Journal of Intellectual Disability Research

484
pp

Maladaptive behaviour in children and adolescents with

Downs syndrome
E. M. Dykens,1 B. Shah,1 J. Sagun,1 T. Beck1 & B. H. King2
1 University of California Los Angeles, Neuropsychiatric Institute, Los Angeles, California, USA
2 Dartmouth Medical School, Hanover, New Hampshire, USA

Abstract community-based adolescents, and % of clinic

adolescents.
Background Although children with Downs syn-
Conclusions Older adolescents with DS may show
drome (DS) are at lower risk for psychopathology
decreased externalizing symptoms and subtle
than others with intellectual disability, they do show
increases in withdrawal. Possible relationships are
more problems than typically developing children.
discussed between these shifts and increased risks
However, age-related trends in these problems
of later-onset depression and Alzheimers disease in
remain unclear.
adults with DS.
Methods The present authors examined age-
related changes in the maladaptive behaviours of Keywords Downs syndrome, children and adoles-
children and adolescents with DS aged between cents, maladaptive behaviour
and years (mean = . years). Most partici-
pants (n = ) were recruited from families resid-
ing in the greater Los Angeles area, California, Introduction
USA, while a minority (n = ) were patients from
Relative to their typically developing peers, children
a clinic specializing in the psychiatric management
with intellectual disability (ID) are at increased
of people with DS. The participants were divided
risk for behavioural and emotional problems, if not
into four age groups: () years, () years,
frank psychiatric disorders (Rutter et al. ;
() years and () years.
Gostason ). Both the type and rate of these
Results Externalizing behaviours were lower across
problems differ across people with different aetiolo-
both the community and clinic samples, while inter-
gies of ID, particularly those with certain genetic
nalizing behaviours were significantly higher in
syndromes (for a review, see Dykens ). Com-
older adolescents aged between and years.
pared to others with ID, for example, individuals
Increases were found in withdrawal, seen in % of
with PraderWilli syndrome (PWS) show high rates
of obsessive-compulsive symptoms (Dykens et al.
Correspondence: Elisabeth Dykens PhD, UCLA Neuropsychiatric
), and people with Williams syndrome show
Institute, Westwood Plaza, Los Angeles, CA , USA elevated levels of anxiety, fears and inattention
(e-mail: edykens@mednet.ucla.edu). (Einfeld et al. ; Dykens, in press).

