
(De la Monte & Hedley-Whyte, 1990; Jernigan, Bellugi, Sowell, Doherty, & Hesselink,
1993; Raz et al., 1995; Wisniewski, 1990) and decreased cellular density, particularly in
those layers from which callosal projections arise, and the region of the corpus callosum
serving frontal neural projections has been found to be underdeveloped (Coyle,
Oster-Granite, & Gearhart, 1986; Wang, Doherty, Hesselink, & Bellugi, 1992).
Holland et al. (2000) and Holland et al. (1998) point out that the frontal lobe
abnormalities found in DS may result in the early manifestation of dementia taking the
form of frontal-type dementia. They do not propose that younger people with DS and
dementia have frontal dementia but that early stage DAT can manifest in a similar form to
frontal dementia. They hypothesized that early behavioural changes at onset of DAT are
the consequence of early, unrecognized, cognitive decline in functions served by the
frontal lobes. Functions served by the prefrontal cortex and its projection areas include
executive function, summarized as goal formation, planning, and carrying out goaldirected plans effectively (Lezak, 1995, pp. 650 651).
Some authors have suggested the presence of executive dysfunction in DS
(e.g. Jernigan et al., 1993; Wisniewski, 1990) and some studies have found impairment
on some tests of executive function (e.g. Bellugi, Wang, & Jernigan, 1994; Burack,
Benedetto, & Frye, 1996; Frith & Frith, 1974; Gibson, 1991). It has, however, been noted
that executive function in DS has rarely been the subject of specific study (Pennington &
Bennetto, 1998; Crnic & Pennington, 2000).
This study examines aspects of executive function in people with DS and free from a
diagnosis of DAT in comparison to a control group matched on age and a measure of
vocabulary. It was hypothesized that the DS group would perform at a lower level on
tests of executive function.
Method
Design
A between-groups design (participants with DS versus learning disabled participants
without DS) was used.
Participants
A sample size of 26 was required to achieve a power level of 0.80 (Bilkman & Rog, 1998),
assuming an effect size of 0.80.

Cognitive executive function
The DS sample was made up of 18 males and 8 females (N 26), and the age range
was 23 years to 40, the upper age limit being set to exclude participants likely to have
preclinical DAT. The non-DS control sample was made up of 15 males and 11 females
(N 26), age range 19 years to 55, with learning disabilities of unknown aetiology.
All participants lived in community settings and were recruited from 4 day centres for
people with learning disabilities. All were educated in the UK. In both groups
the presence of significant sensory impairment, psychiatric or physical illness, or
challenging behaviour were grounds for exclusion, as were any pre-existing concerns
relating to deterioration in behaviour, skills or cognitive ability. DS participants were
required to have a medical diagnosis of DS confirmed by the appropriate learning
disabilities service. Participants with learning disabilities of known developmental
aetiology or with autistic spectrum disorder were excluded from the control group as
there is evidence for executive deficits in autism and possibly in some specific genetic
disorders (Pennington & Bennetto, 1998).
Measures
Executive function is not a unitary function, and there is the possibility that
developmental neuropathology may differentially affect aspects of executive function.
Tests were therefore selected to assess various aspects of executive function.
Pennington and Ozonoff (1996) list areas of executive function open to
neuropsychological assessment: set shifting; planning/problem solving; working
memory; inhibition/perseveration; and fluency. To this list attention was added as it
has been considered to overlap with executive functions and be served by the prefrontal
cortex (Manly & Robertson, 1997; Mirsky, Anthony, Duncan, Ahearn, & Kellam, 1991;
Posner and Peterson, 1990). A measure of vocabulary was included to match
participants. A measure of motor speed was included to control for the speed element in
the timed tests that had a motor speed component.
Tests were selected to cover Pennington and Ozonoffs areas. The criteria for test
selection were previous use with learning disabled participants, acceptable reliability
and validity, portability, minimal reliance on complex verbal instructions, brevity, and
availability in the UK.
Set shifting
Weigl Colour-Form Sort Test
The Weigl Colour-Form Sort Test (Goldstein & Scheerer, 1953) assesses ability to
categorize across two dimensions, involving ignoring a salient dimension (colour) to
categorize by a less salient dimension (form). Instructions for administration and scoring
were taken from Byrne, Bucks, and Cuerden (1998). Test material consisted of nine
tokens. These were three circles, three triangles, and three squares, each shape
coloured blue, red, or yellow on one side and white on the reverse. The participant was
asked to sort the tokens so that they went together and then asked to sort them in a
different way. The scoring system takes into account the established finding that sorting
by form is more difficult than sorting by colour for people with organic impairment, and
therefore awards a higher score to those who sort by form without prompting.
Responses were scored as follows.

John Rowe et al.

