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Abstract
Background Although children with Downs syndrome (DS) are at lower risk for psychopathology
than others with intellectual disability, they do show
more problems than typically developing children.
However, age-related trends in these problems
remain unclear.
Methods The present authors examined agerelated changes in the maladaptive behaviours of
children and adolescents with DS aged between
and years (mean = . years). Most participants (n = ) were recruited from families residing in the greater Los Angeles area, California,
USA, while a minority (n = ) were patients from
a clinic specializing in the psychiatric management
of people with DS. The participants were divided
into four age groups: () years, () years,
() years and () years.
Results Externalizing behaviours were lower across
both the community and clinic samples, while internalizing behaviours were significantly higher in
older adolescents aged between and years.
Increases were found in withdrawal, seen in % of
Introduction
Relative to their typically developing peers, children
with intellectual disability (ID) are at increased
risk for behavioural and emotional problems, if not
frank psychiatric disorders (Rutter et al. ;
Gostason ). Both the type and rate of these
problems differ across people with different aetiologies of ID, particularly those with certain genetic
syndromes (for a review, see Dykens ). Compared to others with ID, for example, individuals
with PraderWilli syndrome (PWS) show high rates
of obsessive-compulsive symptoms (Dykens et al.
), and people with Williams syndrome show
elevated levels of anxiety, fears and inattention
(Einfeld et al. ; Dykens, in press).
Procedures
Child Behavior Checklist
The participants completed the CBCL during a
research session, at home or prior to their clinic
visit. The widely used CBCL asks parents to rate
problem behaviours on a three-point scale:
() not true; () somewhat or sometimes true;
and () very true or often true. The CBCL is
comprised of an internalizing domain (withdrawn,
anxious/depressed and somatic complaints subdomains), and externalizing domain (aggressive
behaviour and delinquent behaviour subdomains)
and three other subdomains (social problems,
thought problems and attention problems) that
sum for a total score. The CBCL has been successfully used in other studies of people with ID (e.g.
Dykens & Cohen ; Dykens et al. ). Unless
otherwise noted, all analyses were conducted with
raw scores.
Results
Maladaptive behaviour
Correlates
The three CBCL domains were examined across
race, and correlated with overall IQ, and maternal
and paternal age. None of these analyses proved
significant.
Child Behavior Checklist domains
Three analyses of variance (ANOVAs) (age
group by gender by clinic versus community) were
conducted with the internalizing, externalizing and
total domains. Significant age effects were found for
the internalizing, externalizing and total domains,
and Table summarizes the means, standard deviations, and F- and P-values for each domain.
NewmanKuels post hocs revealed that, relative to
young children aged years, internalizing
behaviours were significantly higher in - and
-year-olds, while externalizing behaviours
and total scores were highest in -year-olds.
A significant effect was also found for the source of
subjects, with the clinic sample scoring significantly
higher than the community sample in all three
domains (see Table ). There were no significant
interactions.
