Will Hepburn 2015

CHAPTER SIX
Applied Behavior Analysis for

Children with Neurogenetic
Disorders
Elizabeth Will*, 1, Susan Hepburnx
*Department of Human Development and Family Studies, Colorado State University, Fort Collins, CO, USA
x
JFK Partners, Department of Psychiatry, University of Colorado, School of Medicine, Aurora, CO, USA
1
Corresponding author: e-mail address: Elizabeth.will@colostate.edu
Contents
1. Introduction 230
2. Utility of Applied Behavior Analysis: Early Intervention 232
3. Definition and Background 233
4. Foundational Principles of ABA 234
5. Use of ABA Principles in Skill Acquisition (Behavioral Deficits) 235
5.1 Additional Techniques in Using ABA as an Effective Instructional Tool 235
6. Use of ABA Techniques in Addressing Challenging Behavior (Behavioral Excesses) 237
7. Evolution of ABA in the United States 238
7.1 Discrete Trial Training 239
7.2 Pivotal Response Training 239
7.3 ABA in Special Education Settings 240
8. Current Status 241
9. Barriers to the Use of ABA in Neurogenetic Syndromes 242
9.1 Policy 242
9.2 Single-Subject Methodology 243
9.3 Need for Innovation 245
10. ABA in Neurogenetic Syndromes 246
10.1 Current Applications 246
10.2 SmitheMagenis Syndrome 246
10.3 PradereWilli Syndrome 247
10.4 Williams Syndrome 249
10.5 Down Syndrome 249
11. Conclusions 252
References 253
Abstract
Applied behavior analysis (ABA) is a behaviorally based intervention approach that has
significant implications for improving outcomes for individuals with intellectual and
developmental disabilities. Despite its broad utility as an effective instructional tool, it
International Review of Research in Developmental Disabilities, Volume 49
© 2015 Elsevier Inc.
j
ISSN 2211-6095
http://dx.doi.org/10.1016/bs.irrdd.2015.06.004 All rights reserved. 229
230 Elizabeth Will and Susan Hepburn
has been implemented mostly with behaviorally diagnosed conditions, such as autism
and emotionalebehavioral disorders. Individuals with neurogenetic syndromes, such
as Down syndrome, have behavioral profiles that are probabilistically unique to that
syndrome, but that also share considerable overlap in cognitive, language, and adap-
tive abilities with developmental disorders that ABA has proven efficacy for, such as
autism. Application of ABA for individuals with neurogenetic conditions thus far, has
been limited primarily to addressing challenging behavior. The utility of ABA as a
comprehensive, developmentally appropriate, early and intensive behavioral interven-
tion for individuals with neurogenetic syndromes is discussed. Further research on the
efficacy and utility of ABA as an early intensive behavioral intervention for children with
neurogenetic disorders is critical and necessary to move toward innovative early inter-
ventions for this population.
1. INTRODUCTION
Many individuals with developmental disabilities have an intellectual
disability with a genetic etiology. These types of disorders are often referred
to as “neurogenetic” disorders, because the underlying genetic etiology of
the disorder predisposes them to a profile of atypical neurodevelopment.
From this genetic etiology, a characteristic behavioral profile, or behavioral
phenotype, emerges. The underlying genetic origin contributes to (or
shapes) the behavioral phenotype, such that a specific behavioral profile is
uniquely associated with a particular neurogenetic disorder. Behavioral phe-
notypes emerge in a probabilistic manner, often shaped by interactions with
the environment (Dykens & Hodapp, 2001). As such, there is not a one-to-
one correspondence between a neurogenetic disorder and a behavioral pro-
file. Rather, there is a greater likelihood that an individual’s profile will
reflect a particular disorder relative to that of another disorder, but there is
also overlap between neurogenetic behavioral phenotypes, as well as
within-syndrome variability (Dykens, 1995; Fidler, 2005; Hodapp, 1997).
The complex interactive nature of emerging behavioral phenotypes across
the life span suggests that environmental contingencies can be structured
in therapeutic ways to address areas of relative challenge in specific neuro-
genetic disorders (Table 1).
Behavioral phenotypes are characterized as areas of strength and challenge
across domains of functioning, relative to an individual’s developmental sta-
tus. The Down syndrome (DS) behavioral phenotype, for example, is char-
acterized by areas of strength relative in the areas of receptive language, visual
processing, imitation skills, and social relatedness (Daunhauer & Fidler, 2011;
Applied Behavior Analysis for Children with Neurogenetic Disorders
Table 1 Areas of Phenotypic Strength and Challenge in Neurogenetic Syndromes
Relative Strength Relative Challenge Problem Behaviors
SmitheMagenis syndrome Long-term memory Short-term memory Self-injury

Expressive language Attention Aggression
Spatial perception Impulsivity Hyperactivity
Sequential processing Stereotypy
(Arron et al., 2011; Dykens et al., 1997; Garayzabal et al., 2011; Osorio et al., 2012; Taylor & Oliver, 2008)
PradereWilli syndrome Spatial processing Hyperphagia Aggression
Simultaneous processing Obsessive-compulsive behavior Tantrums
(Dykens, 2002; Hiraiwa et al., 2007; Stokes & Luiselli, 2009)
Williams syndrome Auditory processing Visuospatial processing Anxiety and obsessions
Expressive language Fluid language Social difficulties
Attention
(Fidler et al., 2000; Klein-Tasman et al., 2015; Mervis et al., 2000)
Down syndrome Receptive language Expressive language Elopement
Social relatedness Verbal processing Noncompliance
Imitation Attention Task-avoidance
(Feeley & Jones, 2006; Fidler, 2005; Daunhauer, Fidler, & Will, 2014; Kasari & Freeman, 2001)
231
Fidler, 2005). Likewise, it is characterized by areas of challenge in the areas of

verbal processing, expressive language, memory, and motor skills (Fidler,
2005; Fidler & Nadel, 2007). Individuals with DS may have greater strengths
or greater challenges in some of these areas compared to other individuals
with DS, and an individual’s profile may change over time, as often,
memory abilities decline further in later adulthood in DS. Despite within-
syndrome variability, the genetic etiology of Trisomy 21 predisposes indi-
viduals to this behavioral profile. As with both typical development, and
behaviorally diagnosed developmental disorders, foundations created early
on in development facilitate optimal developmental trajectories, and the
same is true for neurogenetic disorders (Baker & Feinfield, 2003; Fidler &
Nadel, 2007).
2. UTILITY OF APPLIED BEHAVIOR ANALYSIS: EARLY