Response
Participant sorts by form or colour without prompting and shifts set when told.
Participant sorts to form and shifts to colour when given a cue.
Participant sorts to colour and shifts to form when given a cue.
Participant sorts to form only and does not shift to colour.
Participant sorts to colour only and does not shift to form.
Participant is unable to sort by form or colour.
Score
5
4
3
2
1
0
Planning/problem solving
Tower of London
The Tower of London (Shallice, 1982) assesses problem solving and spatial planning.
Instructions for administration and scoring were taken from Anderson, Anderson, and
Lajoies (1996) standardized administration and scoring procedures for a paediatric
population, chosen as it gives credit for longer solution times than Shallices (1982)
scoring system. Scores were allocated as follows.
Solution Time
Less than 5 seconds
6 10 seconds
11 20 seconds
21 40 seconds
41 60 seconds
Greater than 60 seconds
Score
9
8
7
6
5
4
Test material consisted of a board, 4 inches by 12 inches, with three pegs of

increasing height and three rings coloured red, blue, and green. The rings were wooden
discs two inches in diameter and half an inch thick with a half-inch diameter central
hole. A5 coloured photographs of the target configurations were provided.
The participant was asked to match the target configuration, shown on a coloured
photograph, from a standard start configuration by moving the rings in accordance with
set rules. The first problem was a training task, following which 12 problems of
increasing complexity were presented, giving a maximum score of 108.
Ravens Coloured Progressive Matrices

Ravens Coloured Progressive Matrices (RCPM, Raven, Court, and Raven, 1990) is a
widely used and standardized test of non-verbal reasoning ability based on figural test
stimuli. Although this test is primarily used as a measure of fluid intelligence, Carpenter,
Just, and Schell (1990), Duncan (1995), and Duncan, Burgess, and Emslie (1995) report
specific impairment on this task in people with frontal lobe lesions compared with
controls. Pennington and Ozonoff (1996) suggest this is because the frontal lobes are
important for fluid intelligence but less so for crystallized intelligence. It was therefore
decided to include this as a test of executive function, rather than a measure of
intellectual ability on which to match participants.
Instructions for administration and scoring were taken from Raven et al. (1990) with
a maximum score of 36.

Working memory
No suitable tests of the central executive component of working memory
were identified. Tasks tapping this function listed by Pennington and Ozonoff (1996)
were considered too complex for participants. Backwards digit span and spatial span,
which involve a processing element, were piloted but found to show floor effects with
many participants. Forward digit span and spatial span were used. These tap the verbal
and visual short term memory (STM) components of working memory in terms of
Baddeleys (1986) model of working memory but not the central executive processing
component.
Digit span
Digit span assesses verbal STM via immediate recall of a spoken digit string, although
Lezak (1995, p. 359) considers this more a test of attention than STM. Stimulus material
and instructions for administration and discontinuation criteria were taken from the Digit
Span subtest of the Wechsler memory scale third edition (WMS-III, The Psychological
Corporation, 1997). One point was given for each digit string correctly repeated, giving a
maximum score of 16.
Spatial span
Spatial span assesses visual STM. People with frontal lobe lesions have been found to
perform less well on this task than controls (De Renzi, Faglioni, and Previdi, 1977).
The participant observes the experimenter tapping blocks in sequence and then is asked
to reproduce that sequence. Stimulus material and instructions for administration and
discontinuation criteria were taken from the Spatial Span subtest of the WMS-III. One
point was given for each correctly reproduced sequence, giving a maximum of 16.
Inhibition/perseveration
Motor perseveration (finger tapping)
This motor perseveration test was used originally by Luria (1980). Instructions for
administration and scoring were taken from the Middlesex Elderly Assessment of
Mental State (MEAMS) manual (Golding, 1989). The participant was asked to tap the
table once if the examiner tapped twice, and tap the table twice if the examiner tapped
once. Ten trials were given. Practice trials were given until the participant gave both
correct responses. The score was the total number of correct trials (maximum 10).
Fluency
Verbal (semantic) fluency
Verbal Fluency, in which the participants produce as many words belonging to the same
category (e.g. same initial letter or same semantic category) as they can within a timed
period, is an established test of executive function. The semantic category of animal
names was used as this form of the test was considered easier for learning disabled
clients than generation of members of an abstract category, such as common initial
letter, and less dependent on literacy skills. Harrison, Buxton, Hussain, and Wise (2000)
report that the animal names version of this test correlates well with the initial letter
(FAS) version. The participant was asked to say as many animal names as they could