Subdomains
Follow-up ANOVAs (age group by gender
by clinic versus community) were conducted with
the subdomains which comprise the internalizing,
externalizing and total domain scores. Using an
adjusted P-value of ., four domains showed significant main effects for age: aggressive behaviour,
delinquent behaviour, withdrawn and social problems. Table summarizes the means, F- and Pvalues for these three subdomains. NewmanKuels
post hocs revealed that relative to younger children,
-year-olds had significantly higher social,
aggressive and delinquent behaviour sores, while
-year-olds and -year-olds had significantly higher scores in the withdrawn subdomain
(see Table ). The withdrawn subdomain was qualified by a significant age by gender interaction, such
that older females aged years scored significantly higher than males in this same age group
Table 1 Mean Child Behavior Checklist (CBCL) raw scores for children with Downs syndrome across age groups, and F- and P-values
for age effects
Age group
(1)
46 years
(2)
79 years
(3)
1013 years
(4)
1419 years
CBCL
Mean
SD
Mean
SD
Mean
SD
Mean
SD
F-value (age)
Post-hocs
Domains
Internalizing
Externalizing
Total
3.93
8.46
31.96
4.43
5.76
18.08
5.21
10.66
36.45
3.95
7.47
18.61
6.94
12.37
42.94
6.18
7.97
22.12
7.16
8.78
35.81
7.49
8.45
25.32
4.21***
3.54**
3.70*
3, 4 > 1
3>1
3>1
Internalizing subdomains
Withdrawn
1.77
Somatic
1.22
Anxious
0.93
1.81
2.52
1.51
2.48
1.04
1.67
2.33
1.29
1.91
3.43
1.66
1.84
2.76
2.73
2.92
4.11
1.32
1.73
3.94
2.01
2.76
5.75***
1.50
2.36
4 > 2; 3, 4 > 1
Externalizing subdomains
Aggression
7.21
Delinquent
1.24
4.94
1.27
8.93
1.73
6.08
1.65
10.02
2.35
6.55
1.97
6.89
1.89
6.12
2.62
3.77**
6.03***
3>1
3>1
Other subdomains
Social
3.57
Thought
1.26
Attention
5.27
2.18
1.55
3.01
4.31
1.67
6.11
2.15
1.65
3.51
5.66
2.23
6.16
3.18
2.15
3.28
4.62
2.05
5.32
2.68
2.25
4.74
6.45***
2.89
1.41
3 > 1, 2
* P < ..
** P < ..
*** P < ..
Community
(n = 180)
Clinic
(n = 31)
CBCL
Mean
SD
Mean
SD
F-value
Domains
Internalizing
Externalizing
Total
4.98
8.90
32.77
4.69
6.09
17.12
9.19
17.13
58.84
8.19
10.37
26.62
10.17**
10.75**
24.86***
1.30
2.53
1.15
4.26
1.48
5.14
1.51
7.38
1.82
2.62
1.70
2.47
1.68
3.06
1.46
5.01
2.74
4.32
2.13
5.74
3.32
9.26
3.26
13.87
3.85
3.18
3.96
3.17
2.28
4.40
3.04
8.03
10.17**
6.08**
2.02
6.53**
26.61***
42.14***
13.57***
15.03***
Subdomains
Anxious/depressed
Withdrawn
Somatic
Social problems
Thought problems
Attention problems
Delinquent behaviour
Aggressive behaviour
** P < ..
*** P < ..
Discussion
Age-related patterns of maladaptive behaviour were
found in both the community and clinical samples
of people with DS. As expected, clinic cases had
Table 3 Percentages of specific Child Behavior Checklist (CBCL) behaviours showing significant age affects in children with Downs
syndrome
Age group
Behaviour
(1) 46 years
(n = 61)
(2) 79 years
(n = 62)
c2
Prefers to be alone
Secretive
Underactive
Argues a lot
Demands attention
Overweight
Swears
Gets teased a lot
Cannot concentrate
28
0
15
51
61
06
06
16
71
45
05
37
68
60
21
10
26
79
66
16
49
76
63
47
23
31
76
63
35
59
50
27
59
30
46
38
21.71***
36.01***
34.57***
21.15**
18.61**
46.21***
15.79**
17.44**
28.25***
** P < ..
*** P < ..
dren with other ID, such effects declined dramatically over the adolescent years. Although further
work is needed, parents may be reacting to their
offsprings subtle changes in sociability.
On the other hand, lower rates of problems
may be associated with the social orientation and
friendly, outgoing personalities which characterize
many with DS (Gibbs & Thorne ; Hornby
; Kasari et al. ). The perception of certain
personality features may also be associated with the
baby face cranio-facial appearance of many people
with the syndrome. Fidler & Hodapp () found
that, relative to others with ID, photographs of
children with DS met baby-faced criteria, and
were perceived by others as having personality traits
associated with baby-faced individuals in general,
including being more immature, warm, kind, naive,
honest, cuddly and compliant. If children and adolescents with DS become less outgoing, or more
mature in appearance as they age, it is possible that
parent-raters are less able to overlook problem
behaviours.
