Child Neuropsychology

A Journal on Normal and Abnormal Development in Childhood and

Adolescence

ISSN: (Print) (Online) Journal homepage: https://www.tandfonline.com/loi/ncny20

The role of distinct executive functions

on adaptive behavior in children and adolescents
with Down syndrome

Elizabeth A. Will, Emily K. Schworer & Anna J. Esbensen

To cite this article: Elizabeth A. Will, Emily K. Schworer & Anna J. Esbensen (2021)

The role of distinct executive functions on adaptive behavior in children

and adolescents with Down syndrome, Child Neuropsychology, 27:8, 1054-1072, DOI:
10.1080/09297049.2021.1917531

To link to this article: https://doi.org/10.1080/09297049.2021.1917531

Published online: 02 May 2021.

Submit your article to this journal

Article views: 442

View related articles

View Crossmark data

Citing articles: 2 View citing articles

Full Terms & Conditions of access and use can be found at

https://www.tandfonline.com/action/journalInformation?journalCode=ncny20
CHILD NEUROPSYCHOLOGY
2021, VOL. 27, NO. 8, 1054–1072
https://doi.org/10.1080/09297049.2021.1917531

The role of distinct executive functions on adaptive

behavior in children and adolescents with Down syndrome
Elizabeth A. Willa, Emily K. Schworerb and Anna J. Esbensenb,c
a
Department of Psychology, University of South Carolina, Columbia, SC, USA; bDivision of Developmental
and Behavioral Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA; cDepartment
of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA

ABSTRACT ARTICLE HISTORY

Difficulties in executive function are a relatively well-characterized Received 25 March 2021
feature of the neuropsychological profile in Down syndrome (DS), Accepted 12 April 2021
yet the impact of these challenges on aspects of daily functioning KEYWORDS
remain poorly understood. We examined the role of specific execu­ Executive function; adaptive
tive functions on domains of adaptive behavior in children and behavior; Down syndrome;
adolescents with DS. Participants included 68 children and adoles­ neurodevelopmental
cents with DS between 6–17 years old (mean chronological disorders; working memory
age = 12.56 years; SD = 3.22) and their caregivers. Parent reported
executive function skills were measured using the BRIEF-2 and
adaptive behavior was measured using the Vineland Adaptive
Behavior Scales-III. Results identified working memory as
a significant predictor of Communication, Daily Living, and
Socialization skills, and Shifting significantly predicted Daily Living
and Socialization. Findings demonstrate the relation between
executive functions and adaptive behavior and highlight the effects
of working memory on aspects of daily functioning for individuals
with DS.

Down syndrome (DS), the leading known genetic cause of intellectual disability (ID), is
characterized by phenotypic areas of strength and challenge relative to developmental
level. One particular area of research interest within the DS behavioral phenotype is
executive function (EF), a collection of interrelated cognitive processes that facilitate
daily functioning (Diamond, 2013; Silverman, 2007). The past 10 years have seen
a substantial amount of progress in identifying areas of particular difficulty and ability
commensurate with developmental status across EFs at various points of development in
DS (Daunhauer, Fidler, Hahn, et al., 2014; Lanfranchi et al., 2010; Lee et al., 2015; Loveall
et al., 2017; Tungate & Conners, 2021). Further, we have begun to ascertain the functional
impact of specific areas of difficulty in EF on various outcomes in DS including language
acquisition (Baddeley & Jarrold, 2007), academic achievement (Will et al., 2017), and
school-participation (Daunhauer, Fidler, & Will, 2014). Although we have a relatively
thorough understanding of how EFs contribute to the execution of daily skills and
adaptive engagement in typical development (Blair & Razza, 2007; Diamond, 2013),

CONTACT Elizabeth A. Will willea@mailbox.sc.edu Department of Psychology, University of South Carolina, 1512
Pendleton St., Columbia, SC 29208.
© 2021 Informa UK Limited, trading as Taylor & Francis Group
 CHILD NEUROPSYCHOLOGY 1055

less is known regarding the impact of specific EFs on aspects of daily adaptive functioning
in DS. As such, we aim to identify the relation between specific EFs and domains of
adaptive functioning to gain a more thorough understanding of the possible functional
consequences of impaired EFs in DS and potentially cultivate knowledge for intervention
targets.

Executive function in Down syndrome

Executive function is a set of dissociable yet interrelated cognitive processes that facilitate
processing of information necessary to engage in goal-directed behavior (Diamond,
2013; Miyake et al., 2000; Tungate & Conners, 2021). The cognitive components most
commonly considered as EFs include inhibition, shifting (i.e., cognitive flexibility or
attention shifting), and working memory (Diamond, 2013; Miyake et al., 2000; Tungate
& Conners, 2021). These components work together to facilitate higher-order cognitive
skills such as goal-directed behavior, yet can be measured distinctively from one another
(Diamond, 2013; Miyake & Friedman, 2012; Miyake et al., 2000). Broadly speaking, EFs
are considered to be an area of distinct challenge for individuals with DS (Lanfranchi
et al., 2010; Lee et al., 2011) relative to individuals with other genetic disorders associated
with ID (Carney et al., 2013; Costanzo et al., 2013; Rowe et al., 2006) and individuals with
typical development (Daunhauer, Fidler, Hahn, et al., 2014; Lanfranchi et al., 2010).
When considering components of EF individually, however, specific EFs appear to be
more impaired relative to other areas (Daunhauer, Fidler, Hahn, et al., 2014; Lee et al.,
2015, 2011; Tungate & Conners, 2021).

Inhibition
One primary EF is inhibition, which refers to the ability to suppress a prepotent response.
Evidence on inhibition skills in DS is somewhat mixed. In children and adolescents with
DS, inhibition emerges as an area of challenge compared to matched groups with ID
without DS and typical controls of the same developmental level (Borella et al., 2013;
Costanzo et al., 2013 2013). Yet, other findings show that inhibition is commensurate
with developmental level for adolescents with DS (Carney et al., 2013; Lanfranchi et al.,
2010), both in the context of ID and TD matched comparisons. In a recent meta-analysis,
inhibition was confirmed as an area of difficulty in DS compared to matched typically
developing controls but was also confirmed to be less impaired relative to the other
primary EFs, such as shifting and working memory (Borella et al., 2013; Carney et al.,
2013; Costanzo et al., 2013; Lanfranchi et al., 2010; Tungate & Conners, 2021).

Shifting
Shifting, a second primary component of EF, is defined as the ability to efficiently and
effectively transition from one task or set of rules to another (Zelazo, 2006). Evidence on
shifting abilities in DS is also somewhat mixed (Tungate & Conners, 2021). Shifting is
characterized as an impaired EF in children and adolescents with DS relative to matched
typically developing controls (Costanzo et al., 2013; Lanfranchi et al., 2010). This pattern
has been replicated in adolescents and adults with DS compared to other individuals with
ID of the same developmental or receptive language level (Phillips et al., 2014; Rowe et al.,
2006). The results from a recent meta-analysis (Tungate & Conners, 2021) on EF in DS
1056 E. A. WILL ET AL.

provides support for shifting as an impaired EF in DS; however, only studies employing
laboratory-based measures of EF abilities were included. Contrary to these findings, the
results from studies focused on parent-reported EFs suggest that shifting may be an area
of strength relative to developmental status (Daunhauer, Fidler, Hahn, et al., 2014) as well
as other EF domains (Lee et al., 2011; Loveall et al., 2017).

