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Down Syndrome and Attention-Deficit/Hyperactivity Disorder (ADHD)

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of Child Neurology

Down Syndrome and Attention-Deficit/Hyperactivity Disorder (ADHD)


Sivan Ekstein, Benjamin Glick, Michal Weill, Barrie Kay and Itai Berger
J Child Neurol 2011 26: 1290 originally published online 31 May 2011
DOI: 10.1177/0883073811405201

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Original Article
Journal of Child Neurology
26(10) 1290-1295
Down Syndrome and Attention-Deficit/ ª The Author(s) 2011
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Hyperactivity Disorder (ADHD) DOI: 10.1177/0883073811405201
http://jcn.sagepub.com

Sivan Ekstein, MD1,2, Benjamin Glick, MD2, Michal Weill, MD2,


Barrie Kay, MD1, and Itai Berger, MD1

Abstract
Clinicians might minimize the prevalence of behavioral disorders among mentally retarded people. Decreased attention,
hyperactivity, and impulsivity are frequently reported in children with Down syndrome, yet the exact prevalence of
attention-deficit/hyperactivity disorder (ADHD) has not been clearly estimated in this population. The objective of this
study was to estimate the prevalence of ADHD in children with Down syndrome and to emphasize the possible
relationship between ADHD symptoms and the level of mental retardation and common medical comorbidity. In this
study, the prevalence of ADHD among Down syndrome children was very high, reaching 43.9%. No significant correlation
was found between ADHD symptoms and the level of mental retardation, but significant correlation was found with
ophthalmologic problems. We conclude that children with Down syndrome are at increased risk for ADHD. When
evaluating children with Down syndrome for attention deficits, psychiatric comorbidity as well as medical problems should
be carefully taken into consideration.

Keywords
Down syndrome, attention-deficit/hyperactivity disorder, comorbidity, ADHD

Received January 25, 2011. Received revised March 6, 2011. Accepted for publication March 6, 2011.

Down syndrome is considered the leading major genetic cause is estimated with a wide range, from 15.55% to 36%, and even
of mental retardation.1 Children with Down syndrome present up to 57% to 64%.12-14 A possible explanation for the high rate
different degrees of cognitive disabilities including comorbid is that individuals with developmental disabilities have lower
behavioral traits and/or psychiatric disorders.2,3 coping abilities because of limited cognitive, communication,
In the past, the idea that persons with mental retardation could and adaptive abilities.15 Environmental as well as genetic fac-
have coexisting mental illnesses was not accepted.4 It was tors may also contribute.3
widely believed that all behavioral disturbances in mentally Decreased attention span, difficulty in concentration, hyper-
retarded people were linked to the cognitive impairment and did activity, and impulsive behavior are frequently reported in
not require further diagnostic consideration or evaluation.4 In children with Down syndrome, yet the exact prevalence of pro-
recent years, there has been wider recognition that the mentally fessionally diagnosed attention-deficit/hyperactivity disorder
retarded can also have a coexisting psychiatric disorder.4,5 (ADHD) was not clearly estimated in this population.4,16,17
Moreover, researchers indicate that the prevalence of psy- The objective of this study is to estimate the prevalence
chiatric disorders in people with developmental disabilities, of ADHD in children with Down syndrome and to emphasize
including mental retardation, is much higher than in the general the possible relationship between ADHD symptoms and
population.6,7 Some authors even use the term diagnostic the level of mental retardation and/or common medical
overshadowing referring to clinicians who minimize the comorbidities.
significance of emotional disorders in persons with mental
retardation and may be displaying overshadowing judgmental
bias.8,9 The diagnosis of comorbid disorder is important 1
Pediatric Neurology Unit, Hadassah-Hebrew University Medical Center,
because such individuals have higher mortality rates and are Jerusalem, Israel
more disabled than those with psychiatric illness alone.10 The
2
Child Development Center, Meuchedet Health Services, Jerusalem, Israel
most significant neurologic/psychiatric/behavioral disorders
Corresponding Author:
in mentally retarded persons are disorders of attention, mood, Itai Berger, MD, Pediatric Neurology Unit, Hadassah-Hebrew University
personality, and thought processes.11 The prevalence of comor- Medical Center, Mount Scopus, PO Box 24035, Jerusalem, 91240 Israel
bid psychiatric disorders in individuals with mental retardation Email: itberg@hadassah.org.il