It is unknown to what extent even subtle
increases in internalizing symptoms over the adolescent years might set the stage for the later onset
of depressive disorders, or be the early harbingers
of mood or behavioural changes associated with
dementia. In this vein, the finding that withdrawal
was higher in adolescent females versus males is
particularly intriguing since women with DS are
approximately . times as likely to develop
Alzheimers disease than men (Lai et al. ).
Among adults with DS, personality and behavioural changes, as opposed to cognitive changes,
are typically the first hints of later-onset dementia
(Alyward et al. ; Holland et al. ). This
pattern supports the hypothesis that certain functions, primarily those associated with the frontal
lobe, are affected relatively early in the progression
of dementia in DS (Holland et al. ). Although
speculative, slight shifts in sociability and withdrawal in adolescence may similarly reflect early
frontal lobe involvement in disorders which manifest much later in life.
Therefore, instead of showing obvious psychopathology, it may be that some adolescents
undergo a period of more subtle shifts in personality, sociability and withdrawal. This hypothesis may
explain why longitudinal studies to date do not find
dramatic increases in psychiatric disorders in adolescents with DS; the outcome measures are simply
too coarse. For example, no significant changes
in psychopathology were found on the clinical
domains of the DBC in children with DS followed over an -year period (Einfeld et al. in press).
Similarly, McCarthy & Boyd () retrospectively
examined psychiatric diagnoses in adults with
DS, and found few significant relationships between
childhood and adult psychiatric disorders. The
outcome measures in these studies may pick up significant psychiatric problems, but not be sensitive
to more subtle1 shifts in personality or sociability.
Being underactive and overweight also increased
significantly across age groups. Many adolescents
and adults with DS are at increased risk of becoming obese (Prasher ), and this risk is associated
with sedentary life styles, limited exercise, poor
diet, lower resting metabolic rates, hypotonia and
hypothyroidism (for a review, see Roizen ). It is
unknown to what extent obesity is associated with
externalizing or internalizing problems in children
or adults with DS, including increased withdrawal.
Most children with DS do not appear to experience significant behavioural problems, with % of
the community-based sample showing non-significant CBCL scores. Thus, clinically elevated scores
were found in % of the community sample, and
while this rate is quite consistent with previous
studies of children with DS (e.g. Myers & Pueschel
; Dykens & Kasari ), it is much lower
than children with ID in general (Einfeld & Tonge
; Einfeld et al. in press). Therefore, a possible
shift toward more internalizing symptoms is likely
to be rather slight or gradual for the vast majority
of children with DS, and unlikely to impede everyday adaptive functioning.
Several limitations of the present study need to
be considered, including the cross-sectional design.
Longitudinal studies are sorely needed which pinpoint exactly when shifts in aggressive and withdrawn behaviours are likely to occur, and how these
relate, if at all, to the later onset of more serious
psychiatric disease. Another concern relates to
sampling. This was not an epidemiological sample
and it is possible that parents interested in a study
1
It seems as if these may indeed be clinically significant (e.g.
harbingers of later difficulty).
Acknowledgements
We thank the families and staff of the Los Angeles
Down Syndrome Association for their enthusiastic
involvement in our research, and Lori Salinas RN
and Henry Messenheimer for their superb work in
the UCLA Down Syndrome Clinic. We are grateful
as well to Robert M. Hodapp and Beth A. Rosner
for their helpful comments on an earlier draft of
this manuscript. This research was supported by
NICHD Grant #HD.
References
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Meyers B. A. & Pueschel S. M. () Psychiatric disorders in persons with Down syndrome. Journal of
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Down syndrome adults. Journal of Intellectual Disability
Research , .
Reiss S. () Prevalence of dual diagnosis in community based day programs in the Chicago metropolitan
area. American Journal on Mental Retardation ,
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Accepted February