Working memory
Working memory, a third primary EF, is a cognitive system used to store, process, and
update information (Baddeley & Jarrold, 2007; Baddeley & Hitch, 1974). This system can
be dissociated into two separate modalities – visual working memory and verbal working
memory (Baddeley & Jarrold, 2007; Jarrold et al., 1999; Lanfranchi et al., 2012;
Numminen et al., 2001), both of which contribute to higher-order goal-directed beha­
vior. Working memory is well researched in DS and has been characterized as an area of
relative difficulty, regardless of whether measured by a lab-based task, parent report, or
teacher report (Conners et al., 2011; Daunhauer, Fidler, Hahn, et al., 2014, 2017; Lee
et al., 2015). Although individuals with DS perform slightly better on visual working
memory tasks relative to auditory working memory tasks (Lanfranchi et al., 2012, 2004;
Næss et al., 2011), performance in both of these areas lags behind typically developing
peers with the same developmental level (Borella et al., 2013; Carney et al., 2013;
Costanzo et al., 2013; Lanfranchi et al., 2012). In addition, individuals with DS show
greater impairments in working memory abilities relative to other individuals with ID
(Carney et al., 2013; Costanzo et al., 2013; Rowe et al., 2006) suggesting a specific DS EF
profile, although this is somewhat dependent on whether the task modality is verbal or
visual (Kittler et al., 2006; Rowe et al., 2006).

Implications for executive impairments

As a collection of interrelated cognitive skills, these primary EFs (i.e., inhibition, shifting,
and working memory) coalesce to facilitate goal-directed behavior, or planning
(Lanfranchi et al., 2010; Miyake et al., 2000). Planning is considered a marked area of
difficulty in EF for individuals with DS. It emerges with a more significant degree of
impairment relative to other domains on parent reported EFs (Daunhauer, Fidler, Hahn,
et al., 2014; Lee et al., 2015, 2011; Loveall et al., 2017), and individuals with DS show
challenges with planning on lab-based tasks compared to matched comparison groups
(Fidler et al., 2014; Rowe et al., 2006).
Impairments in any specific primary domain of EF, or in higher-order EF (e.g.,
planning) as the pinnacle performance of the primary domains, have important implica­
tions for a variety of outcomes. For instance, the ability to successfully inhibit impulsive
behaviors has a significant impact on optimal functioning and participation in the school
setting for children with DS (Daunhauer, Fidler, & Will, 2014). Difficulty with shifting
can lead to missed learning opportunities or hyperfocus on irrelevant stimuli, which may
translate to off task and oppositional behavior around transitions (Will et al., 2016).
Further, working memory is critical in the development and use of language and reading
development (Abbeduto et al., 2007; Jarrold et al., 2009; Næss et al., 2011; Yang et al.,
2014), academic achievement (Will et al., 2017), and employment (Tomaszewski et al.,
2018) for individuals with DS. Considering that planning is a higher-order EF reliant on
 CHILD NEUROPSYCHOLOGY 1057

these specific subdomains, difficulty in this area is likely to manifest across a variety of the
domains affected by difficulties in inhibition, shifting, and working memory. Despite
some understanding of the functional consequences related to executive impairment in
DS, adaptive behavior is another domain likely affected by EFs. However, the specific
association between these phenotypic features in DS remains unclear.

Adaptive behavior
Adaptive behavior is an area of functioning critical for engaging in conceptual, practical,
and social aspects of daily life (Ditterline & Oakland, 2009; Schalock et al., 2010; Tassé
et al., 2016). Measurement of adaptive behavior typically encompasses multiple domains
of functioning, including communication, daily living skills, and socialization.
A proficient adaptive repertoire is associated with higher academic achievement
(Bornstein et al., 2013), reduced levels of maladaptive behaviors (Racz et al., 2017), and
increased independence in adulthood (Woolf et al., 2010). In general, adaptive skills are
an area of marked difficulty in DS (see Daunhauer, 2011 for review), and children with
DS show significantly reduced adaptive skills relative to chronological (Will et al., 2018)
and developmental expectations (Fidler et al., 2006). However, similar to EFs, children
with DS express a profile consisting of proficiencies as well as challenges across the
various domains of adaptive functioning (Daunhauer, 2011; Fidler et al., 2006; Van Duijn
et al., 2010). In studies drawing within-group comparisons across adaptive domains,
socialization (Dykens et al., 1994; Fidler et al., 2006; Marchal et al., 2016), and in some
cases, daily living skills (Dykens et al., 1994) emerge as stronger skills relative to com­
munication abilities.
Along with a well-characterized adaptive profile in DS, there is also an understanding
of certain mechanisms contributing to adaptive impairments. Specifically, attentional
difficulties (Jacola et al., 2014), visual-motor integration (Rihtman et al., 2010), and
repetitive behaviors (Evans et al., 2014) have all been found to predict adaptive abilities
in children with DS. Yet, there is minimal evidence characterizing the relation between
specific EFs and adaptive skills in DS, with, to the best of our knowledge, only one other
study examining this association (Daunhauer et al., 2017). This particular study exam­
ined effects of isolated EFs on self-care skills and found parent reported working memory
abilities to predict self-care outcomes (Daunhauer et al., 2017). While these findings
provide useful insight into the potential association of EF to adaptive skills, they are still
somewhat limited in that only one primary EF (i.e., working memory) was tested as
a predictor of only one domain of adaptive functioning (i.e., self-care). A more complete
understanding of how multiple EFs relate to the various facets of adaptive behavior can
yield insight into optimal intervention targets and strategies to improve adaptive out­
comes in DS.

Current study & aims

Given the preliminary findings associating EF and daily living skills, it is imperative to
understand how specific areas of cognitive impairment, such as EFs, influence domains
of adaptive functioning in DS, especially as individuals with DS experience elevated risk
for vulnerabilities in both cognitive and adaptive skills. As such, we aim to address this
1058 E. A. WILL ET AL.

significant knowledge gap by examining the associations of isolated EFs on domains of

adaptive behavior. Specifically, we utilize a parent report EF measure to identify sig­
nificant EF predictors of communication, daily living, and socialization skills in children
and adolescents with DS. The primary aim of the study was to characterize the relation of
parent reported EFs to parent reported adaptive skills and identify which aspects of EF
are the most salient predictors of adaptive behavior. Working memory and planning have
been consistently established as impaired EFs, and, considering their relation to self-care
skills (Daunhauer et al., 2017), we anticipate these EFs to significantly predict daily living
skills in DS. Further, because working memory is arguably the most significantly affected
EF in DS (Baddeley & Jarrold, 2007; Daunhauer, Fidler, Hahn, et al., 2014; Lanfranchi
et al., 2012, 2010; Will et al., 2017), we also anticipate that this EF will emerge as a salient
predictor across adaptive skills. In post hoc analyses, we considered whether attention
may moderate the association between EFs and adaptive skills, given that attention is
a foundational component for higher order cognitive processes (Diamond, 2013) and
relatively impaired in DS (Ekstein et al., 2011; Jacola et al., 2014; Will et al., 2016).

Methods
Participants
Table 1 presents full participant characteristics. Participants included 68 children with
DS between 6 and 17 years old (CAm = 12.56; IQm = 43.83). The sample was relatively
evenly split across sex (53% male), but primarily Caucasian (88.2%; 3.0% African
American; 4.4% Asian; 4.4% Other) and non-Hispanic (92.6%). Data from the partici­
pants included in the current study is also reported in two other studies focusing on the
psychometric evaluation of neuropsychological measures in DS (Schworer et al., 2021).
Five individuals who participated in the study were excluded from analyses because they

Table 1. Participant characteristics and mean scores for measures of executive func­
tion, adaptive behavior, and ADHD symptomatology, n = 68.
Mean (SD) Minimum Maximum
Chronological age 12.56 (3.22) 6 17
SB-5 ABIQ 48.83 (4.91) 47 76
BRIEF-2 (T scores)
Inhibit 56.25 (10.46) 37 87
Self-Monitor 61.99 (9.41) 39 80
Shift 61.82 (11.11) 44 87
Emotional Control 51.49 (9.28) 40 74
Initiate 61.63 (10.77) 39 90
Working Memory 62.60 (8.85) 42 89
Plan/Organize 57.38 (9.83) 37 80
Task-Monitor 61.34 (8.93) 38 77
Organization of Materials 53.99 (8.79) 38 88
VABS-III ABC standard score 68.60 (10.87) 29 95
Communication standard score 64.19 (15.89) 20 91
Daily Living standard score 66.57 (14.32) 32 104
Socialization standard score 74.10 (13.20) 27 98
Vanderbilt Inattention Sum 11.39 (4.80) 1 23
SB-5 Deviation ABIQ = The Stanford-Binet Intelligence Scales – Fifth Edition abbreviated battery IQ;
BRIEF-2 = Behavior Rating Inventory of Executive Function – Second Edition; VABS-III ABC = Vineland
Adaptive Behavior Scales – 3rd Edition Adaptive Behavior Composite
 CHILD NEUROPSYCHOLOGY 1059

were missing one or more of the parent report questionnaires and analyses required
complete data for those study measures.