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Ekstein et al 1291

Methods Table 1. Distribution of Intellectual Disability Among Study


Population
Participants included all children diagnosed with Down syndrome
who were evaluated by the Down Syndrome Multidisciplinary Clinic MR Level Frequency % (of total)
of Meuchedet Health Services in Jerusalem, from January 2008 to
January 2010. This clinic provides timely neurodevelopmental as well Mild MR 7 17.1
as medical evaluation and support for children with Down syndrome Mild to moderate MR 14 34.1
and their families in Jerusalem. Moderate MR 12 29.3
The diagnosis of ADHD was made by a pediatric neurologist Moderate to severe MR 3 7.3
based on the Diagnostic and Statistical Manual of Mental Disorders Total 36 (out of 41 children) 87.8
(DSM-IV-TR edition).18 Abbreviation: MR, mental retardation.
The diagnostic procedure included an interview with the children
and parents, fulfillment of questionnaires based on DSM-IV-TR by
parents and teachers, and neurological examination. Full results were available in 36 (87.8%) children. Table 1
Inclusion criteria included the complete diagnostic procedure as describes the distribution of intellectual disability level among
described above, available details regarding cognitive abilities, and the study population. Table 2 describes the distribution of
medical conditions, which might have an effect on clinical symptoms ADHD in correlation with intellectual disability level. No
of ADHD as recorded from medical files. significant correlation was found between ADHD symptoms
Exclusion criteria included the following: age < 5 years, lack of and the level of mental retardation (P ¼ .542).
full available information regarding fulfillment of appropriate diag-
nostic procedure and/or inclusion criteria, and another known mental
ADHD and sensory deficits. Ophthalmologic disorders (mainly
disorder (schizophrenia, pervasive developmental disorder [PDD],
refractive errors) were documented in 21 (51.2%) children. A
psychotic, mood, anxiety, personality, or dissociative disorders).
Statistical analysis performed using SPSS 17 program (Statistical significant correlation was found between visual difficulties
Product and Services Solutions, 17 version, SPSS Inc, Chicago, Illinois). and the presence of ADHD symptoms (P ¼ .028). Table 3
Continuous data were reported as mean + standard deviation (SD). describes the prevalence of ADHD regarding ophthalmologic
Ordinal data were reported as numbers and percentages. Comparisons disorders, before necessary correction. Some degree of hearing
between groups were tested for statistical significance using Fisher loss was documented in 21 (51.2%) patients. No significant
exact 2-tailed tests or 2-tailed t tests for independent samples as correlation was found between hearing loss and ADHD
appropriate. symptoms (P ¼ .756).
Ethical approval was received from the Internal Review Board of
Meuchedet Health Services. ADHD and sleep disturbances. Problems in sleep such as snor-
ing, sleep apnea, and frequent arousals were described in 10
Results (24.4%) of the patients. No significant correlation was found
between ADHD and sleep disturbances (P ¼ .724).
From January 2008 to January 2010, 84 children diagnosed
with Down syndrome were evaluated in the clinic. A total of
43 children were excluded from the study: 23 were younger
than 5 years of age and 20 had incomplete medical records,
Discussion
missing parts of necessary information as described in exclu- The diagnosis of ADHD in mentally retarded individuals is
sion criteria. In all, 41 children were eligible for the study. challenging. According to the DSM-IV-TR edition, the symp-
Among this group, 18 children (43.9%) fulfilled the criteria toms of ADHD should be at a degree that is maladaptive and
of ADHD diagnosis according to the DSM-IV-TR criteria, inconsistent with developmental level, and do not occur
9 of them (22%) were treated by methylphenidate. exclusively during the course of other mental disorders such
It is important to clarify that the data provided describe the as mood, anxiety, dissociative, or personality disorders.18
condition of the children at the first clinic visit. The diagnosis Although intellectual disability was not mentioned as exclu-
of medical conditions was leading to appropriate treatment and sion criteria in the DSM-IV-TR, in the past it was common
follow-up as needed. to assume that attention and hyperactivity symptoms resulted
simply from the developmental delay, and an additional diag-
Age and gender. The study population contained 24 males nosis was not often proposed.20 Only in recent years, data
(58.5%) and 17 females (41.5%). No significant association concerning comorbidity in individuals with mental retarda-
was found between gender and ADHD (P ¼ .524) in this group. tion have been published, and several studies estimated the
Mean age at evaluation was 8 years (range, 5-16 years). Mean specific prevalence of ADHD in this population.4,15,17,21,22
age between the groups (ADHD and non-ADHD) was not In their review,22,(p285) Deutsch et al mention that ‘‘the long-
significantly different. standing controversy about the definition of attention deficits in
intellectual developmental disabilities stems from a widely
ADHD and intellectual disability. Children were evaluated for held opinion that such deficits are inherent to intellectual dis-
cognitive dysfunction by the Wechsler Preschool and Primary abilities.’’ At the same time, these and other researchers
Scale of Intelligence-Revised.19 declared that attention deficits and the symptomatology