INTERVENTION
Early comprehensive behavioral interventions, grounded in theory
and practice of Applied behavior analysis (ABA), have been championed
in treating autism spectrum disorder (ASD), and have been shown to be
highly successful at remediating areas of deficit that are often also character-
istic of neurogenetic disorders (Dawson et al., 2009; Koegel & Koegel, 1988;
Lovaas, 2002). Although individuals with neurogenetic disorders potentially
receive many types of behavioral intervention services, there has been a sig-
nificant lack of emphasis on early comprehensive behavioral intervention for
these children. Comprehensive early behavioral intervention that targets
multiple developmental domains, such as language, motor, play skills, and
socioemotional development, has been shown to be highly effective at
remediating deficits in behaviorally diagnosed developmental disorders,
such as ASD when implemented early (Bailey, Aytch, Odom, Symons, &
Wolery, 1999; Hadders-Algra, 2011; Rogers & Dawson, 2010). Because
neurogenetic disorders are diagnosed very early in the life span, these chil-
dren have a wide and optimal window for early comprehensive interven-
tion, which could facilitate optimal trajectories; however, comprehensive
behavioral interventions for young children with neurogenetic disorders
are underutilized and understudied.
ABA has been demonstrated as a highly effective methodology for teaching
skills across multiple domains of development and addressing challenging
behaviors that impede daily functioning (Koegel & Koegel, 1988; Lovaas,
Berberich, Perloff, & Schaeffer, 1966; Lovaas, Freitag, Gold, & Kassorla,
Applied Behavior Analysis for Children with Neurogenetic Disorders 233
1965; Rogers & Dawson, 2010). Because ABA is a behaviorally based meth-
odology, efforts toward demonstrating these effects have focused on disorders
that begin with atypical behavior, rather than an underlying genetic etiology.
Although benefits of ABA for populations with behavioral disorders have
been great, individuals with genetic disorders have not necessarily had the op-
portunity for such benefit. The focus of this paper is to consider, based on the
underlying theoretical and core principles of ABA, its application and utility
for populations of individuals with neurogenetic disorders, particularly DS.
3. DEFINITION AND BACKGROUND

ABA is the systematic study of behavior and application of techniques
to promote behavior that leads to an improved quality of life for individuals
(Cooper, Heron, & Heward, 2007). ABA is rooted in learning theory and
Skinner’s theory of operant behavior and has gradually evolved over the
course of the past 60 years (Lerman, Iwata, & Hanley, 2013). Skinner
defined operant learning as the interaction between behavior and environ-
ment, from which an individual’s behavioral repertoire is developed
(Skinner, 1953). Within the context of the operant framework and manip-
ulating environmental events, early research began to show the effectiveness
of changing behavior in a way that allowed for learning to occur. Parallel to
the occurrence of learning, research began to show that less desirable behav-
iors, such as aggression and self-injury could be managed effectively (Lerman
et al., 2013). Over time, ABA has evolved to encompass the application of
effective instructional strategies and systematic use of the environment in
addressing two key areas for individuals: (1) teaching effective and necessary
skills; and (2) shaping a behavioral repertoire to minimize challenging be-
haviors that impede an individual’s daily life, while promoting optimal be-
haviors that improve functioning.
Core tenets of ABA contribute to its utility as an intervention method.
First and foremost, because it is applied in nature, it is used to target neces-
sary, meaningful, and observable behaviors in a practical context, to the
benefit of the individual (Cooper et al., 2007; Lerman et al., 2013). Behav-
iors, whether a skill being taught, or a challenging behavior to be minimized,
are systematically broken down into components to allow for adequate anal-
ysis and to address the factors responsible for instilling a skill or diminishing a
problematic behavior (Umbreit, Ferro, Liaupsin, & Lane, 2007). In this
systematic process, behaviors, their maintaining factors, and intervention
strategies are well defined and measured using data, in order to ensure
replicability and efficacy of the intervention. In particular, the level change

in behavior is evaluated to determine whether it is sizable enough to make a
practical difference in the individual’s life, and remain consistent across a va-
riety of contexts (Baer, Wolf, & Risley, 1968; Cooper et al., 2007; Lerman
et al., 2013; Umbreit et al., 2007). These core tenets, embedded in the
fundamental ABA approach, collectively facilitate a highly individualized
intervention approach, which has contributed to the efficacy of ABA as a
treatment for individuals with behavioral disorders.
4. FOUNDATIONAL PRINCIPLES OF ABA

ABA is a conceptual framework from which intervention approaches
and teaching strategies are derived. In addition to the core tenets described
above, ABA practices are consistent with specific foundational principles,
which help to differentiate aspects of an ABA intervention, relative to other
approaches. The foundational principles of ABA, embedded in the concep-
tual framework, revolve around operant contingencies (Baer et al., 1968;
Catania, 2007; Cooper et al., 2007). That is, behavior occurs following a
stimulus, and the behavior is then followed by a consequence, which that
increases or decreases the likelihood of the behavior occurring again (Baer
et al., 1968). For example, if a mother gives her toddler a choice between
goldfish and raisins for a snack, and the toddler points to the goldfish, the
mother is likely to then give the toddler the goldfish. In this scenario,
the stimulus (antecedent) is the presented choice, the toddler pointing is
the behavior, and the mom giving the toddler the goldfish is the conse-
quence. Basic operant learning theory states that behavior is elicited by a pre-
ceding event, and maintained by what occurs immediately following the
behavior. The antecedent essentially prompts the behavior, and the conse-
quence reinforces or motivates the behavior. By receiving goldfish in
response to pointing, the toddler has learned that by pointing, he/she can
acquire a desired item, and he/she is more likely to point in pursuit of a
desired item in the future. Operant learning is centralized around creating
an association between the stimulus and the behavior through reinforce-
ment. This antecedent-behavior-consequence sequence is referred to as
the three-term contingency and is utilized in both targeted skill acquisition
and management of challenging behaviors (Cooper et al., 2007; Foxx,
1982a, 1982b; Umbreit et al., 2007). The following section discusses tech-
niques of ABA, including the three-term contingency, utilized in each
application of ABA, behavioral deficits, and behavioral excesses.
5. USE OF ABA PRINCIPLES IN SKILL ACQUISITION

(BEHAVIORAL DEFICITS)
One primary utility of ABA is targeted skill acquisition through effec-
tive instruction. The sequence of events (antecedent-behavior-consequence)
is viewed as a complete learning trial used in teaching a targeted skill. From
this approach, a variety of skills and behaviors can be taught. ABA has effec-
tively been used to teach skills across several domains of development,
including imitation, play skills, receptive and expressive language, cognition,
and motor skills, which is why it is often delivered in a comprehensive and
effective early intervention model (Dawson et al., 2009; Lovaas, 2002;
Rogers & Dawson, 2010).
5.1 Additional Techniques in Using ABA as an Effective