Procedures
Procedures for this multi-site study were approved by the Streamlined, Multisite,
Accelerated Resources for Trials (SMART) IRB platform. Participants were recruited
through local DS organizations and a medical center to take part in a larger longitudinal
study on cognition in DS. To be eligible for the study, participants were required to speak
English as their primary language and have a parent reported nonverbal mental age of
approximately 36 months to compete neuropsychology assessments in the larger long­
itudinal study. Documented diagnosis of DS was also required for participation. Study
visits took place at a medical clinic or university laboratory located in midwestern and
western US cities. Data from the first visit of the longitudinal study were used in the
current study. Parents of participants provided written consent and filled out question­
naires on their child’s daily EF skills, adaptive behavior, and ADHD symptomatology.
Participants engaged in a larger battery of neuropsychological assessments, including an
assessment of IQ for the purposes of this study.

Measures
Cognitive ability
The Stanford-Binet Intelligence Scales – Fifth Edition (SB-5; Roid, 2003) was used as
a measure of IQ to describe the participant sample. The SB-5 is a standardized measure of
intelligence and the abbreviated battery IQ (ABIQ) was administered, which includes one
nonverbal subtest (Fluid Reasoning) and one verbal subtest (Knowledge). Reliability for
the ABIQ is high (r = 0.85–0.96) and correlations are strong between the ABIQ and full-
scale IQ (r = 0.89) in other clinical samples (Twomey et al., 2018). Three participants
were missing the SB-5 ABIQ.

Executive functions
Executive function skills were measured using the Behavior Rating Inventory of
Executive Function – Second Edition (BRIEF-2; Goia et al., 2015). The BRIEF-2 is
a standardized parent or teacher report measure of daily EF skills consisting of 63
items which are rated on a 3-point likert scale indicating how frequently a child engages
in a given behavior: never (0), sometimes (1), or often (2); thus, higher scores indicate
greater EF difficulties. The parent-report BRIEF-2, used in the present study, measures
EF skills across three indices: Behavior Regulation (Inhibit, Self-Monitor), Emotion
Regulation (Shift, Emotional Control), and Cognitive Regulation (Initiate, Working
Memory, Plan/Organize, Task-Monitor, Organization of Materials). The BRIEF-2 parent
form has high internal consistency (α = 0.76–0.97) and stable test–retest reliability
(r = 0.67 to 0.92). Previous research establishes the BRIEF (largely similar to the
BRIEF-2) as an appropriate measure of EF in children and adolescents with DS
(Esbensen et al., 2019). For the purposes of this study, index sub-domain T scores
(M = 50; SD = 10) were used as predictors of adaptive behavior outcomes to determine
the isolated effect of specific EFs on adaptive behavior domains. This approach provided
1060 E. A. WILL ET AL.

predictive value for each primary EF – Inhibit, Shift, and Working Memory – as well as
their proximally associated EFs (i.e., Self-Monitor, Emotional Control, Initiate, Plan/
Organize, Task-Monitor, and Organization of Materials).

Adaptive behavior
Adaptive behavior was measured using the Vineland Adaptive Behavior Scales – 3rd
Edition (VABS-III; Sparrow et al., 2016) Parent/Caregiver Form. The VABS-III caregiver
form is a parent report questionnaire that comprehensively measures adaptive behavior
across four domains including Communication, Socialization, Daily Living Skills, and
Motor Skills. Items are scored on an ordinal scale which indicates the frequency at which
the individual independently performs a skill – usually (2), sometimes (1), or never (0).
The VABS-III provides domain standard scores and an overall composite score, the
Adaptive Behavior Composite, each of which have a mean of 100 and standard deviation
of 15. Caregiver form test–retest reliability (r = 0.64–0.94) and internal consistency
(α = 0.95–0.99) are high and the VABS-III is suitable for assessing adaptive skills in DS
(Esbensen et al., 2017). Due to availability of data, the Communication, Socialization, and
Daily Living scores were used in analyses and all analyses used standard scores.

Inattention
The Vanderbilt ADHD Parent Rating Scale (Wolraich et al., 2003) provided a measure of
inattention, which was used in post-hoc moderation analyses (see Data Analyses) to
determine whether significant associations between domains of EF and adaptive behavior
varied as a function of attentional difficulties. The Vanderbilt ADHD includes domains
across Inattention, Hyperactivity, Conduct/oppositional, and Anxiety/Depression.
Parents rate ADHD symptoms on a 4-point Likert scale of 0 to 3 which indicates whether
the symptom occurs: never (0), occasionally (1), often (2), or very often (3) and assesses
level of functional impairment as a result of symptoms. The measure is psychometrically
sound, with high internal consistency (alpha >0.80), reliability and validity (r > 0.80) and
has been recommended for use in individuals with ID (Esbensen et al., 2017). For the
purposes of this study, the summed score of the nine inattention items was used as an
indicator of inattention.

Data analyses
All data were examined for normality of distribution and descriptive statistics were
computed (see Table 1). Preliminary bivariate correlations were tested to determine the
magnitude of correlation between IQ and BRIEF-2 index subdomains, and IQ and
adaptive behavior domains, respectively. As anticipated, IQ was moderately correlated
with all adaptive domains (rs = 0.25–0.36; p-values = .003 – .045). However, IQ was not
significantly correlated with any EF subdomains on the BRIEF-2 (rs = −0.14–0.23; all p-
values > .05) and was therefore determined to not be a potential confound of the
association between EFs and adaptive behavior. We also evaluated CA as a potential
covariate, but no significant correlation was identified between CA and any EF index
subdomain (r = −0.21 – 0.12; all p-values > .05). Given intercorrelations among BRIEF-2
index subdomains (r =0.29–0.73; p-values< .001), we elected to use multivariate regres­
sion models. This modeling approach accounts for potential issues of multicollinearity by
 CHILD NEUROPSYCHOLOGY 1061

yielding the isolated effects of a single independent variable on a single outcome (Johnson
& Wichern, 2007). To maximize statistical power, three separate multivariate models
were estimated in accordance with the three BRIEF-2 index domains: 1) a Behavior
Regulation model, which included Inhibit and Self-Monitor subdomains as predictors; 2)
an Emotion Regulation model that included Shift and Emotional Control subdomains as
predictors; and 3) a Cognitive Regulation model, which included Initiate, Working
Memory, Plan/Organize, Task-Monitor, and Organize Materials subdomains as predic­
tor variables. In each of these models, Communication, Daily Living, and Socialization
standard scores were regressed on each set of predictors associated with the BRIEF-2
Indices (i.e, Behavior, Emotion, and Cognitive Regulation). Using a multivariate regres­
sion approach enabled us to determine the unique predictive value and variance
accounted for (ηp2) by each specific EF on each domain of adaptive functioning.
Further, effect sizes (ηp2) provided information on the salience of each EF for each
adaptive domain and enabled comparisons across EFs that were not included in the
same model.
Finally, significant associations identified between specific EF index subdomains and
adaptive domains prompted post-hoc analyses of the effect between specific EFs and
adaptive domains being moderated by degree of inattention as measured by the
Vanderbilt ADHD parent rating form. We estimated post-hoc moderated regression
models to test whether the effect of EF on adaptive behavior varied as a function of
inattention. Results are outlined below.