Blackwell Science Ltd

 Journal of Intellectual Disability Research
485
E. M. Dykens et al. Maladaptive behaviour
Psychopathology in individuals with Downs
syndrome (DS) is distinctive from these and other
groups in several ways. Perhaps best known of these
differences is the elevated risk for Alzheimer-type
dementia (Zigman et al. ). Most people with
DS aged years and older show neuropathological
signs of dementia, and clinical symptoms of demen-
tia are seen in approximately % of individuals
aged years and older (Zigman et al. ).
Adults with DS also show increased rates of depres-
sion (Warren et al. ). Thus, while depressive
disorders can be identified in from .% to .%
of adults with DS (Meyers & Pueschel ;
Collacott et al. ), only .% to .% of adults
with mixed aetiologies of ID are similarly affected
(Lund ; La Malfa et al. ).
Interestingly, in comparison to most other groups
with ID, children with DS are at lower risk for sig-
nificant psychopathology. Children with DS score
significantly lower than their counterparts with
ID on standardized rating scales of maladaptive
behaviour (Dykens & Kasari ; Stores et al.
; Einfeld et al. in press), including the Aberrant
Behavior Checklist (ABC; Aman et al. ), the
Developmental Behaviour Checklist (DBC; Einfeld
& Tonge ) and the Child Behavior Checklist
(CBCL; Achenbach ). Furthermore, while
% of children with ID of mixed aetiologies
show significant psychopathology (Rutter et al.
; Reiss ; Einfeld & Tonge ), only
% of children with DS appear to do so
(Meyers & Pueschel ; Dykens & Kasari ).
Thus, although rates of psychopathology are rela-
tively low in children with DS, these children are
far from problem-free. Children with DS do show
more behavioural problems than their typically
developing siblings or typically developing children
from the general population (Gath & Gumley
; Pueschel et al. ; Cuskelly & Dadds ; year-old patients from a larger group of patients
Coe et al. ). In particular, they have more
externalizing types of problems than normal
controls, including stubbornness, oppositionality
and inattention.
In all this work, researchers have yet to address
how behavioural problems in children with DS
change over the course of development. Only a few
studies have preliminarily assessed such age effects.
Stores et al. () found a significant decline in
the hyperactivity domain of the ABC, with DS chil-
Blackwell Science Ltd, Journal of Intellectual Disability Research ,
dren aged years scoring significantly higher
than -year-old adolescents. Dykens & Kasari
() found a significant, positive correlation
between age and the internalizing domain of the
CBCL in -year-old youngsters with DS. This
relationship was not found in the two comparison
groups with other causes of ID. Such age-related
patterns are important to clarify since they may
shed some light on the later onset of depression or
dementia seen in many adults with DS. To this aim,
the present authors examined age-related changes
in the maladaptive behaviour in a large cohort of
children and adolescents with DS aged years.
Subjects and methods
Participants
A total of children and adolescents with DS
( males and females), who ranged in age
from to years, were enrolled in the present
study. The mean age ( SD) of these participants
was . . years. Behavioural surveys were
completed in % of cases by mothers, who had a
mean age ( SD) of . . years, and % by
fathers, who had a mean age ( SD) . .
years. All subjects with DS had trisomy ; four
individuals with either mosaicism or translocations
were not included in data analyses.
Most of the families (%, n = ) were
recruited through the Los Angeles Down Syndrome
Association via announcements in newsletters (on a
study on development and behaviour), including
announcements geared to the -year-old age
group. These participants attended school and
lived at home with their families in the greater
Los Angeles area, California, USA. The remaining
participants (%, n = ) were a subset of -
attending a university-based specialized psychiatric
clinic for people with DS. Out of the partici-
pants, % were Caucasian, % Hispanic, %
African-American, % Asian and % other or
mixed.
Fifty-four per cent of the participants
completed their questionnaires during a research
appointment at the university, while % completed
them at home and returned them in a stamped,
self-addressed envelope. No differences in maladap-
 Journal of Intellectual Disability Research
486
E. M. Dykens et al. Maladaptive behaviour
tive behaviour scores were found between those
participants who attended a research session
versus those who completed their questionnaires
at home.
Participants attending a research session also
received one of two standardized intelligence
tests, depending on time availability. The mean
IQ ( SD) of the participants receiving the
Kaufman Brief Intelligence Test (K-BIT; Kaufman
& Kaufman ) was . ., and the mean
IQ ( SD) of the participants receiving the
StanfordBinet Intelligence Test (Thorndike et al.
) was . .. group by gender by clinic versus community) were
The children were divided into four age groups.
Initially, the present authors had planned to divide
the participants into just two broad developmental
groups: children versus adolescents. However, given
the predominance of young children in the study,
they elected instead to divide the sample into
more finely tuned age groups. Sixty-one children
aged years comprised group (mean SD =
. . years), and children aged years -year-olds, while externalizing behaviours
comprised group (mean SD = . .
years). Group consisted of children aged
years (mean SD = . . years), subjects, with the clinic sample scoring significantly
and group consisted of adolescents aged
years (mean SD = . . years). domains (see Table ). There were no significant
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 sub-
domains), 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 success-
fully 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.
Blackwell Science Ltd, Journal of Intellectual Disability Research ,
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
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 de-
viations, 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
and total scores were highest in -year-olds.
A significant effect was also found for the source of
higher than the community sample in all three
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 sig-
nificant main effects for age: aggressive behaviour,
delinquent behaviour, withdrawn and social prob-
lems. Table summarizes the means, F- and P-
values 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 signifi-
cantly higher scores in the withdrawn subdomain
(see Table ). The withdrawn subdomain was quali-
fied by a significant age by gender interaction, such
that older females aged years scored signifi-
cantly higher than males in this same age group
 Journal of Intellectual Disability Research
487
E. M. Dykens et al. Maladaptive behaviour

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) (2) (3) (4)