Instructional Tool
Foundations in operant learning theory, such as the antecedent-behavior-
consequence sequence described above, allow for ABA to be implemented
as a highly effective instructional tool. These basic concepts of behavioral
reinforcement are also used in other ABA techniques that are highly effective
in targeted skill acquisition. These techniques include effective prompting,
behavioral shaping, behavioral chaining, and effective use of consequences
(Table 2).
When teaching a new skill, effective prompting is critical in eliciting the
desired behavior or response (Foxx, 1982a). Often, the skill does not exist
in the individual’s repertoire, or if it does, it is not emitted consistently or
appropriately. Effective prompting enables for the targeted behavior to
occur, and then be reinforced. As the behavior is reinforced, learning occurs,
and prompting becomes less necessary, as the behavior is built into the in-
dividual’s repertoire. Prompts are gradually faded as the association between
the stimulus and behavior becomes stronger through reinforcement of the
behavior, in order to decrease the child’s dependency on the prompts
(Cooper et al., 2007; Foxx, 1982a).
Behavioral shaping is another useful technique in targeted skill acquisi-
tion, because it is not expected that a child produce the most developed iter-
ation of a desired skill or behavior immediately when instruction begins
(Cooper et al., 2007). Rather, behaviors and skills often begin as close ap-
proximations of the final iteration of the well-developed skill or behavior.
For example, in teaching language, children often begin by producing parts
of words, or even single sounds in a word, rather than the whole word
Table 2 Key Terminology
Prompts Additional antecedent stimuli that elicit the target behavior

(e.g., verbal, gestural/modeling, or physical)
Shaping Use of differential and strategic reinforcement of close
approximations of a targeted behavior as the behavior is
taught to its fullest iteration
Chaining Targeted teaching of sequential behaviors from which each
reinforcement within the chain serves as the stimulus for
the next behavior within the chain
Differential Selective reinforcement of response behaviors
reinforcement
Three-term Contingency
Antecedent Stimulus or event preceding a behavior
Behavior Behavior that is emitted in response to stimulus (antecedent)
Consequence Stimulus that immediately follows a behavior that results in
increased (reinforcement) or decreased (punishment)
likelihood of the behavior occurring again
Functions of Behavior
Access Positive social (access to attention) or tangible reinforcement
(access to items/activities)
Escape Avoiding or ending an aversive stimulus such as unwanted
attention or nonpreferred task
Automatic Behaviors that directly produce own reinforcement value,
such as self-stimulatory behavior or stopping an aversive
stimulus (e.g., loud noise)
Catania (2007) and Cooper et al. (2007).
(e.g., “wa” rather than “water”). Behavioral shaping involves reinforcing

such approximations, while continuing to prompt the final iteration, in
this case the whole word. Reinforcing close approximations through to
the most developed iteration while using prompting, provides scaffolding
for the child and allows them to be successful in their early attempts.
Often, targeted skills and behaviors involve complex, multisequenced
steps. Chaining can be especially useful in conjunction with task analyses,
which systematically break down a skill step-by-step. Behavioral chaining
involves linking multiple steps together to teach a new skill, as well as stra-
tegic prompting and reinforcement of each step along the way (Catania,
2007; Cooper et al., 2007). In behavioral chaining, one step is targeted for
independence at a time, and prompting is used for all other steps in the
chain. When the child becomes independent with a step, prompting for that
step is faded, and the next step in the sequence is targeted. This procedure is
continued until each step in the sequence can be completed independently.
Reinforcement is delivered throughout the chain to ensure learning of each
step, and prompting is used strategically and then faded, in order to ensure
independence and generalization of the learned skill to other contexts
(Cooper et al., 2007). Chaining can be done in a forward sequence, teaching
the first step and moving to the next, or a backward sequence, beginning
with teaching the last step first and working backward (Catania, 2007;
Cooper et al., 2007). Behavioral chaining is effective for teaching self-care
skills, language, and many other multistep skills (Rogers & Dawson, 2010).
When applied in any context, these techniques are highly effective tools
for basic instruction. When applied within the context of a comprehensive
and early developmental intervention, these techniques facilitate and sup-
port the growth of skills across multiple domains of development. Collec-
tively, these techniques can effectively keep the child engaged during the
intervention session, teach for long-term acquisition of skills, and address
many areas of development that are affected in the presence of a develop-
mental and neurogenetic disorder.
6. USE OF ABA TECHNIQUES IN ADDRESSING

CHALLENGING BEHAVIOR (BEHAVIORAL EXCESSES)
From a behavior analytic perspective, three main functions drive
behavior: access to a desired item, avoidance of something aversive, or auto-
matic reinforcement (Umbreit et al., 2007). Functional assessments are used
in determining the maintaining function of a behavior, based on the ante-
cedent, or stimulus, and the consequence, or what happens after. For
example, property destruction and elopement (escape) could be considered
escape-maintained behaviors, if the child engages in the behavior when pre-
sented with a task, and if the demand of the task is subsequently removed after
the behavior is emitted, such as in the event of a time-out. In conjunction
with determining the motivation and function of the behavior, appropriate
replacement behaviors, such as requesting a break from work, are also taught
and reinforced using positive behavior supports, in order to ultimately reduce
the challenging behavior (Foxx, 1982a, 1982b; Umbreit et al., 2007). Mod-
ifications of antecedents in the environment are also utilized in addressing
challenging behavior. These include providing positive social reinforcement
or attention for appropriate behaviors, adapting demands to optimize success
on tasks, teaching functional communication, and providing choices between

tasks (Rogers & Dawson, 2010). This approach of ABA is often used to
target challenging behaviors associated with ASD, such as stereotypy and
self-injurious behavior (Hanley, Iwata, & McCord, 2003). A comprehensive
discussion of functional behavioral assessment is beyond the scope of this
paper, but can be found elsewhere (Umbreit et al., 2007).
Addressing behavioral and skill deficits, and behavioral excesses of chal-
lenging behavior are main approaches that utilize ABA principles and that crit-
ical to target in tandem, as behavioral excesses often impede learning of
targeted skill deficits (Foxx, 1982a, 1982b). It is important, however, to
recognize that ABA is a larger framework from which these approaches
have emerged. What is known regarding the application of ABA in address-
ing behavioral deficits and behavioral excesses is the culmination of several
decades of basic research and application of the described techniques to indi-
viduals who could benefit from them. ABA has had a considerable evolution
since its inception, and many shifts within the field have been to the benefit of
individuals with developmental and intellectual disabilities, particularly ASD.
7. EVOLUTION OF ABA IN THE UNITED STATES

ABA began over 60 years ago, as a subfield of psychology that focused
mainly on the experimental analysis of behavior in a laboratory setting
(Lerman et al., 2013). It grew from foundations of operant learning theory
and behaviorism into an applied approach with various utility (Baer et al.,
1968). ABA began to be applied as a method of behavioral change for adults
and children with disabilities, as well as adults with psychiatric disorders, and
in particular, with individuals with autism (Lerman et al., 2013; Lovaas et al.,
1965, 1966; Lutzker & Whitaker, 2005; Wolf et al., 1964). ABA as a field has
commendably and effectively evolved over time to greater emphasize the
individual being treated, as well as positive behavioral approaches (Cooper
et al., 2007; Lerman et al., 2013). Many intervention models that utilize
core ABA principles shown to be effective in early studies, have been devel-
oped out of the necessity for more sensitive, person and child-focused, and
positive support-oriented approaches that are in the best interest of the in-
dividual. This evolution has resulted in a modern conceptualization of
ABA as a beneficial and effective approach in the clinical and special educa-
tion settings (Dunlap, Kern, & Worcester, 2001). Although ABA has grown
across many different intervention models, it has remained somewhat
limited in its scope of utility across a variety of developmental disorders.
7.1 Discrete Trial Training