Results
Behavior regulation index
The first multivariate regression model tested effects of Inhibit and Self-Monitor on
adaptive behavior domains outcomes. Results showed no significant findings of either

Table 2. Multivariate regression model results.

Communication Daily Living Socialization
B SE(b) p ηp2 B SE(b) p ηp2 B SE(b) p ηp2
Behavior Regulation Model
Intercept 82.01 13.89 <.001 .35 96.12 12.08 <.001 .49 107.53 10.90 <.001 .60
Inhibit −0.14 0.21 .503 .01 −0.30 0.18 .104 .04 −0.27 0.17 .107 .04
Self- Monitor −0.16 0.23 .501 .01 −0.20 0.20 .325 .02 −0.29 0.18 .115 .04
Adjusted R2 < 0.01 Adjusted R2 = 0.07 Adjusted R2 = 0.11
Emotion Regulation Model
Intercept 86.64 12.53 <.001 .42 90.45 11.03 <.001 .51 104.01 9.90 <.001 .63
Shift −0.27 0.20 .180 .03 −0.41 0.17 .023 .08 −0.41 0.16 .011 .10
Emotional Control −0.11 0.24 .631 .00 0.02 0.21 .916 .00 −0.09 0.19 .626 .00
Adjusted R2 = 0.02 Adjusted R2 = 0.07 Adjusted R2 = 0.12
Cognitive Regulation Model
Intercept 74.85 14.55 <.001 .30 84.18 13.34 <.001 .39 103.90 11.42 <.001 .57
Initiate −0.19 0.27 .476 .01 −0.33 0.25 .188 .03 −0.27 0.21 .208 .03
Working Memory −1.06 0.34 .003 .14 −0.69 0.31 .030 .07 −0.94 0.27 .001 .17
Plan/Organize 0.30 0.28 .290 .02 0.36 0.26 .164 .03 0.24 0.22 .288 .02
Task-Monitor 0.62 0.32 .056 .06 0.21 0.29 .473 .01 0.38 0.25 .131 .04
Organization of Materials 0.23 0.27 .405 .01 0.22 0.25 .392 .01 0.16 0.22 .459 .01
2 2 2
Adjusted R = 0.13 Adjusted R = 0.10 Adjusted R = 0.22
1062 E. A. WILL ET AL.

Inhibit or Self-Monitor as a predictor of Communication, Daily Living, or Socialization

adaptive domains (see Table 2).

Emotion regulation index

The second multivariate regression model tested effects of Shift and Emotional Control as
predictors of Communication, Daily Living, or Socialization skills. Overall model results
indicated that Emotion Regulation features (i.e., Shift and Emotional Control) accounted
for a significant portion of variance in Daily Living (AdjR2 = 0.07) and Socialization
(AdjR2 = 0.12), and also revealed a significant overall effect for Shift and adaptive behavior
(F(3, 63) = 3.41; p = .023; ηp2 = 0.14). Individual parameter estimates indicated that Shift
significantly predicted both Daily Living (B = −0.41; p = .023; ηp2 = 0.08), and Socialization
(B = −0.41; p = .011; ηp2 = 0.10). Effect size estimates showed that Shifting accounted for
approximately 8% of unique variance in Daily Living (ηp2 = 0.08) and 10% of the unique
variance in Socialization (ηp2 = 0.10). Each was a moderate effect, and trends indicated that
greater impairments in Shift predicted lower Daily Living and Socialization skills. Shift was
not found to significantly predict Communication (B = −0.27; p = .180). In addition,
Emotional Control was not identified as a significant predictor of any adaptive behavior
domains (see Table 2).

Cognitive regulation index

The third multivariate regression model tested effects of Initiate, Working Memory,
Plan/Organize, Task-Monitor, and Organization of Materials on Communication, Daily
Living, or Socialization skills. Overall model results model results indicated that
Cognitive Regulation features accounted for a significant portion of the variance in
Communication (AdjR2 = 0.13), Daily Living (AdjR2 = 0.10), and Socialization
(AdjR2 = 0.22). Model results also showed an overall significant effect for Working
Memory on adaptive behavior domains (F(3,60) = 4.32; p = .008). Individual parameter
estimates indicated that main effects were primarily driven by Working Memory, with
significant effects on all three domains of adaptive behavior. Specifically, Working
Memory was found to significantly predict Communication (B = −1.06; p = .003; ηp
2
= 0.14), Daily Living (B = −0.69; p = .030; ηp2 = 0.07), and Socialization (B = −0.94;
p = .001; ηp2 = 0.17). Effect size estimates indicated that Working Memory accounted for
approximately 14% of the unique variance in Communication (ηp2 = 0.14), about 7% of
the unique variance in Daily Living Skills (ηp2 = 0.07), and 17% of the unique variance in
Socialization (ηp2 = 0.17). Each of these were moderate-to-large effects, and trends
indicated that lower Working Memory predicted poorer adaptive functioning in each
domain. No other significant effects were identified between Initiate, Plan/Organize,
Task-Monitor, and Organization of Materials and adaptive behavior domains (see
Table 2).

Post hoc moderation analyses

Following our multivariate regression analyses, an additional question arose: Does the
association between EF and adaptive behavior vary as a function of attentional
 CHILD NEUROPSYCHOLOGY 1063

difficulties? To address this post-hoc question, we tested additional moderated regression

models for each significant effect between EFs (i.e., Shift or Working Memory) and
Communication, Daily Living, or Socialization. Each moderated regression model
included the EF predictor, inattention, and an EF x Inattention interaction term.
Results showed no main effects of inattention as a predictor of any adaptive domain;
however, trends toward statistical significance were identified for the association between
inattention and Communication (b = 1.03; p = .078) accounting for Working Memory,
and between inattention and Daily Living (b = 1.00; p = .061) accounting for Working
Memory. In addition, model results showed that inattention did not significantly mod­
erate the effect of Shifting on Daily Living or Socialization, or of Working Memory on
Communication, Daily Living, or Socialization. Interestingly, Shift remained a significant
predictor of Daily Living (b = −0.44; p = .011) and Socialization (b = −0.36; p = .019) even
when accounting for inattention. Likewise, Working Memory also remained a significant
predictor of Communication (b = −0.83; p = .011), Daily Living (b = −0.88; p = .003), and
Socialization (b = −0.72; p = .006) when accounting for inattention.

Discussion
This study presents new findings regarding the influence of specific EF components on
adaptive behavior domains in children and adolescents with DS. Shifting and working
memory, two of the three primary EFs, were related to several aspects of adaptive
functioning. BRIEF-2 Working Memory emerged as the most salient predictor across
each model, relating to all three adaptive domains – Communication, Daily Living Skills,
and Socialization – and demonstrating the largest effect sizes. Although attentional
capacities fundamentally support executive skills, inattention was not found to signifi­
cantly moderate effects between EF components and adaptive domains in our post-hoc
analyses. Interestingly, no other EFs, primary or proximal, on the BRIEF-2 significantly
related to domains of adaptive skills. Collectively, these findings are relatively consistent
with prior work on EF in DS, and they provide insight into the nature and association
between EF and adaptive skills that can inform targets for intervention.

Shifting and adaptive skills

Shifting represents the ability to flexibly focus attention and cognitive effort on relevant
stimuli. Generally regarded as an impaired EF in DS (Tungate & Conners, 2021), shifting
abilities tend to decrease across middle childhood and adolescence in DS (Loveall et al.,
2017). Along with our findings, this suggests increasingly impaired shifting abilities have
a functional impact on multiple adaptive skills for children and adolescents with DS.
Specifically, our results show that greater shifting impairments predict lower adaptive
functioning in both daily living and socialization skills. Daily living skills like dressing,
bathing, and taking medication, naturally require the ability to flexibly reorient attention
as various steps of the task are carried out, or as modifications to the task are required.
Whereas other predictors of daily living abilities have been identified in DS (Daunhauer
et al., 2017), our results provide new evidence that shifting is another EF related to
successful daily adaptation.
1064 E. A. WILL ET AL.