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John Rowe et al.
within one minute. The score was the total number of unique animal names generated
in 60 seconds.
Attention
Attention Sustained Leiter International Performance Scale revised
Attention Sustained is a visual search and cancellation task from the Leiter International
Performance Scale-Revised (LIPS-R) attention and memory battery (Roid & Miller, 1997).
Instructions for administration were taken from the LIPS-R manual (Roid and Miller,
1997), except that all booklets (A, B, and C) were administered. On each trial
participants were required to strike out with a pencil line target items within a given
time period (30 seconds or 60 seconds). The score was not the LIPS-R scaled score but
the total number of target items cancelled minus the total number of errors (non-target
items cancelled), giving a maximum of 355 points.
Verbal ability
British Picture Vocabulary Scale short form
Participants were not matched on the common tests of intellectual function, WAIS-III,
LIPS-R, and RCPM, as subtests from the WAIS-III and LIPS-R and the entire RCPM were
used as tests of executive function. To match groups on intellectual ability, a vocabulary
measure (British Picture Vocabulary Scale short form, BPVS, Dunn, Dunn, Whetton,
and Pintile, 1982) was used. Though not a measure of general intelligence, vocabulary is
an important contributor to general intelligence (Kaufman & Lictenberger, 1999; Lezak,
1995, p. 539; Satler & Ryan, 1998) and has been used to match learning disabled
participants in other studies (Jarrold & Baddeley, 1997; Jarrold, Baddeley, & Hewes,
1999; Jarrold, Baddeley, & Hewes, 2000) and can readily be assessed. Administration was
modified from the BPVS manual (Dunn et al., 1982). The score was the total number of
correct answers, discontinuation criteria were four incorrect responses out of six
successive items. Testing began at the first trial, (not at the BPVS manual age-indicated
trial) giving a maximum score of 32.
Motor speed
Crayton Oliver Card Sorting Test
As the Attention Sustained and Tower of London tests have a motor speed component,
this was separately assessed by a motor task that had minimal attentional or problem
solving demands. The Crayton Oliver Card-sorting task (Crayton, Oliver, Holland,
Bradbury, & Hall, 1998) was used. Test material consisted of twenty-six black and twentysix white tokens measuring 7 mm by 10 mm, made locally from 3 mm Perspex.
Instructions for administration were taken from Oliver and Crayton (1993). Twentyfive black and twenty-five white tokens were placed in a randomly sorted pile in front of
the participant. One extra black and one extra white card were placed to the participants
left and right respectively as models. The participant was asked to sort the tokens into
piles by colour, black to the left and white to the right. The score was the time taken from
touching the first card to completion. Note: no reliability or validity data were located for
this task.

11
Data management
Data were analysed via SPSS v. 9.0 for Windows. As the hypothesis predicts that the DS
group would be relatively impaired on tests of executive function, one-tailed levels of
significance were used except where stated. Normality of distribution was assessed via
the Kolmogor-Smirnov test. Homogeneity of variance was assessed via the F-test.
Comparison of means was via the unrelated t test if requirements for parametric tests
were met, otherwise the Mann-Whitney test was used. The level of significance was set
at p , :05 unless corrected for multiple comparisons. Effect size was calculated by
dividing the difference between group means by their mean standard deviation.
Results
Group characteristics
Characteristics of the DS and non-DS control groups are shown in Table 1. There was no
significant between-group difference for age or vocabulary (BPVS score). A significant
between-group difference for Crayton Oliver Card Sorting Test time was found with the
DS group showing a longer (slower) sorting time.
Executive function
Before correction for multiple comparisons significant between-group differences were
found for scores on: Verbal Fluency, Weigl Colour-Form Sort Test, Digit Span, Attention
Sustained, Motor Perseveration, Tower of London, and RCPM. The Spatial Span group
effect was not significant. Results are shown in Table 2. As multiple comparisons
(eight) were made on tests of executive function Bonferroni correction was carried out
and the level of significance set to p , :006. After Bonferroni correction a significant
group effect remained for the Weigl Colour-Form Sort Test, Attention Sustained, and
RCPM.
Differences in short term memory

Two-way ANOVA was carried out to assess the effects of modality of presentation
(verbal versus visual) in short term memory in the two groups by comparing scores on
the Digit Span with scores on the Spatial Span. The between-group interaction effect,
group by modality, was significant, (F1; 49 16:81, p :027). The DS group showed
lowered Digit Span performance than the non-DS group, whilst both groups showed
similar levels of performance on the Spatial Span.
Effects of motor speed on timed tests of executive function