Much of the work conducted by Lovaas, utilizing the three-term contin-
gency learning trials, resulted in behavioral intervention for individuals (pri-
marily with autism) consisting of mass-trial learning. This became known as
“discrete trial training,” (DTT) where each three-term contingency trial was
considered a discrete trial. In the “Lovaas approach” (Lovaas, 2002), mass
learning trials were often delivered at a table, and performed in conjunction
with application of the three-term contingency to manage challenging be-
haviors, such as aggression, stereotypy, and self-injury (Lovaas, 1987, 2002).
In the seminal paper presenting the efficacy of this method in treating
autism, effects were shown to improve autism symptomology at a dosage
of 40 h per week for 2 years (Lovaas, 1987). Outcomes also indicated an
improvement in IQ, problem behavior, and educational functioning.
Although this particular efficacy study was considered controversial for
many different reasons, it shifted treatment in the direction or early and
intensive (over 25 h per week) behavioral intervention for children with
ASD, in the hopes of improving their overall developmental trajectories
(Lovaas, 1987, 2002).
7.2 Pivotal Response Training

Although there have been many variations of early behavioral interven-
tions for treating autism that utilize ABA principles, one particularly
notable one is pivotal response training (PRT; Koegel, Koegel, Koegel,
& Vernon, 2014; Koegel & Koegel, 1988; Schreibman, Stahmer, & Pierce,
1996). PRT developed from the more traditional ABA approach of DTT,
where the three-term contingency, creating associations between stimulus,
behavior, and consequence, is delivered in a mass-trial manner. PRT uti-
lized the same ABA principles embedded in the three-term contingency,
but added the component of increasing child motivation. Though initially
implemented as an intervention targeting communication abilities in chil-
dren with ASD, application extended to academics, self-care, and social
skills (Koegel et al., 2014). The primary shift PRT made from more tradi-
tional styles of ABA-based interventions, such as the Lovaas method, was to
focus on child motivation for learning and to implement in a more natural-
istic way than mass discrete trials. PRT shifted focus toward child motiva-
tion to increase engagement from children, and therefore expand learning
potential. Motivation was increased by following the child’s lead and
choice of activity, using higher positive affect, embedding learning trials
into naturalistic play, and implementing natural reinforcement (e.g., giving

a toy for an appropriate request; Koegel et al., 2014). The collective shift
toward these approaches has shown efficacy in addressing areas of skill def-
icits for children with ASD in a relatively smaller dosage of direct therapy
(Koegel et al., 2014; Simpson, 2005). Although these approaches have
been used across different intervention methods, PRT was one of the
earliest shifts from traditional ABA techniques of DTT, serving as a foun-
dation for further development and evolution of ABA-based intervention
models.
7.3 ABA in Special Education Settings

In addition to influencing the development of intervention models for chil-
dren with ASD, ABA has also been significantly influential on special edu-
cation throughout its evolution (Dunlap et al., 2001). Primary contributions
have been described in the development of antecedent-based interventions
for students, and curriculum-based assessment (Dunlap et al., 2001). In
conjunction with the shift of focus in ABA from management consequences
to address challenging behavior toward positive behavioral supports, this
particular shift was observed in the special education, as instructors began
adapting the environment to better support students’ learning needs. Evi-
dence from the evolution of traditional ABA approaches began to indicate
that reactive procedures resulted in greater use of punishment procedures,
greater intensity of such procedures, less person-focused, and resulted in
poorer generalization of learned skills to other contexts (Dunlap et al.,
2001). Conversely, an antecedent-based approach adapts the environment
in a way that is conducive for minimizing triggers of problem behavior.
Evidence indicates that these types of approaches, in conjunction with inter-
vention to target skill acquisition, are more effective and long-term, as the
adapted environmental contingencies along with less problematic behavior
enables the student to better learn (Bloom, Iwata, Fritz, Roscoe, & Carreau,
2011; Dunlap et al., 2001).
Throughout the history and evolution of ABA as a field, individuals with
ASD have been the primary population of focus, particularly in the area of
early intensive intervention (Koegal et al., 2014; Lovaas et al., 1965, 1966).
Although this shift into special education has benefited many students
without ASD, and potentially those with neurogenetic disorders, its applica-
tion in this setting has also been somewhat limited in scope. Generally, the
focus in this setting has been on managing problematic behaviors, and has
been primarily implemented with individuals with behaviorally diagnosed
disorders. Despite its significant evolution, ABA has not entered the main-
stream of early intensive intervention for children with developmental dis-
orders that are not on the autism spectrum. Below, we discuss the current
status of ABA and some barriers to its use with individuals with neurogenetic
disorders.
8. CURRENT STATUS
Currently, foundational ABA principles are heavily utilized in the spe-
cial education setting (Bloh & Axelrod, 2008; Dardig et al., 2005; Dunlap
et al., 2001), and are shown to be effective in increasing learning as well
as managing challenging behavior that can be a barrier to academic and func-
tional achievement (Bloom et al., 2011; Sutherland, Lewis-Palmer, Stichter,
& Morgan, 2008). ABA has become most widely used in its application to
treat young children with ASD (Axelrod, McElrath, & Wine, 2012;
Reichow & Wolery, 2009; Rosenwasser & Axelrod, 2001). Additionally,
intervention models that utilize ABA as the underlying foundational princi-
ples are stated to be the most effective interventions for targeting behavioral
deficits and areas of challenge for children with ASD (Dawson et al., 2009;
Koegel et al., 2014; National Autism Center, 2009; National Research
Council, 2001; Reichow & Wolery, 2009; Simpson, 2005).
Regardless of which setting ABA is applied in, classroom, clinic, or home,
the majority of evidence regarding the utility of ABA for intellectual and
developmental disabilities focuses on behaviorally diagnosed disorders, such
as attention deficit hyperactivity disorder (ADHD), emotionalebehavioral
disorders, and autism (see Axelrod et al. (2012) for review; Bicard & Neef,
2002; Cook, Bradley-Johnson, & Merle Johnson, 2014; Flood, Wilder,
Flood, & Masuda, 2002; Neef, Bicard, & Endo, 2001; Sutherland et al.,
2008; Wolery, Barton, & Hine, 2005). Federal legislation of the Individuals
with Disabilities Education Improvement Act (IDEIA; P.L. 1008-446,
IDEA) was enacted in 2004, increasing the use of ABA in the school setting.
Specific applications of ABA were outlined for functional assessments of
problematic behavior, behavior management, and implementation of posi-
tive behavior supports for students (Bloh & Axelrod, 2008). Although the
use of underlying principles in ABA in the classroom setting has increased,
it again has been targeted toward the management of problematic behavior,
often co-occurring with learning disabilities, emotionalebehavioral disor-
ders, and developmental disorders such as autism and ADHD (Axelrod
et al., 2012; McKenna, Flower, Kyung Kim, Ciullo, & Haring, 2015;
Sutherland et al., 2008; Wolery et al., 2005).
For students with ADHD, ABA has been shown to be effective in man-
aging task-related behavior, impulsivity, and academic responding (Bicard &
Neef, 2002; Cook et al., 2014; Flood et al., 2002; Neef et al., 2001). Stra-
tegies shown to be effective for managing these behaviors for students with
ADHD include effective prompting, reinforcement, and adjustment of
environmental contingencies to enhance student learning. These strategies,
along with functional assessment of problematic behavior, have also been
shown to be effective for students with emotionalebehavioral disorders,
and learning disabilities (McKenna et al., 2015; Sutherland et al., 2008). Stu-
dents with ASD have particularly benefited from the application of ABA
across settings to enhance learning and address challenging behavior. ABA
has also been utilized as an effective instruction tool across a multitude of set-
tings for students with ASD (Axelrod et al., 2012; Bloh & Axelrod, 2008;
Dunlap et al., 2001; Rosenwasser & Axelrod, 2001; Wolery et al., 2005).
Culmination of continued investigation into the utility of ABA for behav-
iorally diagnosed disorders, particularly ASD has culminated in ABA being
the most widely implemented early and intensive behavioral intervention
for children with ASD (Koegel et al., 2014).
Evidence clearly supports the efficacy of the application of ABA for stu-
dents and children with behaviorally diagnosed disorders. Some argue that
the focus within the classroom setting for students with behaviorally diag-
nosed disorders comes at the expense of the generalization of ABA to general
education settings as well (Axelrod et al., 2012). Although this may be true to
some extent, there is an additional and potentially greater expense, for the
lack of application of ABA as a comprehensive behavioral intervention to
children with neurogenetic disorders across multiple settings, including the
classroom. The lack of application of ABA to neurogenetic populations is
arguably the result of multiple factors, including policy, restricted perspec-
tives of research designs, and limited efficacy studies with these populations.
9. BARRIERS TO THE USE OF ABA IN NEUROGENETIC

