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Journal of Intellectual Disability Research


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

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).

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Journal of Intellectual Disability Research
E. M. Dykens et al. Maladaptive behaviour

Psychopathology in individuals with Downs dren aged years scoring significantly higher
syndrome (DS) is distinctive from these and other than -year-old adolescents. Dykens & Kasari
groups in several ways. Perhaps best known of these () found a significant, positive correlation
differences is the elevated risk for Alzheimer-type between age and the internalizing domain of the
dementia (Zigman et al. ). Most people with CBCL in -year-old youngsters with DS. This
DS aged years and older show neuropathological relationship was not found in the two comparison
signs of dementia, and clinical symptoms of demen- groups with other causes of ID. Such age-related
tia are seen in approximately % of individuals patterns are important to clarify since they may
aged years and older (Zigman et al. ). shed some light on the later onset of depression or
Adults with DS also show increased rates of depres- dementia seen in many adults with DS. To this aim,
sion (Warren et al. ). Thus, while depressive the present authors examined age-related changes
disorders can be identified in from .% to .% in the maladaptive behaviour in a large cohort of
of adults with DS (Meyers & Pueschel ; children and adolescents with DS aged years.
Collacott et al. ), only .% to .% of adults
with mixed aetiologies of ID are similarly affected
(Lund ; La Malfa et al. ). Subjects and methods
Interestingly, in comparison to most other groups
with ID, children with DS are at lower risk for sig-
nificant psychopathology. Children with DS score A total of children and adolescents with DS
significantly lower than their counterparts with ( males and females), who ranged in age
ID on standardized rating scales of maladaptive from to years, were enrolled in the present
behaviour (Dykens & Kasari ; Stores et al. study. The mean age ( SD) of these participants
; Einfeld et al. in press), including the Aberrant was . . years. Behavioural surveys were
Behavior Checklist (ABC; Aman et al. ), the completed in % of cases by mothers, who had a
Developmental Behaviour Checklist (DBC; Einfeld mean age ( SD) of . . years, and % by
& Tonge ) and the Child Behavior Checklist fathers, who had a mean age ( SD) . .
(CBCL; Achenbach ). Furthermore, while years. All subjects with DS had trisomy ; four
% of children with ID of mixed aetiologies individuals with either mosaicism or translocations
show significant psychopathology (Rutter et al. were not included in data analyses.
; Reiss ; Einfeld & Tonge ), only Most of the families (%, n = ) were
% of children with DS appear to do so recruited through the Los Angeles Down Syndrome
(Meyers & Pueschel ; Dykens & Kasari ). Association via announcements in newsletters (on a
Thus, although rates of psychopathology are rela- study on development and behaviour), including
tively low in children with DS, these children are announcements geared to the -year-old age
far from problem-free. Children with DS do show group. These participants attended school and
more behavioural problems than their typically lived at home with their families in the greater
developing siblings or typically developing children Los Angeles area, California, USA. The remaining
from the general population (Gath & Gumley participants (%, n = ) were a subset of -
; Pueschel et al. ; Cuskelly & Dadds ; year-old patients from a larger group of patients
Coe et al. ). In particular, they have more attending a university-based specialized psychiatric
externalizing types of problems than normal clinic for people with DS. Out of the partici-
controls, including stubbornness, oppositionality pants, % were Caucasian, % Hispanic, %
and inattention. African-American, % Asian and % other or
In all this work, researchers have yet to address mixed.
how behavioural problems in children with DS Fifty-four per cent of the participants
change over the course of development. Only a few completed their questionnaires during a research
studies have preliminarily assessed such age effects. appointment at the university, while % completed
Stores et al. () found a significant decline in them at home and returned them in a stamped,
the hyperactivity domain of the ABC, with DS chil- self-addressed envelope. No differences in maladap-
Blackwell Science Ltd, Journal of Intellectual Disability Research ,
Journal of Intellectual Disability Research
E. M. Dykens et al. Maladaptive behaviour