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1292 Journal of Child Neurology 26(10)

Table 2. Distribution of ADHD Symptoms According to Intellectual Disability Level

Non-ADHD ADHD Total

Intellectual disability level Mild MR Number 2 5 7


% 28.6 71.4 100.0
Mild to moderate MR Number 8 6 14
% 57.1 42.9 100.0
Moderate MR Number 7 5 12
% 58.3 41.7 100.0
Moderate to severe MR Number 1 2 3
% 33.3 66.7 100.0
Total Number 18 18 36
% 50.0 50.0 100.0
Abbreviations: ADHD, attention-deficit/hyperactivity disorder; MR, mental retardation.

associated with ADHD appear with greater frequency in people Table 3. Prevalence of ADHD Regarding Ophthalmologic
with intellectual disabilities.4,16,17,22,23 However, they also Abnormalities
warn that ‘‘the epidemiology of ADHD in mental retardation
was in a very early stage of investigation and that good esti- Non-ADHD ADHD Total
mates of the base rates of ADHD symptoms in mental retarda- Ophthalmologic No Number 15 5 20
tion were not yet currently available.’’22,(p286),23 abnormality % 75.0 25.0 100.0
The prevalence of ADHD diagnosed according to the Yes Number 8 13 21
DSM-IV-TR was 13.5% in a sample of 184 individuals with % 38.1 61.9 100.0
mental retardation.11 In a sample of 474 individuals 7 to 20 Total Total 23 18 41
years old with intellectual disability, 14.8% met the criteria for % 56.1 43.9 100.0
ADHD according to the DSM-IV.13 Using the ICD-10, 15.7% Abbreviation: ADHD, attention-deficit/hyperactivity disorder.
of children were diagnosed with hyperkinesias from a sample
of 178 children aged 8 to 13 years.24 In a recent study, Fidan ADHD was diagnosed in 30%.32 The high prevalence of
et al explained why it is especially important to address this ADHD found in our study requires discussion regarding pos-
issue in a group with known etiology, because the rate of sible factors.
ADHD among 167 children and adolescents with mental retar-
dation of unknown etiology was 41.7%.21
Down syndrome is of special interest among intellectual Cognitive performance. According to the DSM-IV-TR
disability populations, with birth prevalence of 8.32 to criteria, the diagnosis of ADHD in a person with intellectual
14.47 per 10 000 live births.25,26 This is therefore the most disability is possible if the symptoms of inattention, impulsiv-
common chromosomal disorder in the population.27 This fact ity, and hyperactivity are disproportionate with the mental age
is even more significant in Jerusalem where the prevalence is and result in significant academic or social impairment.18
17.75 per 10 000 live births, higher than in other medical cen- Theoretically, these subjective instructions may lead to bias
ters (personal communication with Prof M. S. Schimmel, and false results, because the assessment can be easily biased
Shaare-Zedek Medical Center, Jerusalem, Israel). This high and depends on cultural and personal views.4 The degree of
prevalence is attributed to the uniqueness of our population, mental impairment in Down syndrome is variable, ranging
mainly ultra-Orthodox Jewish mothers, who have a high rate from mild (IQ: 50-70) to moderate (IQ: 35-50), and occasion-
of delivery over the age of 35 years and high parity and who ally to severe (IQ: 20-35).33
abstain from prenatal screening and abortions because of This fact might have biased previous studies. This possible
strong theological and cultural beliefs.28,29 In our population, bias can be isolated or overcome when the cognitive perfor-
the prevalence of ADHD among children with Down syn- mance of the study group is known and the participants are
drome aged 5 to 16 years was 43.9% according to the DSM- matched with the appropriate (same mental age) control
IV-TR criteria. This number is higher than previously group.22 In such a comparison, attention deficits and the symp-
described. Hyperkinetic disorder among children with devel- tomatology associated with ADHD indeed appear with greater
opmental delay according to ICD-9 was reported in 9% of 193 frequency in intellectual developmental disability samples than
children with Down syndrome aged 6 to 17 years.30 Hyperki- in matched samples drawn from the general population.22 In
nesia was found in 16% of 178 children according to the this study, we used the same available criteria (DSM criteria)
ICD-10 criteria.24 Määttä et al reported a 33% prevalence of used in other studies, but this study includes a relatively iso-
ADHD symptoms in 129 adults with Down syndrome through lated group of children because all of them share a common
the evaluation of health records.31 Green et al evaluated a cultural background and the cognitive performance was deter-
group of 13 Down syndrome children aged 2 to 4 years; mined in most of them (87.8%). Moreover, in this study, no