SYNDROMES
9.1 Policy
Change in policy has been an impetus for increased application of
ABA to individuals with behaviorally diagnosed disorders, particularly
ASD (Axelrod et al., 2012; Shiozawa, 2014). As discussed previously, change

in educational policy has led to the increased implementation of ABA in the
educational setting as an effective approach to manage behavior (Axelrod
et al., 2012). Legislation around insurance has also facilitated the increased
application of ABA in the clinical setting (Shiozawa, 2014; National Confer-
ence of State Legislatures, http://www.ncsl.org/research/health/autism-
and-insurance-coverage-state-laws.aspx). Because ABA is shown to be a
highly effective treatment for ASD, legislation has shifted toward mandating
insurance coverage for ABA treatment. As of 2014, five states and the Dis-
trict of Columbia have statutes that may require limited coverage of treat-
ment services for ASD through insurance, with 32 states specifically
requiring insurance coverage for ASD, and 21 of these states explicitly listing
coverage of ABA services (National Conference of State Legislatures). It is
important to note that specifics of coverage vary across states, and coverage
for ABA services specifically, is not always explicitly stated. These policies
are potentially beneficial for families of children with ASD who are seeking
early and intensive intervention, but cost of services, estimated to be around
$50,000 per year (National Conference of State Legislatures), may still be a
consideration to these families, even with some amount of coverage. How-
ever, with a strong evidence base for the benefit of early and intensive ABA
intervention for optimal outcomes for children with ASD, families may be
willing to pursue ABA services despite the cost.
For individuals and families with a neurogenetic disorder, cost as well as a
lack of evidence may be a barrier to pursuing ABA services as an early and
comprehensive intervention approach. Although some states do have legis-
lation stipulating coverage for genetic conditions (National Conference of
State Legislatures), ABA services are not explicitly stated, presenting a barrier
to families seeking this type of intervention. Families of children with neuro-
genetic disorders may choose to pursue ABA regardless of the cost if a sub-
stantial evidence basis for the benefit of these services existed; however,
research on ABA for neurogenetic disorders has severely lagged behind
research on ABA for ASD. Thus, further research in this area is critical
and necessary in order to provide this evidence base, which ideally would
in turn, increases access to services for these individuals.
9.2 Single-Subject Methodology

Another potential barrier to the widespread use of ABA for nonbehaviorally
diagnosed disorders, and potentially a factor contributing to the lacking ev-
idence of large group studies on ABA for neurogenetic disorders, is the
reliance on single-subject research methodology (Lutzker & Whitaker,

2005). Single-subject research methodology grew from the behavioral tradi-
tion that developed into ABA (Baer et al., 1968; Wolery, 2013). In this
methodological approach, an individual serves as his/her own control, as
intervention methods and environmental contingencies are evaluated for ef-
ficacy in increasing a desired behavior (i.e., teaching a skill), or decreasing
challenging behavior (Gast, 2010). Although single-subject research is
incredibly useful for applying ABA approaches to individuals with neuroge-
netic disorders, it is not a widely accepted methodology among group re-
searchers, which has potentially inhibited the wider use and acceptance of
ABA for different populations (Lutzker & Whitaker, 2005).
Single-subject methodology has enabled the growth of ABA as a field, as
new studies provided information regarding improvement of interventions
for individuals with developmental and intellectual disabilities throughout
its evolution (Dunlap et al., 2001; Lutzker & Whitaker, 2005). Additionally,
the dynamic nature of single-subject methodology creates a highly useful
approach in intervention research. Because it is grounded in the behavioral
tradition, it takes into account the individual, and interventions are thus,
highly individualized (Baer et al., 1968; Lutzker & Whitaker, 2005; Wolery,
2013). This is an optimal approach for interventions, because they can better
serve the precise behavioral needs of the individual. Furthermore, results
from single-subject intervention methods can be interpreted precisely
within an individual, or across few individuals, rather than analyzed and
interpreted across the mean of many individuals, as occurs in group design
research (Wolery, 2013).
Group researchers cite the issue of generalizability in the event of
single-subject methodology (Kratochwill et al., 2010; Lutzker & Whitaker,
2005). Experts in single-subject methodology acknowledge the risk of
interpreting only one single-subject study, and instead argue for systematic
replication of studies as well as the synthesis of many single-subject studies to
assess generalizability (Wolery, 2013). Additional contention between
group research methodology and single-subject methodology lies in the
application of statistical analyses to single-subject studies, which rely on
visual analysis (Gast, 2010). Although these issues are beyond the scope of
the present paper, it is noted that use of statistical analysis provides potential
utility to single-subject methodology (Kratochwill et al., 2010), but single-
subject methodology should not necessarily be eschewed due to its use of
visual analysis.
This ongoing tension between these two methodologies has poten-

tially limited the application of ABA for certain groups of individuals
on a larger scale. Although there have been large-scale studies showing
the efficacy of early and intensive behavioral intervention (Dawson
et al., 2009; Koegel et al., 2014; Lovaas, 1987; Mohammadzaheri, Koegel,
Rezaee, & Rafiee, 2014), these have been with children with ASD and
started with few individuals (Lovaas et al., 1965, 1966; Rogers et al.,
2006). For ABA to become widely available as an early and intensive
behavioral intervention for children with neurogenetic disorders, it
may be critical to consider important first steps toward demonstrating effi-
cacy, as single case studies, moving toward large randomized-controlled
trials.
9.3 Need for Innovation