46 years 79 years 1013 years 1419 years

CBCL Mean SD Mean SD Mean SD Mean SD F-value (age) Post-hocs

Domains
Internalizing 3.93 4.43 5.21 3.95 6.94 6.18 7.16 7.49 4.21*** 3, 4 > 1
Externalizing 8.46 5.76 10.66 7.47 12.37 7.97 8.78 8.45 3.54** 3>1
Total 31.96 18.08 36.45 18.61 42.94 22.12 35.81 25.32 3.70* 3>1
Internalizing subdomains
Withdrawn 1.77 1.81 2.48 2.33 3.43 2.76 4.11 3.94 5.75*** 4 > 2; 3, 4 > 1
Somatic 1.22 2.52 1.04 1.29 1.66 2.73 1.32 2.01 1.50
Anxious 0.93 1.51 1.67 1.91 1.84 2.92 1.73 2.76 2.36
Externalizing subdomains
Aggression 7.21 4.94 8.93 6.08 10.02 6.55 6.89 6.12 3.77** 3>1
Delinquent 1.24 1.27 1.73 1.65 2.35 1.97 1.89 2.62 6.03*** 3>1
Other subdomains
Social 3.57 2.18 4.31 2.15 5.66 3.18 4.62 2.68 6.45*** 3 > 1, 2
Thought 1.26 1.55 1.67 1.65 2.23 2.15 2.05 2.25 2.89
Attention 5.27 3.01 6.11 3.51 6.16 3.28 5.32 4.74 1.41

* P < ..
** P < ..
*** P < ..

Table 2 Mean Child Behavior Checklist

Community Clinic (CBCL) raw scores, and F- and P-values,
(n = 180) (n = 31) in clinic versus community samples of
children with Downs syndrome
CBCL Mean SD Mean SD F-value

Domains
Internalizing 4.98 4.69 9.19 8.19 10.17**
Externalizing 8.90 6.09 17.13 10.37 10.75**
Total 32.77 17.12 58.84 26.62 24.86***
Subdomains
Anxious/depressed 1.30 1.82 2.74 3.85 10.17**
Withdrawn 2.53 2.62 4.32 3.18 6.08**
Somatic 1.15 1.70 2.13 3.96 2.02
Social problems 4.26 2.47 5.74 3.17 6.53**
Thought problems 1.48 1.68 3.32 2.28 26.61***
Attention problems 5.14 3.06 9.26 4.40 42.14***
Delinquent behaviour 1.51 1.46 3.26 3.04 13.57***
Aggressive behaviour 7.38 5.01 13.87 8.03 15.03***

** P < ..
*** P < ..

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E. M. Dykens et al. Maladaptive behaviour

(means = . versus ., respectively). The problems (% of sample); stubbornness (%);

remaining interactions were non-significant. disobedience (%); fears (%); and impulsivity
Significant main effects were found for the source (%).
of participants, with clinic cases scoring signifi-
cantly higher than the community sample in seven Clinical significance
out of the eight subdomains. Table presents
The CBCL total domain raw scores were converted
means, standard deviations, and F- and P-values
to T-scores, and the percentages of subjects were
for each subdomain across the two sources of
identified who showed clinically significant levels of
participants.
problems. Clinically significant T-scores are those
Because the standard deviations for several of the
above , as established by Achenbach () using
domains and subdomains in Tables and were
large epidemiological samples of children with
larger than the means, analyses were re-conducted
and without identified problems. For participants
using the non-parametric KruskalWallis test, with
derived from the community, % had clinically
age and source of subjects examined separately. The
elevated scores, % had scores in the borderline
findings remained the same for all analyses.
range (T-scores from to ) and % had non-
significant levels. For the clinic sample, % had
Specific behaviours
clinically elevated scores, % scored in the border-
Using an adjusted value of P < ., chi-square line range and % had non-significant scores. A
analyses were conducted across age groups on the chi-square (clinical level versus clinic versus
frequencies of specific behaviours which comprised community participants) was significant
the four subdomains which showed significant age (c2(2) = ., P < .).
effects (i.e. withdrawn, aggressive and delinquent
behaviour, and social problems). Nine behaviours
showed significant differences across age groups;
Discussion
these are listed in Table , along with the percent-
ages of children in each age group exhibiting these Age-related patterns of maladaptive behaviour were
behaviours. High-frequency behaviours which did found in both the community and clinical samples
not show significant age effects included: speech 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

(1) 46 years (2) 79 years (3) 1013 years (4) 1419 years
Behaviour (n = 61) (n = 62) (n = 51) (n = 37) c2

Prefers to be alone 28 45 66 63 21.71***

Secretive 0 05 16 35 36.01***
Underactive 15 37 49 59 34.57***
Argues a lot 51 68 76 50 21.15**
Demands attention 61 60 63 27 18.61**
Overweight 06 21 47 59 46.21***
Swears 06 10 23 30 15.79**
Gets teased a lot 16 26 31 46 17.44**
Cannot concentrate 71 79 76 38 28.25***