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Ekstein et al 1293

significant correlation was found between ADHD symptoms developmental disorder, and psychotic, mood, anxiety,
and the level of mental retardation. Current results support the personality, or dissociative disorders. It is important to men-
results of a previous study.21,32 tion that even after using the psychopathology rating scale
in 60 children with Down syndrome aged 4 to 21 years, atten-
Medical problems. Among Down syndrome children there is tion deficit behaviors were still a common diagnosis.10 This
an increased risk of hearing loss (75%), eye disease (60%), data further supports the results of the current study.
including cataracts (15%), and severe refractive errors (50%), As stated by others,21,22 many aspects of ADHD in mental
obstructive sleep apnea (50%-75%), and thyroid disease retardation such as epidemiology, proper diagnostic methods,
(15%).28 All of these disorders can impair attention and beha- appropriate recommendations for clinicians and parents, and
vior and mimic ADHD. teachers’ assessment of tolerance and expectations are all in
Sensory deficits are especially important, while discussing a very early stage of investigation and good estimates where
ADHD, because within the lateral prefrontal cortex there are conclusions are not yet currently available.
preferential targets of projections from visual, auditory, and
somatosensory cortices associated with directing attention
and monitoring responses for specific tasks.34 Abnormalities
Conclusions
in the sensorimotor region of the brain are associated with This study demonstrates that children with Down syndrome are
ADHD.34,35 It is suggested that reactivity to sensory input at an increased risk for ADHD with a prevalence as high as
reflects a link between perception and action, and that 43.9%. It also demonstrates the special difficulties in making
abnormalities in this reactivity might reflect impairment in the diagnosis among children with mental disabilities. We con-
perception-to-action mechanisms.34,36,37 ADHD individuals clude that when evaluating children with Down syndrome for
are impaired in tasks requiring sensorimotor function, attention, attention deficits, psychiatric comorbidity as well as medical
and inhibition. Dock Stader et al proved abnormal sensory mod- problems should be carefully taken into consideration. More
ulation in adult ADHD suggesting a possible underlying deficit research is needed to determine the preferred assessment
in the perception-to-action system.38 methods and behavioral and medical treatment approach
In this study, significant correlation was found only between among these patients.
ADHD diagnosis and ophthalmologic problems (mainly
refractive errors). Visual deficits, such as abnormal or Acknowledgments
immature saccade eye movements, are suggested as part of the This study was performed at the Child Development Center,
dysfunction in response inhibition among ADHD children.39 Meuchedet Health Services, Jerusalem, Israel.
Oculomotor findings suggest that deficits in prefrontal func-
tions, in particular response inhibition, contribute to behavioral Author Contributions
abnormalities observed in ADHD.40 It is possible that visual SE and IB participated in study design, data analysis, and wrote the
deficits may be especially debilitating to individuals with first draft of the manuscript. BG and MW participated in study design,
intellectual impairments, resulting in more restricted adaptive data collection, and analysis. BK participated in data analysis. All
behavior and problem solving repertoires.41 Another authors participated in manuscript preparation and approved its final
explanation is that visual attention is the main difficulty among version.
children with Down syndrome, thus ophthalmic impairment
has a more significant effect. This hypothesis is supported by Declaration of Conflicting Interests
the finding that individuals with Down syndrome had deficits The authors declared no potential conflicts of interest with respect to
in visual spatial working memory in comparison with controls, the authorship and/or publication of this article.
while asked to process more than 1 item at a time.42 The
limitations of the study include a relatively small number of Financial Disclosure/Funding
children, and the fact that comorbid psychopathology was not The authors received no financial support for the research and/or
separately evaluated. The rate of psychiatric comorbidity in authorship of this article.
Down syndrome might be relatively high including mood dis-
order, phobia, and pervasive developmental disorder.43 Ethical Approval
The presence of another psychopathology might complicate The Internal Review Board of Meuchedet Health Services approved
the diagnosis of ADHD in Down syndrome because there the study.
might be some overlap in symptoms. To overcome this prob-
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