Our findings also highlight a relation between shifting and socialization skills in DS,
and, more specifically, showed that greater difficulties in shifting were associated with
lower socialization skills. Within a social context, shifting is required to hold
a conversation that may transition across various topics, or to maintain interaction
across multiple social partners. Thus, our findings suggest that the shifting difficulties
experienced by children with DS may affect the complexity of their social interactions
and consequently hinder the development of more advanced social skills and/or social
relationships in later development. Shifting difficulties are shown to increase in DS at the
same developmental period in which social demands increase – adolescence (Loveall
et al., 2017). It may be the case that as social expectations increase with age and
development, demands exceed social capabilities of older children and adolescents with
DS due to their challenges with cognitive shifting.

Working memory – a highly salient EF

Our findings identified working memory as the most salient EF associated with adaptive
behavior in that it was associated with all three domains of adaptive functioning and
demonstrated the largest effects with communication and socialization skills.
A considerable amount of work has unequivocally established working memory as an
impaired EF in DS (see Tungate & Conners, 2021 for review), and characterized its role
for reading abilities and academic achievement in DS (Lemons & Fuchs, 2010; Will et al.,
2017). Our specific result that greater difficulties in working memory were associated
with lower daily living skills is consistent with prior evidence for longitudinal associa­
tions between working memory and self-care skills in older children with DS (Daunhauer
et al., 2017). Taken together, these findings underscore the cognitively taxing nature of
daily living skills, which often require holding a substantial amount of information or
tasks in mind while completing various steps (e.g., dressing), in the context of working
memory challenges such as those characterized in DS.
While the relation between working memory and the concrete multi-step skills
involved in executing daily living activities may be clearer, its role in communication
and socialization skills is perhaps slightly more nuanced. Both communication and
socialization skills require real-time updating of stored contextual cues and active recall
of temporarily stored information. As such, proficient working memory abilities are
necessary to successfully understand and produce language and communicative beha­
viors in real-time to effectively convey needs and participate in conversations, as well as
navigate social contexts. The particularly strong effects between working memory and
these adaptive domains suggest that the pronounced working memory difficulties asso­
ciated with the DS phenotype likely have more extensive functional consequences than
previously considered.

Implications for socialization

Of particular note, the largest overall effect size and the greatest amount of variance
accounted for in an adaptive domain was driven by working memory and its effect on
socialization skills. This finding is consistent with prior work in which working
memory was found to account for over 50% of the variance in social cognition in
 CHILD NEUROPSYCHOLOGY 1065

a sample of 6–12-year-old children with DS (Amadó et al., 2016). These collective

findings, along with our result that shifting difficulties were associated with lower
socialization skills, provide insight into features of the DS behavioral phenotype.
Specifically, social abilities have long been regarded as an area of strength in the DS
phenotype (Daunhauer, 2011; Fidler et al., 2008). This characterization is firmly
grounded in evidence from toddlerhood in DS, showing strong social interest (Kasari
& Freeman, 2001) and social abilities commensurate with developmental expectations
(Fidler et al., 2008). Further, when examining within-group adaptive performance,
socialization is often the least affected domain when compared to others for children
with DS (Dykens et al., 1994; Fidler et al., 2006).
However, it may be the case that social abilities in DS are more affected than
previously understood, or more affected at certain developmental periods than
others. Much of the evidence demonstrating strong socialization in DS has emerged
from early developmental periods or from comparisons to inherently less social
groups, such as autism spectrum disorder (Cebula et al., 2010). The degree of shifting
and working memory difficulties identified in DS (Baddeley & Jarrold, 2007;
Daunhauer, Fidler, Hahn, et al., 2014; Tungate & Conners, 2021) paired with pre­
sented and previous findings that establish a link to socialization suggests these EFs
likely hinder effective socialization in later childhood and adolescence. Children and
adolescents with DS may be able to meet basic expectations in a social context, such
as mirror body language, identify emotions expressed by a conversational partner, or
follow subtle social cues, but challenges in both shifting and working memory are
likely to result in challenges or even a full inability to effectively coordinate all the
skills necessary for an effective social interaction. In typical development, shifting
emerges during preschool ages, whereas for working memory, basic abilities to store
information begins in infancy and progresses toward more complex manipulation
across later childhood (Diamond, 2013). As such, significant and increasing (Loveall
et al., 2017) challenges in these EFs in DS are likely to also impact the development
of, or perhaps, the ability to maintain, proficient socialization skills across time.

Implications for other EFs

Somewhat unexpectedly, no other EF components were identified as significant pre­
dictors of adaptive domains in the present study. Inhibition, though impaired in DS
relative to typical development, is a lesser impaired EF relative to both shifting and
working memory (Tungate & Conners, 2021); this profile of impairment may explain
the lack of association between inhibition and the adaptive domains we examined.
Higher-order EF (e.g., Plan/Organize, Task-Monitor, and Organization of Materials),
on the other hand, is well established as particularly impaired in DS (Daunhauer,
Fidler, Hahn, et al., 2014; Fidler et al., 2014; Lee et al., 2011). Additionally, as the
evident culmination of primary EF components, planning is seemingly integral to
successful adaptation, and reasonably so across communication, daily living, and
socialization skills. However, our results did not support a significant relation between
planning and any domain of adaptive behavior we examined. This was also true of
proximal EF components, such as task monitoring or organization of materials. These
findings may indicate that adaptive behavior measures potentially decentralize skills
1066 E. A. WILL ET AL.

into incremental parts, thus failing to capture the full skill expression that higher-order
EFs would require. Alternatively, it could be the case that because primary EFs are
foundational, they have a greater association to adaptive skills.

Effects of attention on EF-adaptive associations

Attention is a core component of EF as focused attention facilitates other primary EFs
(Diamond, 2013). Accordingly, we questioned whether inattention would moderate the
effects of specific EFs on adaptive domains and tested this hypothesis for each statistically
significant EF-adaptive relation identified. However, no significant moderating effects of
attention were found. This was not only surprising given the foundational role attention
plays in EF processes, but also in that attention is considerably disrupted in DS (Ekstein
et al., 2011). Further, links between attention and adaptive skills have been previously
identified in adolescents with DS (Jacola et al., 2014), indicating even stronger evidence
that attention may affect the relation between EF and adaptive behavior. The lack of
significant effects in our study suggests that attentional difficulties in DS may in fact exert
a moderating influence on the relation between EF and adaptive skills, but perhaps just
not as measured in the present study. Given how central attention is to the primary EFs
(Diamond, 2013), certain measures of attention, or EF for that matter, may pose too great
a challenge in disentangling these various components in order to effectively detect their
distinct roles in relation to one another.

Implications of findings for intervention targets

Considerable evidence demonstrates the malleability of EFs (Bergman Nutley et al., 2011;
Diamond & Lee, 2011), and our results offer implications for improved EFs in children
and adolescents with DS. Primarily, our findings provide support for shifting and work­
ing memory as potential intervention targets to improve adaptive skill outcomes.
Further, the magnitude of association between working memory and all adaptive
domains we examined suggests particular emphasis on improving this primary EF
component could likely lead to enhanced adaptive outcomes for children and adolescents
with DS. There is encouraging evidence that working memory can be improved through
various types of training approaches for children and/or adolescents with DS (Conners
et al., 2008; Conners et al., 2001; Sabou et al., 2012). However, much still remains to
understand regarding working memory interventions in DS. For instance, there have
been mixed results regarding efficacy depending on whether auditory or visuospatial
modalities are targeted in training (see Conners et al., 2001). Further, the majority of
research on working memory training in DS, or other populations with ID, have focused
on whether training improves working memory specifically, rather than distal effects on
other domains of functioning, such as adaptive behavior. Accordingly, further investiga­
tion into how EF improvement relates to additional outcomes is warranted.