** P < ..
*** P < ..

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 Journal of Intellectual Disability Research
489
E. M. Dykens et al. Maladaptive behaviour
significantly higher ratings of psychopathology, but
both groups showed a similar pattern of decreased
externalizing symptoms in older adolescence as well
as increased internalizing symptoms during these
same years. The present findings have diagnostic
and practical implications, and paint a more com-
plete picture of the DS behavioural phenotype.
Specifically, the aggressive behaviour domain
was highest in the -year-old period, but then
declined significantly and was lowest among older
adolescents. Similarly, relative to younger children,
delinquent behaviour was highest among the -
year-old group. These patterns seemed particularly
true of more low-level aggressive behaviours, such
as being argumentative, demanding attention or
swearing. Rates of other behaviours remained fairly
constant across age groups, such as stubbornness
(seen in % of the total sample) and disobedience
(%). Stubbornness is often cast as being highly
characteristic of DS. However, it is unclear what
parents mean by this global descriptor, or how high
rates of stubbornness in DS differs from similarly
high rates of stubbornness seen in others, including
those with PWS (%), and children and adoles-
cents with mixed aetiologies of ID (%) (Dykens
& Kasari ).
However, the low frequency of more extreme
aggressive behaviours in individuals with DS is of
note, with just % of the sample engaging in fights
and % in physically aggressive acts. Low rates of
extreme aggression have also been found in other
DS samples, including adults (Collacott et al. ;
Cooper & Prasher ). In a similar vein, Carr
() followed children with DS from the ages
of to years, and found that mothers described
their offspring as easier to manage as they got
older.
At the same time as certain externalizing behav-
iours declined, internalizing behaviours increased,
primarily the withdrawn domain. Indeed, as many
as % of adolescents were described as preferring
to be alone than with others, and approximately
one-third were cast as secretive and not wanting to
talk. Such shifts in personality may be associated
with an adjustment in parents as well, primarily in
how rewarded they feel by their offspring. Examin-
ing children and adolescents with DS, Hodapp
et al. () found that while parents of children
with DS felt more rewarded than parents of chil-
Blackwell Science Ltd, Journal of Intellectual Disability Research ,
dren with other ID, such effects declined dramati-
cally 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 ado-
lescents 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 adoles-
cent 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 behav-
ioural 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 func-
tions, 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 with-
drawal in adolescence may similarly reflect early
frontal lobe involvement in disorders which mani-
fest much later in life.
Therefore, instead of showing obvious psy-
chopathology, it may be that some adolescents
undergo a period of more subtle shifts in personal-
ity, sociability and withdrawal. This hypothesis may
explain why longitudinal studies to date do not find
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490
E. M. Dykens et al. Maladaptive behaviour
dramatic increases in psychiatric disorders in ado-
lescents 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 fol-
lowed 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 sig-
nificant 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 becom-
ing 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 experi-
ence significant behavioural problems, with % of
the community-based sample showing non-signifi-
cant 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 every-
day adaptive functioning.
Several limitations of the present study need to
be considered, including the cross-sectional design.
Longitudinal studies are sorely needed which pin-
point exactly when shifts in aggressive and with-
drawn 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).
Blackwell Science Ltd, Journal of Intellectual Disability Research ,
of behaviour are those whose children are more
likely to have behavioural disturbance. Thus, the
reported rates of problems may be elevated. A
related concern is that the present study used the
CBCL, which does not measure certain behaviours
which are distinctive to some people with ID, such
as stereotypies or self-injury. In this case, the fre-
quency of problems may be underestimated. Addi-
tional limitations were that the authors did, not
screen their community sample for psychiatric diag-
noses nor did they use a standardized psychiatric
interview in their smaller clinical sample.
Even with these limitations, the present study
identifies age-related changes in maladaptive behav-
iour in a large cohort of young people with DS, and
sets the stage for future longitudinal research. Ulti-
mately, such work may identify when children with
DS are at highest risk for withdrawal or other per-
sonality shifts, leading to more rigorous screening
and interventions at these time points. Although
relatively few children with DS have severe mal-
adaptive behaviour, interventions which minimize
withdrawal and nurture sociability may help amelio-
rate these problems, as well as improve the quality
of life for young people with DS in general.
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.
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