tive behaviour scores were found between those Results

participants who attended a research session
Maladaptive behaviour
versus those who completed their questionnaires
at home. Correlates
Participants attending a research session also
The three CBCL domains were examined across
received one of two standardized intelligence
race, and correlated with overall IQ, and maternal
tests, depending on time availability. The mean
and paternal age. None of these analyses proved
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 Child Behavior Checklist domains
StanfordBinet Intelligence Test (Thorndike et al. Three analyses of variance (ANOVAs) (age
) was . .. group by gender by clinic versus community) were
The children were divided into four age groups. conducted with the internalizing, externalizing and
Initially, the present authors had planned to divide total domains. Significant age effects were found for
the participants into just two broad developmental the internalizing, externalizing and total domains,
groups: children versus adolescents. However, given and Table summarizes the means, standard de-
the predominance of young children in the study, viations, and F- and P-values for each domain.
they elected instead to divide the sample into NewmanKuels post hocs revealed that, relative to
more finely tuned age groups. Sixty-one children young children aged years, internalizing
aged years comprised group (mean SD = behaviours were significantly higher in - and
. . years), and children aged years -year-olds, while externalizing behaviours
comprised group (mean SD = . . and total scores were highest in -year-olds.
years). Group consisted of children aged A significant effect was also found for the source of
years (mean SD = . . years), subjects, with the clinic sample scoring significantly
and group consisted of adolescents aged higher than the community sample in all three
years (mean SD = . . years). domains (see Table ). There were no significant

Child Behavior Checklist
Follow-up ANOVAs (age group by gender
The participants completed the CBCL during a by clinic versus community) were conducted with
research session, at home or prior to their clinic the subdomains which comprise the internalizing,
visit. The widely used CBCL asks parents to rate externalizing and total domain scores. Using an
problem behaviours on a three-point scale: adjusted P-value of ., four domains showed sig-
() not true; () somewhat or sometimes true; nificant main effects for age: aggressive behaviour,
and () very true or often true. The CBCL is delinquent behaviour, withdrawn and social prob-
comprised of an internalizing domain (withdrawn, lems. Table summarizes the means, F- and P-
anxious/depressed and somatic complaints sub- values for these three subdomains. NewmanKuels
domains), and externalizing domain (aggressive post hocs revealed that relative to younger children,
behaviour and delinquent behaviour subdomains) -year-olds had significantly higher social,
and three other subdomains (social problems, aggressive and delinquent behaviour sores, while
thought problems and attention problems) that -year-olds and -year-olds had signifi-
sum for a total score. The CBCL has been success- cantly higher scores in the withdrawn subdomain
fully used in other studies of people with ID (e.g. (see Table ). The withdrawn subdomain was quali-
Dykens & Cohen ; Dykens et al. ). Unless fied by a significant age by gender interaction, such
otherwise noted, all analyses were conducted with that older females aged years scored signifi-
raw scores. cantly higher than males in this same age group
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Journal of Intellectual Disability Research
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

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

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***
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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Journal of Intellectual Disability Research
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
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;
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

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
E. M. Dykens et al. Maladaptive behaviour

significantly higher ratings of psychopathology, but dren with other ID, such effects declined dramati-
both groups showed a similar pattern of decreased cally over the adolescent years. Although further
externalizing symptoms in older adolescence as well work is needed, parents may be reacting to their
as increased internalizing symptoms during these offsprings subtle changes in sociability.
same years. The present findings have diagnostic On the other hand, lower rates of problems
and practical implications, and paint a more com- may be associated with the social orientation and
plete picture of the DS behavioural phenotype. friendly, outgoing personalities which characterize
Specifically, the aggressive behaviour domain many with DS (Gibbs & Thorne ; Hornby
was highest in the -year-old period, but then ; Kasari et al. ). The perception of certain
declined significantly and was lowest among older personality features may also be associated with the
adolescents. Similarly, relative to younger children, baby face cranio-facial appearance of many people
delinquent behaviour was highest among the - with the syndrome. Fidler & Hodapp () found
year-old group. These patterns seemed particularly that, relative to others with ID, photographs of
true of more low-level aggressive behaviours, such children with DS met baby-faced criteria, and
as being argumentative, demanding attention or were perceived by others as having personality traits
swearing. Rates of other behaviours remained fairly associated with baby-faced individuals in general,
constant across age groups, such as stubbornness including being more immature, warm, kind, naive,
(seen in % of the total sample) and disobedience honest, cuddly and compliant. If children and ado-
(%). Stubbornness is often cast as being highly lescents with DS become less outgoing, or more
characteristic of DS. However, it is unclear what mature in appearance as they age, it is possible that
parents mean by this global descriptor, or how high parent-raters are less able to overlook problem
rates of stubbornness in DS differs from similarly behaviours.
high rates of stubbornness seen in others, including It is unknown to what extent even subtle
those with PWS (%), and children and adoles- increases in internalizing symptoms over the adoles-
cents with mixed aetiologies of ID (%) (Dykens cent years might set the stage for the later onset
& Kasari ). of depressive disorders, or be the early harbingers
However, the low frequency of more extreme of mood or behavioural changes associated with
aggressive behaviours in individuals with DS is of dementia. In this vein, the finding that withdrawal
note, with just % of the sample engaging in fights was higher in adolescent females versus males is
and % in physically aggressive acts. Low rates of particularly intriguing since women with DS are
extreme aggression have also been found in other approximately . times as likely to develop
DS samples, including adults (Collacott et al. ; Alzheimers disease than men (Lai et al. ).
Cooper & Prasher ). In a similar vein, Carr Among adults with DS, personality and behav-
() followed children with DS from the ages ioural changes, as opposed to cognitive changes,
of to years, and found that mothers described are typically the first hints of later-onset dementia
their offspring as easier to manage as they got (Alyward et al. ; Holland et al. ). This
older. pattern supports the hypothesis that certain func-
At the same time as certain externalizing behav- tions, primarily those associated with the frontal
iours declined, internalizing behaviours increased, lobe, are affected relatively early in the progression
primarily the withdrawn domain. Indeed, as many of dementia in DS (Holland et al. ). Although
as % of adolescents were described as preferring speculative, slight shifts in sociability and with-
to be alone than with others, and approximately drawal in adolescence may similarly reflect early
one-third were cast as secretive and not wanting to frontal lobe involvement in disorders which mani-
talk. Such shifts in personality may be associated fest much later in life.
with an adjustment in parents as well, primarily in Therefore, instead of showing obvious psy-
how rewarded they feel by their offspring. Examin- chopathology, it may be that some adolescents
ing children and adolescents with DS, Hodapp undergo a period of more subtle shifts in personal-
et al. () found that while parents of children ity, sociability and withdrawal. This hypothesis may
with DS felt more rewarded than parents of chil- explain why longitudinal studies to date do not find
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Journal of Intellectual Disability Research
E. M. Dykens et al. Maladaptive behaviour