Study limitations and future directions

Our study provides novel insight into the role of specific EFs on communication, daily
living, and socialization skills in children and adolescents with DS, though there were
 CHILD NEUROPSYCHOLOGY 1067

some limitations. Primarily, our findings are restricted to the specific characteristics of
the included sample and measurement approaches. Our study also lacked a comparison
group, either to typical development, or another group with a neurogenetic condition,
which restricts the generalizability of our findings to some degree. Finally, our measures
consisted of parent-report instruments which introduces the possibility of shared method
variance contributing to effects, and may also provide a narrow assessment of the nature
or degree to which EF impacts adaptive skills in DS.
Despite these few limitations, our findings provide several avenues for future
research. Future work should aim toward replication of our findings with laboratory-
based EF measures and also focus on identifying longitudinal age-related trends in
EFs and influences on adaptive functioning. These efforts, provided findings are
replicated, would further strengthen support for the evident relation between EFs
and adaptive functioning identified in our study. Additionally, future work should
investigate the extent to which our findings may be specific to DS, or to ID more
broadly, which could inform syndrome-specific intervention targets. Finally, EF
training programs, and perhaps working memory training programs more specifi­
cally, should be further tested to confirm the potential efficacy in not only improving
working memory, but also targeting adaptive behavior skills for children and/or
adolescents with DS.

Summary and conclusions

Our study was a novel investigation into the role of and degree to which specific EFs
influence specific aspects of adaptive behavior/functioning. We identified working mem­
ory as the most salient EF associated with adaptive functioning, as it was the only EF
domain to significantly relate to communication, daily living, and socialization skills.
While significant effects for shifting on some aspects of adaptive behavior were also
found, our findings provide new evidence for the extended influence of working memory
on yet another aspect of functioning for individuals with DS. Along with future research,
these findings can contribute to improved adaptive outcomes for DS through interven­
tion development.

Disclosure statement
No potential conflict of interest was reported by the author(s).

Funding
This manuscript was prepared with support from the Eunice Kennedy Shriver National Institute of
Child Health and Human Development of the National Institutes of Health [F32HD097877, Will
PI; L40 HD103202, Will PI; R01 HD093754, Esbensen PI] and the National Institute of Mental
Health [L40 MH117727, Will PI]. The content is solely the responsibility of the authors and does
not necessarily represent the official views of the National Institutes of Health. This research would
not have been possible without the contributions of the participating families and the community
support.
1068 E. A. WILL ET AL.

References
Abbeduto, L., Warren, S. F., & Conners, F. A. (2007). Language development in Down syndrome:
From the prelinguistic period to the acquisition of literacy. Mental Retardation and
Developmental Disabilities Research Reviews, 13(3), 247–261. https://doi.org/10.1002/mrdd.
20158
Amadó, A., Serrat, E., & Vallès-Majoral, E. (2016). The role of executive functions in social
cognition among children with Down syndrome: Relationship patterns. Frontiers in
Psychology, 7, 1363. https://doi.org/10.3389/fpsyg.2016.01363
Baddeley, A., & Jarrold, C. (2007). Working memory and Down syndrome. Journal of Intellectual
Disability Research: JIDR, 51(Pt12), 925–931. MEDLINE with Full Text. https://doi.org/10.
1111/j.1365-2788.2007.00979.x
Baddeley, A. D., & Hitch, G. (1974). Working memory. In G. A. Bower (Ed.), The psychology of
learning and motivation (Vol. 8, pp. 47–89). Academic Press.
Bergman Nutley, S., Söderqvist, S., Bryde, S., Thorell, L. B., Humphreys, K., & Klingberg, T. (2011).
Gains in fluid intelligence after training non-verbal reasoning in 4-year-old children:
A controlled, randomized study. Developmental Science, 14(3), 591–601. https://doi.org/10.
1111/j.1467-7687.2010.01022.x
Blair, C., & Razza, R. P. (2007). Relating effortful control, executive function, and false belief
understanding to emerging math and literacy ability in kindergarten. Child Development, 78(2),
647–663. MEDLINE with Full Text. https://doi.org/10.1111/j.1467-8624.2007.01019.x
Borella, E., Carretti, B., & Lanfranchi, S. (2013). Inhibitory mechanisms in Down syndrome: Is
there a specific or general deficit? Research in Developmental Disabilities, 34(1), 65–71. https://
doi.org/10.1016/j.ridd.2012.07.017
Bornstein, M. H., Hahn, C.-S., & Suwalsky, J. T. D. (2013). Physically developed and exploratory
young infants contribute to their own long-term academic achievement. Psychological Science,
24(10), 1906–1917. https://doi.org/10.1177/0956797613479974
Carney, D. P. J., Brown, J. H., & Henry, L. A. (2013). Executive function in Williams and Down
syndromes. Research in Developmental Disabilities, 34(1), 46–55. https://doi.org/10.1016/j.ridd.
2012.07.013
Cebula, K. R., Moore, D. G., & Wishart, J. G. (2010). Social cognition in children with Down’s
syndrome: Challenges to research and theory building. Journal of Intellectual Disability
Research, 54(2), 113–134. https://doi.org/10.1111/j.1365-2788.2009.01215.x
Conners, F., Rosenquist, C., & Taylor, L. (2001). Memory training for children with Down
syndrome. Down Syndrome Research and Practice, 7(1), 25–33. https://doi.org/10.3104/
reports.111
Conners, F. A., Moore, M. S., Loveall, S. J., & Merrill, E. C. (2011). Memory profiles of Down,
Williams, and Fragile X syndromes: Implications for reading development. Journal of
Developmental & Behavioral Pediatrics, 32(5), 405–417. https://doi.org/10.1097/DBP.
0b013e3182168f95
Conners, F. A., Rosenquist, C. J., Arnett, L., Moore, M. S., & Hume, L. E. (2008). Improving
memory span in children with Down syndrome. Journal of Intellectual Disability Research, 52
(3), 244–255. https://doi.org/10.1111/j.1365-2788.2007.01015.x
Costanzo, F., Varuzza, C., Menghini, D., Addona, F., Gianesini, T., & Vicari, S. (2013). Executive
functions in intellectual disabilities: A comparison between Williams syndrome and Down
syndrome. Research in Developmental Disabilities, 34(5), 1770–1780. https://doi.org/10.1016/j.
ridd.2013.01.024
Daunhauer, L. (2011). The early development of adaptive behavior and functional performance in
young children with Down syndrome: Current knowledge and future directions. In D. J. Fidler
(Ed.), International review of research in developmental disabilities (Vol. 40, pp. 109–137).
Elsevier. https://doi.org/10.1016/B978-0-12-374478-4.00005-8
Daunhauer, L. A., Fidler, D. J., Hahn, L., Will, E., Lee, N. R., & Hepburn, S. (2014). Profiles of
everyday executive functioning in young children with Down syndrome. American Journal on
 CHILD NEUROPSYCHOLOGY 1069

Intellectual and Developmental Disabilities, 119(4), 303–318. https://doi.org/10.1352/1944-