Another barrier to the application of ABA as an early and intensive
behavioral intervention is the lack of innovation in this direction. Prin-
ciples of ABA have been applied to populations with neurogenetic
disorders; however, this is almost exclusively applied to behavior manage-
ment and not toward the comprehensive development of skills across a
variety of developmental domains (Kennedy, Caruso, & Thompson,
2001; Schroeder et al., 2001). ABA as a comprehensive, early, and intensive
behavioral intervention appears to have become conceptualized as only
or most relevant to children with ASD (Koegel et al., 2014; Rogers &
Dawson, 2010). Given that individuals with neurogenetic disorders, such
as SmitheMagenis syndrome (SMS), Williams syndrome (WS), Pradere
Willi syndrome (PWS), and DS, all struggle deficits in many domains simi-
larly affected in ASD. Thus, early and intensive behavioral intervention to
remediate deficits in areas, such as language, communication, motor skills,
impulsivity, social behavior, and stereotypy, would likely be highly effec-
tive for these individuals as well. As mentioned previously, an even greater
disservice to individuals of neurogenetic populations regarding early and
intensive behavioral intervention, is that families often receive these diag-
noses at or relatively close to birth, optimizing the window for shaping
developmental trajectories with early intervention (Baker & Feinfield,
2003; Fidler & Nadel, 2007). Because of its individualized approach,
ABA offers unique utility and application for children with any develop-
mental disorder when developmentally appropriate targets are selected
and addressed.
10. ABA IN NEUROGENETIC SYNDROMES

10.1 Current Applications
Individuals with neurogenetic syndromes show complex behavioral
profiles of language, cognition, and behavior. Many neurogenetic disorders
are characterized by deficits in language, motor, cognition, social behavior,
and adaptive functioning, as well as severe problem behavior (Tassé &
Lecavalier, 2000). Before exploring the potential utility of ABA for this pop-
ulation, an important consideration should be noted. It is possible that some
presentations of a given neurogenetic phenotype may not necessarily be
straightforwardly addressed utilizing an ABA approach, or rather, may
require a very targeted and intensive approach to address. Existing research
utilizing an ABA approach in addressing behaviors associated with certain
neurogenetic disorders is limited in quantity and scope. Further research is
necessary to evaluate the extent to which ABA can be effectively imple-
mented to address problematic and highly pronounced areas of a given
phenotype. However, use of ABA in behaviorally disordered populations,
as well as existing evidence on the use of ABA in neurogenetic disorders sup-
ports the notion that problematic areas of a given phenotype can be highly
modifiable using an ABA approach.
10.2 SmitheMagenis Syndrome

SmitheMagenis is a genetic syndrome caused by deletion on chromosome
17 (Juyal et al., 1996). It occurs in approximately 1 of every 25,000 births
and is characterized by a unique phenotype (Allanson, Greenberg, & Smith,
1999; Greenberg et al., 1991). SMS is somewhat under-researched, but ev-
idence supports a behavioral profile characterized by moderate intellectual
disability and difficulty with attention, impulsivity, short-term memory,
and sequential processing (Dykens, Finucane, & Gayley, 1997; Os orio
et al., 2012). It is also characterized by areas of relative strength in long-
term memory, expressive language, and spatial perception (Dykens et al.,
1997; Garayzabal et al., 2011). Problem behaviors associated with SMS
include self-injury, aggression, hyperactivity, mood fluctuations (Arron,
Oliver, Moss, Berg, & Burbidge, 2011; Taylor & Oliver, 2008), and stereo-
typical behavior (Dykens et al., 1997). These problem behaviors are quite
serious in nature, and therefore, have been the target of much empirical
investigation in SMS (Langthorne & McGill, 2012; Taylor & Oliver, 2008).
Despite the prevalence of problematic behavior in SMS, little evidence
of function-based interventions within an ABA framework exists. Much
evidence explores the nature and function of problem behavior in SMS,

including aggression and self-injury (Arron et al., 2011; Sloneem, Oliver,
Udwin, & Woodcock, 2011; Taylor & Oliver, 2008). Research shows
that much of the problem behavior in SMS is maintained by environmental
contingencies (Sloneem et al., 2011), social reinforcement (Taylor & Oliver,
2008). Despite the evidence of maintaining functions of problem behavior
in SMS, little has been done to investigate the efficacy of function-based in-
terventions for this population. This is surprising given that problematic be-
haviors such as self-injury and aggression are not only impeding to overall
daily functioning, but also potentially harmful to oneself and/or others,
and would typically be the first target of a function-based intervention
(Umbreit et al., 2007). Additionally, other aspects of the SMS behavioral
phenotype, such as expressive language, impulsivity, and attention could
also be targeted through ABA-based intervention by modifying environ-
mental contingencies, prompting and shaping of expressive language targets,
and reinforcement of appropriate (i.e., nonimpulsive) behavior. These types
of interventions could be especially effective in utilizing social reinforcement
for desired behavior and targeted skill acquisition, given that attention has
been shown to be a strong maintaining function of behavior (Arron et al.,
2011; Sloneem et al., 2011; Taylor & Oliver, 2008).
10.3 PradereWilli Syndrome

PradereWilli is another neurogenetic syndrome with a distinctive behav-
ioral phenotype. PWS is caused by deletion or disomy of chromosome 15
(Ledbetter et al., 1981; Nicholls, Knoll, Butler, Karam, & Lalande, 1989)
and it occurs in approximately 1 in 10,000 live births (Holm, 1981). The
physical phenotype is characterized by short stature, hypotonia, obesity,
and hypogonadism (Holm et al., 1992). The behavioral phenotype is char-
acterized by mild to moderate intellectual disabilities, food preoccupation
(and hyperphagia), and externalizing behavior problems including self-
injury, behavioral outbursts, and noncompliance (Hiraiwa, Maegaki, Oka,
& Ohno, 2007; Stokes & Luiselli, 2009). Hyperphagia is a pronounced
area of difficulty for individuals with PWS, and one that may not be as
directly addressed by ABA intervention alone. However, adapting environ-
mental contingencies has the potential to address this area of concern, and
further research should investigate the utility of an ABA approach for
food preoccupation in PWS.
Prior research on function-based interventions to address self-injury in
PWS, though limited has shown promising results. Functional assessment
determined that self-injury was maintained by attention, escape, and