dramatic increases in psychiatric disorders in ado- of behaviour are those whose children are more
lescents with DS; the outcome measures are simply likely to have behavioural disturbance. Thus, the
too coarse. For example, no significant changes reported rates of problems may be elevated. A
in psychopathology were found on the clinical related concern is that the present study used the
domains of the DBC in children with DS fol- CBCL, which does not measure certain behaviours
lowed over an -year period (Einfeld et al. in press). which are distinctive to some people with ID, such
Similarly, McCarthy & Boyd () retrospectively as stereotypies or self-injury. In this case, the fre-
examined psychiatric diagnoses in adults with quency of problems may be underestimated. Addi-
DS, and found few significant relationships between tional limitations were that the authors did, not
childhood and adult psychiatric disorders. The screen their community sample for psychiatric diag-
outcome measures in these studies may pick up sig- noses nor did they use a standardized psychiatric
nificant psychiatric problems, but not be sensitive interview in their smaller clinical sample.
to more subtle1 shifts in personality or sociability. Even with these limitations, the present study
Being underactive and overweight also increased identifies age-related changes in maladaptive behav-
significantly across age groups. Many adolescents iour in a large cohort of young people with DS, and
and adults with DS are at increased risk of becom- sets the stage for future longitudinal research. Ulti-
ing obese (Prasher ), and this risk is associated mately, such work may identify when children with
with sedentary life styles, limited exercise, poor DS are at highest risk for withdrawal or other per-
diet, lower resting metabolic rates, hypotonia and sonality shifts, leading to more rigorous screening
hypothyroidism (for a review, see Roizen ). It is and interventions at these time points. Although
unknown to what extent obesity is associated with relatively few children with DS have severe mal-
externalizing or internalizing problems in children adaptive behaviour, interventions which minimize
or adults with DS, including increased withdrawal. withdrawal and nurture sociability may help amelio-
Most children with DS do not appear to experi- rate these problems, as well as improve the quality
ence significant behavioural problems, with % of of life for young people with DS in general.
the community-based sample showing non-signifi-
cant CBCL scores. Thus, clinically elevated scores
were found in % of the community sample, and Acknowledgements
while this rate is quite consistent with previous
We thank the families and staff of the Los Angeles
studies of children with DS (e.g. Myers & Pueschel
Down Syndrome Association for their enthusiastic
; Dykens & Kasari ), it is much lower
involvement in our research, and Lori Salinas RN
than children with ID in general (Einfeld & Tonge
and Henry Messenheimer for their superb work in
; Einfeld et al. in press). Therefore, a possible
the UCLA Down Syndrome Clinic. We are grateful
shift toward more internalizing symptoms is likely
as well to Robert M. Hodapp and Beth A. Rosner
to be rather slight or gradual for the vast majority
for their helpful comments on an earlier draft of
of children with DS, and unlikely to impede every-
this manuscript. This research was supported by
day adaptive functioning.
NICHD Grant #HD.
Several limitations of the present study need to
be considered, including the cross-sectional design.
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