7558-119.4.303
Daunhauer, L. A., Fidler, D. J., & Will, E. (2014). School function in students with Down
syndrome. American Journal of Occupational Therapy, 68(2), 167–176. https://doi.org/10.
5014/ajot.2014.009274
Daunhauer, L. A., Gerlach-mcdonald, B., Will, E., & Fidler, D. J. (2017). Performance and ratings
based measures of executive function in school-aged children with Down syndrome.
Developmental Neuropsychology, 42(6), 351–368. https://doi.org/10.1080/87565641.2017.
1360303
Diamond, A. (2013). Executive functions. Annual Review of Psychology, 64(1), 135–168. https://
doi.org/10.1146/annurev-psych-113011-143750
Diamond, A., & Lee, K. (2011). Interventions shown to aid executive function development in
children 4 to 12 years old. Science, 333(6045), 959–964. https://doi.org/10.1126/science.
1204529
Ditterline, J., & Oakland, T. (2009). Relationships between adaptive behavior and impairment. In
J. Naglieri & S. Goldstein (Eds.), Assessing impairment: From theory to practice (pp. 31–48).
Springer US. https://doi.org/10.1007/978-1-387-87542-2_4
Dykens, E., Hodapp, R., & Evans, D. (1994). Profiles and development of adaptive behavior in
children with Down syndrome. American Journal on Mental Retardation, 98(5), 580–587.
PMID: 8192903.
Ekstein, S., Glick, B., Weill, M., Kay, B., & Berger, I. (2011). Down syndrome and attention-deficit/
hyperactivity disorder (ADHD). Journal of Child Neurology, 26(10), 1290–1295. https://doi.org/
10.1177/0883073811405201
Esbensen, A. J., Hoffman, E. K., Shaffer, R., Chen, E., Patel, L., & Jacola, L. (2019). Reliability of
informant-report measures of executive functioning in children with Down syndrome.
American Journal on Intellectual and Developmental Disabilities, 124(3), 220–233. https://doi.
org/10.1352/1944-7558-124.3.220
Esbensen, A. J., Hooper, S. R., Fidler, D., Hartley, S. L., Edgin, J., d’Ardhuy, X. L., Capone, G.,
Conners, F. A., Mervis, C. B., Abbeduto, L., Rafii, M., Krinsky-mchale, S. J., & Urv, T., & Group,
O. M. W. (2017). Outcome measures for clinical trials in Down syndrome. American Journal on
Intellectual and Developmental Disabilities, 122(3), 247–281. https://doi.org/10.1352/1944-
7558-122.3.247
Evans, D. W., Kleinpeter, F. L., Slane, M. M., & Boomer, K. B. (2014). Adaptive and maladaptive
correlates of repetitive behavior and restricted interests in persons with Down syndrome and
developmentally-matched typical children: A two-year longitudinal sequential design. PLoS
ONE, 9(4), e93951. https://doi.org/10.1371/journal.pone.0093951
Fidler, D., Hepburn, S., & Rogers, S. (2006). Early learning and adaptive behaviour in toddlers with
Down syndrome: Evidence for an emerging behavioural phenotype? Down Syndrome Research
and Practice, 9(3), 37–44. https://doi.org/10.3104/reports.297
Fidler, D. J., Most, D. E., Booth-laforce, C., & Kelly, J. F. (2008). Emerging social strengths in young
children with Down syndrome. Infants and Young Children, 21(3), 207–220. https://doi.org/10.
1097/01.IYC.0000324550.39446.1f
Fidler, D. J., Will, E., Daunhauer, L. A., Gerlach-mcdonald, B., & Visootsak, J. (2014). Object-
related generativity in children with Down syndrome. Research in Developmental Disabilities, 35
(12), 3379–3385. https://doi.org/10.1016/j.ridd.2014.07.024
Goia, G. A., Isquith, P. K., Guy, S. C., & Kenworthy, L. (2015). Brief-2: Behavior rating inventory of
executive function: Professional manual. Psychological Assessment Resources.
Jacola, L. M., Hickey, F., Howe, S. R., Esbensen, A., & Shear, P. K. (2014). Behavior and adaptive
functioning in adolescents with Down syndrome: Specifying targets for intervention. Journal of
Mental Health Research in Intellectual Disabilities, 7(4), 287–305. https://doi.org/10.1080/
19315864.2014.920941
Jarrold, C., Baddeley, A. D., & Hewes, A. K. (1999). Genetically dissociated components of working
memory: Evidence from Downs and Williams syndrome. Neuropsychologia, 37(6), 637–651.
https://doi.org/10.1016/S0028-3932(98)00128-6
1070 E. A. WILL ET AL.

Jarrold, C., Thorn, A. S. C., & Stephens, E. (2009). The relationships among verbal short-term
memory, phonological awareness, and new word learning: Evidence from typical development
and Down syndrome. Journal of Experimental Child Psychology, 102(2), 196–218. https://doi.
org/10.1016/j.jecp.2008.07.001
Johnson, R., & Wichern, D. (2007). Applied multivariate statistical analysis (6th ed.). Prentice-Hall.
Kasari, C., & Freeman, S. F. N. (2001). Task-related social behavior in children with Down
syndrome. American Journal on Mental Retardation, 106(3), 253–264. https://doi.org/10.1352/
0895-8017(2001)106%3C0253:TRSBIC%3E2.0.CO;2
Kittler, P., Krinsky-mchale, S. J., & Devenny, D. A. (2006). Verbal intrusions precede memory
decline in adults with Down syndrome. Journal of Intellectual Disability Research: JIDR, 50(Pt
1), 1–10. https://doi.org/10.1111/j.1365-2788.2005.00715.x
Lanfranchi, S., Baddeley, A., Gathercole, S., & Vianello, R. (2012). Working memory in Down
syndrome: Is there a dual task deficit?: Executive processing in Down syndrome. Journal of
Intellectual Disability Research, 56(2), 157–166. https://doi.org/10.1111/j.1365-2788.2011.01444.x
Lanfranchi, S., Cornoldi, C., & Vianello, R. (2004). Verbal and visuospatial working memory
deficits in children with Down syndrome. American Journal on Mental Retardation, 109(6), 11.
https://doi.org/10.1352/0895-8017(2004)109<456:VAVWMD>2.0.CO;2
Lanfranchi, S., Jerman, O., Dal Pont, E., Alberti, A., & Vianello, R. (2010). Executive function in
adolescents with Down syndrome: Executive function in Down syndrome. Journal of
Intellectual Disability Research, 54(4), 308–319. https://doi.org/10.1111/j.1365-2788.2010.
01262.x
Lee, N. R., Anand, P., Will, E., Adeyemi, E. I., Clasen, L. S., Blumenthal, J. D., Giedd, J. N.,
Daunhauer, L. A., Fidler, D. J., & Edgin, J. O. (2015). Everyday executive functions in Down
syndrome from early childhood to young adulthood: Evidence for both unique and shared
characteristics compared to youth with sex chromosome trisomy (XXX and XXY). Frontiers in
Behavioral Neuroscience, 9, 264. https://doi.org/10.3389/fnbeh.2015.00264
Lee, N. R., Fidler, D. J., Blakeley-Smith, A., Daunhauer, L., Robinson, C., & Hepburn, S. L. (2011).
Caregiver report of executive functioning in a population-based sample of young children with
Down syndrome. American Journal on Intellectual and Developmental Disabilities, 116(4),
290–304. https://doi.org/10.1352/1944-7558-116.4.290
Lemons, C. J., & Fuchs, D. (2010). Phonological awareness of children with Down syndrome: Its
role in learning to read and the effectiveness of related interventions. Research in Developmental
Disabilities, 31(2), 316–330. https://doi.org/10.1016/j.ridd.2009.11.002
Loveall, S. J., Conners, F. A., Tungate, A. S., Hahn, L. J., & Osso, T. D. (2017). A cross-sectional
analysis of executive function in Down syndrome from 2 to 35 years: A cross-sectional analysis
of executive function in Down syndrome. Journal of Intellectual Disability Research, 61(9),
877–887. https://doi.org/10.1111/jir.12396
Marchal, J. P., Maurice-Stam, H., Houtzager, B. A., Rutgers Van Rozenburg-marres, S. L.,
Oostrom, K. J., Grootenhuis, M. A., & Van Trotsenburg, A. S. P. (2016). Growing up with
Down syndrome: Development from 6 months to 10.7 years. Research in Developmental
Disabilities, 59, 437–450. https://doi.org/10.1016/j.ridd.2016.09.019
Miyake, A., & Friedman, N. P. (2012). The nature and organization of individual differences in
executive functions: Four general conclusions. Current Directions in Psychological Science, 21
(1), 8–14. https://doi.org/10.1177/0963721411429458
Miyake, A., Friedman, N. P., Emerson, M. J., Witzki, A. H., Howerter, A., & Wager, T. D. (2000).
The unity and diversity of executive functions and their contributions to complex “Frontal
Lobe” tasks: A latent variable analysis. Cognitive Psychology, 41(1), 49–100. https://doi.org/10.
1006/cogp.1999.0734
Næss, K.-A. B., Lyster, S.-A. H., Hulme, C., & Melby-Lervåg, M. (2011). Language and verbal
short-term memory skills in children with Down syndrome: A meta-analytic review. Research in
Developmental Disabilities, 32(6), 2225–2234. https://doi.org/10.1016/j.ridd.2011.05.014
Numminen, H., Service, E., Ahonen, T., & Ruoppila, I. (2001). Working memory and everyday
cognition in adults with Down’s syndrome. Journal of Intellectual Disability Research, 45(2),
157–168. https://doi.org/10.1046/j.1365-2788.2001.00298.x
 CHILD NEUROPSYCHOLOGY 1071