automatic reinforcement, and a function-based intervention addressing
environmental contingencies and teaching effective replacement behav-
iors (i.e., functional communication) was effective at eliminating the
self-injurious behavior (Stokes & Luiselli, 2009). This example is ex-
tremely limited in that it was a single case study, but provides support
for contingency and function-based interventions in addressing some
problem behavior associated with PWS. It may also have implications
for intervening on similar behavior in other neurogenetic syndromes,
such as self-injury in SMS.
Beyond self-injury, research has shown there are other areas of func-
tioning that could benefit from ABA-based interventions for individuals
with PWS. For example, evidence indicates that there is overlap in social
responsivity between individuals with PWS and individuals with ASD
(Dimitropoulos, Ho, & Feldman, 2013). There have been many ABA-
based social interventions for children with ASD, as that is a core area of
deficit for diagnostic categorization (DSM-V; 2012). Although some evi-
dence supports within-syndrome variability of social behavior in PWS
(Dimitropoulos, Ferranti, & Lemleer, 2013), this is nevertheless an area
for potentially targeted intervention utilizing operant foundations along
with effective prompting, shaping, and reinforcement to develop appro-
priate social behavior (Cooper et al., 2007).
Language and pretend play are additional areas of challenge in PWS
that could potentially be targeted with ABA-oriented interventions.
Evidence indicates that each of these areas is an area of challenge for
individuals with PWS, and pretend play in particular is consistent with
abilities in ASD (Dimitropoulos, Ferranti et al., 2013; Zyga, Russ,
Ievers-Landis, & Dimitropoulos, 2015). Expressive language (communica-
tion and requesting in particular) and play skills have each and often
simultaneously targeted through ABA-based interventions for children
with ASD (Dawson et al., 2009; Koegel et al., 2014; Rogers & Dawson,
2010). Naturalistic teaching utilizing ABA instructional methods have
been shown to increase both expressive language, communication, and
play skills in ASD (Dawson et al., 2009; Koegel et al., 2014; Rogers &
Dawson, 2010). Therefore, because there is already an evident basis
for improving outcomes in these areas for children with ASD, it is likely
similar approaches targeting similar areas of deficit in a neurogenetic
disorder (i.e., PWS) would have a beneficial outcome for these children
as well.
10.4 Williams Syndrome

WS is caused by a microdeletion on the seventh chromosome (Hillier et al.,
2003), and is considered to be relatively rare, occurring in 1 in 7500 live births
(Strømme, Bjømstad, & Ramstad, 2002). The WS behavioral phenotype is
characterized by mild to moderate intellectual disability or learning disability,
hypersociability, relative strengths in auditory processing and expressive lan-
guage, and relative weakness in visuospatial processing and fluid language
abilities (Mervis et al., 2000). Areas of problem behavior observed in WS
include anxiety, obsessions and compulsions, attention difficulties, and social
difficulties (Fidler, Hodapp, & Dykens, 2000; Klein-Tasman, Lira, Li-Barber,
Gallo, & Brei, 2015; Klein-Tasman & Mervis, 2003; Leyfer, Woodruff-
Borden, Klein-Tasman, Fricke, & Mervis, 2006; Mervis & Klein-Tasman,
2000). Although these types of behavioral problems are arguable less severe
than self-injury and aggression, they are observed across contexts (i.e., school
and home) and also at sometimes clinical levels in children with WS, indicating
an overall debilitating quality to the behavior (Klein-Tasman et al., 2015).
A main tenant of ABA is to address behaviors in order to improve the qual-
ity of life for an individual. Given the debilitating nature of problem behaviors
associated with WS, such as anxiety, it is extremely likely that ABA-targeted
interventions could be beneficial in reducing the behaviors and ultimately
improving the quality of life for individuals with WS. For social difficulties,
strategies such as social stories (Gray, 1998; Hutchins & Prelock, 2013) and
teaching effective communication have shown to be helpful in addressing
similar challenges in ASD (Hutchins & Prelock, 2013; Rispoli, Camargo,
Machalicek, Lang, & Sigafoos, 2014) and could be of potential benefit for
individuals with WS. Anxiety is another phenotypic area that is highly pro-
nounced for individuals with WS, and therefore may require a more inten-
sive ABA approach. For difficulties related to anxiety, evidence has shown
support for systematic exposure therapy for individuals with ASD (Reaven
et al., 2014) and is another approach that could have potential implications
for reducing anxiety, compulsions, and obsessions for individuals with WS.
It is worth further investigation into the malleability of anxiety in WS using
an ABA approach, as improving this highly affected area would translate to an
improved quality of life for individuals with WS.
10.5 Down Syndrome

DS is a neurogenetic disorder that occurs in approximately 1 in approxi-
mately 700 live births (Canfield et al., 2006). It is the most common genetic
cause of intellectual disability (Canfield et al., 2006), and as described previ-

ously, is associated with a specific behavioral profile (Fidler, 2005). The DS
behavioral phenotype is characterized by areas of relative challenge that could
benefit from comprehensive behavioral intervention, such as expressive
language difficulties, delayed motor development, instrumental communica-
tion, and problems with attention (Fidler, 2005; Fidler, Philofsky, Hepburn,
& Rogers, 2005; Mundy, Kasari, Sigman, & Ruskin, 1995). DS is also char-
acterized by areas of relative strength in the areas of social relatedness, imita-
tion skills, and receptive language (Fidler, 2005). Although these approaches
have been utilized by some to target certain skills in DS, existing evidence is
severely limited, and has also focused on limited areas of challenge in DS
(Bauer & Jones, 2015; Feeley & Jones, 2006; Feeley, Jones, Blackburn, &
Bauer, 2011).
Although a few ABA-oriented interventions have effectively targeted
requesting and verbal imitation in children with DS (Bauer & Jones,
2015; Feeley et al., 2011), others utilizing components of ABA have shown
less of an effect on intervention outcomes for children with DS compared to
children with other neurodevelopmental disorders (Fey, Yoder, Warren, &
Bredin-Oja, 2013; Yoder, Woynaroski, Fey, & Warren, 2014; Yoder &
Warren, 2002). In one study, this differential outcome specific to DS was
attributed to decreased interest in objects (Yoder et al., 2014). Collectively,
this research suggests that further investigation into the application of ABA-
oriented interventions is warranted, and also that these interventions should
utilize evidence from the comprehensive DS behavioral phenotype in an
intervention approach.
Children with DS show poor object knowledge early on in the develop-
mental trajectory (Fidler, Hepburn, Mankin, & Rogers, 2005; Fidler,
Philofsky, et al., 2005; MacTurk, Vietze, McCarthy, McQuiston, & Yarrow,
1985; Yoder et al., 2014), which compromises their ability to communicate
around objects (Fidler, Philofsky, et al., 2005), as well as their goal-directed
behavior (Fidler, Hepburn, et al., 2005). Further evidence also shows that
children with DS engage in social behavior to avoid object-related tasks
and problem-solving (Fidler, Philofsky et al., 2005; Kasari & Freeman,
2001; Moore, Oates, Hobson, & Goodwin, 2002). Although social related-
ness is an area of relative strength for individuals with DS, it may in fact be
compromising learning opportunities related to objects and problem-solving
tasks (Wishart, 1993, 2007).
Therefore, an optimal ABA-oriented intervention should take into ac-
count the combination of these aspects associated with the DS behavioral
phenotype in addressing areas of challenge. For example, a comprehensive