There was a significant between-groups difference on motor speed scores as measured
by the Crayton Oliver Card Sorting Test. The executive function tests included two with
a timed motor speed component, the Attention Sustained and Tower of London tests.
ANCOVA was carried out on Attention Sustained scores, but not on Tower of London
scores as they did not reach significance after correction and the data were not suitable
for parametric analysis. After controlling for motor speed the group effect on Attention
Sustained remained significant; F1; 49 5:244, p :027.
14.50
102.96
33.25
BPVS score
COCST score
Age
3.80
35.22
5.30
SD
COCST Crayton Oliver Card Sorting Test.
Mean
Measure
7 22
42 186
23.8 40
Range (max min)
DS group N 26
15.64
78.58
33.45
Mean
4.12
27.10
9.65
SD
7 25
38 149
19 55
Range (max min)
Non-DS group N 26
0.93
2.73
0.27
t=Z
0.36ns
0.009
0.80ns
Sig. p value
0.29
0.78
0.03
Effect size d
12
Table 1. Group characteristics
John Rowe et al.
10.27
2.50
3.88
4.19
115
4.46
26.5
13.5
Verbal fluency
WCFST
Digit span
Spatial span
Attention sustained
Motor perseveration
Tower of London
RCPM
SD
3.80
1.30
2.05
1.74
57
3.92
11.47
13.46
WCFST Weigl Colour-Form Sort Test.
Mean
Measure
4 20
15
09
09
10 199
0 10
7 46
8 18
Range (max min)
DS group N 26
Table 2. Group comparisons on tests of executive function
12.69
4.04
5.40
4.08
178
7.64
33.5
16
Mean
4.17
0.99
2.45
1.98
46
2.96
13.76
4.83
SD
5 20
2 5
2 12
0 10
20 259
0 10
21 78
12 28
Range (max min)
Non-DS group N 26
2.19
4.78
2.38
0.21
5.12
5.74
2.48
4.40
t=Z
0.033
, .001
.021
.415ns
, .001
.020
.013
, .001
Sig. p Value
0.61
1.34
0.67
0.06
1.22
0.92
0.55
0.27
Effect size d

13

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John Rowe et al.
Discussion
The two groups did not differ significantly in age or verbal ability as measured by the
BPVS. The DS group were significantly slower on the Crayton Oliver Card Sorting Test.
On all tests of executive function, except for Spatial Span, the DS group scored at a
lower level than the control group. After correction for multiple comparisons significant
between-group differences were found for performance on the Weigl Colour-Form Sort
Test, Attention Sustained, and RCPM. Performance differences on the Tower of London
only narrowly missed significance.
Effect size gives a measure of the degree of overlap between experimental and
control groups, and hence the magnitude of deficit. Whilst statistically significant
deficits were found on measures of executive function in DS, these were not necessarily
clinically significant, for which an effect size of d . 3:00 would be required
(Zakanis, Leach, and Kaplan, 1999). Standard deviations and hence variance on Verbal
Fluency, Digit Span, Attention Sustained, and Motor Perseveration were high and would
argue for the importance of comparison against individual baseline scores as opposed to
comparison with group norms.
On measures of visual STM (Spatial Span) and verbal STM (Digit Span) a significant
between-group interaction for modality was found. The two groups showed similar
performance on the visual STM measure but the DS group showed significantly lower
performance on the verbal STM measure. The results support previous findings
(Baddeley, 1993; Bower & Hayes, 1994; Jarrold & Baddeley, 1997; Jarrold, et al., 1999;
Wang & Bellugi, 1994) of lowered verbal STM in DS with relatively unimpaired visual STM
and provide evidence for the hypothesis that verbal and visual STM are dissociated.
Tests of executive function could usefully be included in baseline neuropsychological
batteries, though a longitudinal study would need to be carried out to determine their
sensitivity and specificity. A number of tests used in this study were shown to be
potentially suitable for administration to a population with high-moderate to mild
learning disabilities and with a UK educational background. These are the: Weigl ColourForm Sort Test, Verbal (semantic) Fluency, WMS-III Digit Span and Spatial Span, Tower of
London, RCPM, MEAMS Motor Perseveration, LIPS-R Attention sustained. Some tests
(Digit Span, Spatial Span, and Motor Perseveration) showed floor effects with some
participants, rendering them unsuitable for use with more impaired clients.
The sensitivity and specificity of tests of executive function to onset of DAT in DS
would need to be determined by a longitudinal study.
People with DS without DAT are not generally reported to show social and adaptive
impairments relative to people with learning disabilities of other aetiologies.
Lezak (1982) points out that executive functions are at the heart of all socially useful,
personally enhancing, constructive, and creative activities. It might be expected that
executive function impairment would adversely affect these domains. This is an area for
future research.
This study found that impairment of executive function, relative to intellectual ability
(as estimated by lexical knowledge), is a feature of DS, possibly as a consequence of
impaired development of the prefrontal cortex. Deterioration of executive function as
determined by neuropsychological testing may be an indicator of DAT onset in DS.
Future research on this topic could usefully consider matching participants on a wider
range of measures including adaptive and social functioning.

15
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Received 20 November 2002; revised version received 4 August 2004

Cognitive Executive Function in Down's Syndrome

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