Phillips, B. A., Conners, F. A., Merrill, E., & Klinger, M. R. (2014). Rule-based category learning in
Down syndrome. American Journal on Intellectual and Developmental Disabilities, 119(3),
220–234. https://doi.org/10.1352/1944-7558-119.3.220
Racz, S. J., Putnick, D. L., Suwalsky, J. T. D., Hendricks, C., & Bornstein, M. H. (2017). Cognitive
abilities, social adaptation, and externalizing behavior problems in childhood and adolescence:
Specific cascade effects across development. Journal of Youth and Adolescence, 46(8),
1688–1701. https://doi.org/10.1007/s10964-016-0602-3
Rihtman, T., Tekuzener, E., Parush, S., Tenenbaum, A., Bachrach, S. J., & Ornoy, A. (2010). Are the
cognitive functions of children with Down syndrome related to their participation?
Developmental Medicine and Child Neurology, 52(1), 72–78. https://doi.org/10.1111/j.1469-
8749.2009.03356.x
Roid, G. H. (2003). Stanford-BInet Intelligence Scales (SB-5). Riverside Publishing.
Rowe, J., Lavender, A., & Turk, V. (2006). Cognitive executive function in Down’s syndrome.
British Journal of Clinical Psychology, 45(1), 5–17. https://doi.org/10.1348/014466505X29594
Sabou, A. M., Moldovan, M., Cosma, A. M., & Visu-Petra, L. (2012). Working memory training in
typical and atypical development: Revisiting the evidence. Cognitie, Creier, Comportament/
Cognition, Brain, Behavior, 16(1), 1–47.
Schalock, R. L., Borthwick-Duffy, S. A., Bradley, V. J., Buntinx, W. H. E., Coulter, D. L.,
Craig, E. M., Gomez, S. C., Lachapelle, Y., Luckasson, R., Reeve, A., Shogren, K. A.,
Snell, M. E., Spreat, S., Tasse, M. J., Thompson, J. R., Verdugo-Alonso, M. A.,
Wehmeyer, M. L., & Yeager, M. H. (2010). Intellectual disability: Definition, classification, and
systems of supports (11th ed.). American Association on Intellectual and Developmental
Disabilities.
Schworer, E. K., Esbensen, A. J., Fidler, D. J., Beebe, D. W., Carle, A., & Wiley, S. (2021). Evaluating
working memory outcome measures for children with Down syndrome. Journal of Intellectual
Disability Research. Advance online publication. https://doi.org/10.1111/jir.12833
Silverman, W. (2007). Down syndrome: Cognitive phenotype. Mental Retardation and
Developmental Disabilities Research Reviews, 13(3), 228–236. https://doi.org/10.1002/mrdd.
20156
Sparrow, S. S., Cicchetti, D. V., & Saulnier, C. A. (2016). Vineland Adaptive Behavior Scales (3rd
ed.). (Vineland-3). PsychCorp.
Tassé, M. J., Luckasson, R., & Schalock, R. L. (2016). The relation between intellectual functioning
and adaptive behavior in the diagnosis of intellectual disability. Intellectual and Developmental
Disabilities, 54(6), 381–390. https://doi.org/10.1352/1934-9556-54.6.381
Tomaszewski, B., Fidler, D., Talapatra, D., & Riley, K. (2018). Adaptive behaviour, executive
function and employment in adults with Down syndrome: Employment in adults with Down
syndrome. Journal of Intellectual Disability Research, 62(1), 41–52. https://doi.org/10.1111/jir.
12450
Tungate, A. S., & Conners, F. A. (2021). Executive function in Down syndrome: A meta-analysis.
Research in Developmental Disabilities, 108, 103802. https://doi.org/10.1016/j.ridd.2020.
103802
Twomey, C., O’Connell, H., Lillis, M., Tarpey, S. L., & O’Reilly, G. (2018). Utility of an abbreviated
version of the Stanford-Binet intelligence scales (5th ed.) in estimating ‘full scale’ IQ for young
children with autism spectrum disorder. Autism Research, 11(3), 503–508. https://doi.org/10.
1002/aur.1911
Van Duijn, G., Dijkxhoorn, Y., Scholte, E. M., & Van Berckelaer-Onnes, I. A. (2010). The
development of adaptive skills in young people with Down syndrome: Adaptive skills in
young people with Down syndrome. Journal of Intellectual Disability Research, 54(11),
943–954. https://doi.org/10.1111/j.1365-2788.2010.01316.x
Will, E., Fidler, D. J., Daunhauer, L., & Gerlach-mcdonald, B. (2017). Executive function and
academic achievement in primary-grade students with Down syndrome: EF and academics in
Down syndrome. Journal of Intellectual Disability Research, 61(2), 181–195. https://doi.org/10.
1111/jir.12313
1072 E. A. WILL ET AL.

Will, E. A., Caravella, K. E., Hahn, L. J., Fidler, D. J., & Roberts, J. E. (2018). Adaptive behavior in
infants and toddlers with Down syndrome and Fragile X syndrome. American Journal of
Medical Genetics Part B: Neuropsychiatric Genetics, 177(3), 358–368. https://doi.org/10.1002/
ajmg.b.32619
Will, E. A., Gerlach-mcdonald, B., Fidler, D. J., & Daunhauer, L. A. (2016). Impact of maladaptive
behavior on school function in Down syndrome. Research in Developmental Disabilities, 59,
328–337. https://doi.org/10.1016/j.ridd.2016.08.018
Wolraich, M. L., Lambert, W., Doffing, M. A., Bickman, L., Simmons, T., & Worley, K. (2003).
Psychometric properties of the Vanderbilt ADHD diagnostic parent rating scale in a referred
population. Journal of Pediatric Psychology, 28(8), 559–568. https://doi.org/10.1093/jpepsy/
jsg046
Woolf, S., Woolf, C. M., & Oakland, T. (2010). Adaptive behavior among adults with intellectual
disabilities and its relationship to community independence. Intellectual and Developmental
Disabilities, 48(3), 209–215. https://doi.org/10.1352/1944-7558-48.3.209
Yang, Y., Conners, F. A., & Merrill, E. C. (2014). Visuo-spatial ability in individuals with Down
syndrome: Is it really a strength? Research in Developmental Disabilities, 35(7), 1473–1500.
https://doi.org/10.1016/j.ridd.2014.04.002
Zelazo, P. D. (2006). The dimensional change card sort (DCCS): A method of assessing executive
function in children. Nature Protocols, 1(1), 297–301. http://dx.doi.org.pallas2.tcl.sc.edu/10.
1038/nprot.2006.46