approach might target instrumental (e.g., object-related) requesting, uti-
lizing effective prompting hierarchy, shaping, and high rates of social
reinforcement, while also teaching functional use of objects through
imitation. This approach would potentially target challenges related to
instrumental requesting, use of objects, and depleted interest in objects,
while capitalizing on the relative strength in social relatedness through
reinforcement. With this approach, there are multiple functional targets
taught simultaneously, including knowledge and interest in objects, tar-
geting not only cognition, but a foundational mechanism of language
development (object interaction; object knowledge), which has been
shown to be affected in language interventions previously (Yoder et al.,
2014).
An ABA-oriented approach is particularly ideal for targeting these areas
in DS for several reasons. First, the three-term contingency is optimal for
teaching these types of skills utilizing social reinforcement as the conse-
quence. Given the relative strength in social relatedness observe in DS
(Fidler, 2005; Fidler, Most, Booth-LaForce, & Kelly, 2006), utilizing social
reinforcement would likely create a strong association between the ante-
cedent (e.g., encountering a desired object), and the desired behavior
(e.g., requesting for that object with coordinated gaze and vocalization).
Additionally, because children with DS show relative strengths in imitation
skills (Fidler, 2005; Wright, Lewis, & Collis, 2006), effective prompting
would likely result in efficient learning of target behaviors, as children
with DS would readily imitate the target behavior. An additional feature
of ABA that would be especially effective for targeted intervention in DS
is differential reinforcement (Catania, 2007). Children with DS frequently
engage in social behavior as a mechanism to avoid goal-directed and prob-
lem-solving tasks (Fidler, Philofsky, et al., 2005; Kasari & Freeman, 2001).
While social reinforcement can be highly beneficial within the three-term
contingency to create learned associations between stimulus and target
behavior, implementing social reinforcement of escape (e.g., task-related)
behavior could have a contraindicated effect. This use of social reinforce-
ment would effectively shape the escape behavior to the extent that chil-
dren with DS would learn to get avoid tasks by engaging in social
behavior. Utilizing differential reinforcement, where social reinforcement
is provided for desired behaviors, but escape behaviors are not attended
to would effectively circumvent shaping escape from demands through so-
cial engagement.
There is also utility in an ABA approach to address challenging

behavior observed in individuals with DS. Although DS is regarded as a
neurogenetic disorder associated with relatively low levels of challenging
behavior compared to other neurogenetic disorders, as well as autism,
evidence is mixed and it is likely that problem behaviors are underesti-
mated in this population. Individuals with DS show difficulty with expres-
sive language (Fidler, 2005), which is an area specifically linked to increased
risk in challenging behavior (Arron et al., 2011; McClintock, Hall, &
Oliver, 2003). Additionally, a subset of children with DS, approximately
7%, has comorbid ASD (DiGuisseppi et al., 2010), which also increases
the risk of challenging behaviors, such as stereotypy and self-injury. Evi-
dence has also shown that students with DS engage in behaviors at school
that decrease their overall safety, specifically elopement (Daunhauer et al.,
2014). Many of these areas of behavioral challenges associated with DS
could benefit from the application of ABA. From an ABA approach utiliz-
ing functional behavior assessments, environmental contingencies could be
modified to increase safety, compliance, and increase on-task behavior. Spe-
cifically, these strategies may be especially effective if utilizing visual sup-
ports, which may capitalize on the area of relative strength in visual
processing abilities in DS. Evidence exploring the benefits and utility of
ABA for DS is extremely limited, and extensive research needs to be
done in this area.
11. CONCLUSIONS
ABA as a field has made great strides in improving the lives of indi-
viduals with developmental disorders in a very short time. Evidence over
the years has illustrated its utility in addressing challenging behavior that
impedes daily functioning and learning, as well as teaching functional skills
and behaviors to optimize developmental and educational outcomes.
Persistent work in this area has had a significant influence on policy, which
in turn, has improved the lives of even more individuals. This evidence and
shifts in policy, however, have been extremely limited, primarily focusing
on behaviorally diagnosed disorders. Individuals with neurogenetic disor-
ders have many of the same developmental challenges that individuals
with behaviorally diagnosed disorders, yet ABA has not been applied to
them in the same way. Individuals with neurogenetic disorders often strug-
gle with language, cognition, and challenging behavior, such as noncom-
pliance, self-injury, anxiety, and off-task behavior. A comprehensive ABA
intervention approach, targeting skill acquisition, and challenging behavior

would potentially be of great benefit to these individuals, particularly if
delivered in an early and intensive way. Future directions taken should
begin with a systematic review of ABA interventions applied to individuals
with neurogenetic disorders. Given the complex nature of behavioral
phenotypes and how they are shaped through genetic underpinnings and
interactions with the environment, systematic investigation, the utility of
ABA for individuals with neurogenetic disorders is necessary. This line
of investigation is long overdue, as ABA as a comprehensive and early
intervention approach could very likely have long-term developmental
implications for optimizing outcomes for individuals with neurogenetic
disorders.
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CHAPTER SIX

Applied Behavior Analysis for

SmitheMagenis syndrome Long-term memory Short-term memory Self-injury

Fidler, 2005). Likewise, it is characterized by areas of challenge in the areas of

2. UTILITY OF APPLIED BEHAVIOR ANALYSIS: EARLY

3. DEFINITION AND BACKGROUND

replicability and efﬁcacy of the intervention. In particular, the level change

4. FOUNDATIONAL PRINCIPLES OF ABA

5. USE OF ABA PRINCIPLES IN SKILL ACQUISITION

5.1 Additional Techniques in Using ABA as an Effective

Table 2 Key Terminology

Prompts Additional antecedent stimuli that elicit the target behavior

(e.g., “wa” rather than “water”). Behavioral shaping involves reinforcing

6. USE OF ABA TECHNIQUES IN ADDRESSING

on tasks, teaching functional communication, and providing choices between

7. EVOLUTION OF ABA IN THE UNITED STATES

7.1 Discrete Trial Training

7.2 Pivotal Response Training

into naturalistic play, and implementing natural reinforcement (e.g., giving

7.3 ABA in Special Education Settings

9. BARRIERS TO THE USE OF ABA IN NEUROGENETIC

ASD (Axelrod et al., 2012; Shiozawa, 2014). As discussed previously, change

9.2 Single-Subject Methodology

reliance on single-subject research methodology (Lutzker & Whitaker,

This ongoing tension between these two methodologies has poten-

9.3 Need for Innovation

10. ABA IN NEUROGENETIC SYNDROMES

10.2 SmitheMagenis Syndrome

evidence explores the nature and function of problem behavior in SMS,

10.3 PradereWilli Syndrome

determined that self-injury was maintained by attention, escape, and

10.4 Williams Syndrome

10.5 Down Syndrome

cause of intellectual disability (Canﬁeld et al., 2006), and as described previ-

phenotype in addressing areas of challenge. For example, a comprehensive

There is also utility in an ABA approach to address challenging

intervention approach, targeting skill acquisition, and challenging behavior

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