Intellectual Disabilities

M M Griffin, M M Burke, and R M Hodapp, Vanderbilt University, Nashville, TN, USA

ã 2012 Elsevier Inc. All rights reserved.

Glossary Inclusive education Placement in the general education

Adaptive behavior The set of behaviors necessary for classroom with individualized supports and
individuals to function safely and appropriately in the range accommodations.
of activities and environments presented in daily life. Intellectual disability Significantly subaverage intellectual
Autism spectrum disorders A spectrum of developmental functioning which leads to impairments in adaptive
disorders manifested by impaired social interaction and behavior, all of which are first manifested during
communication, as well as stereotypic behaviors. childhood.
Behavior skills training Training in a behavior or skill that Prader–Willi syndrome A genetic disorder caused
involves teaching, modeling, rehearsal, and feedback. either by a deletion of a certain part of the chromosome
Cognitive behavioral therapy Therapeutic treatments, 15 contributed by the father, or from having two
based on cognitive and behavioral theories and research, chromosome 15s from the mother.
used to address various mental health disorders. Psychopathology Behaviors symptomatic of mental illness
Down syndrome A genetic disorder caused by the presence or psychological impairment.
of an extra chromosome 21. Task analysis The delineation of component steps required
Dual diagnosis Comorbidity of intellectual disability and to complete a more complex task.
mental health disorder in an individual. Williams syndrome A genetic disorder caused by a deletion
Etiology The cause or origin of a disability; in this text, the on one of the chromosome 7s.
genetic cause of an intellectual disability.

Issues Related to Intellectual Disabilities More recently, the field has contributed to advances in
genetics and biomedicine. Over 1000 different genetic condi-
Although long considered a less interesting or exciting area, the tions have now been associated with intellectual disabilities,
field of intellectual disabilities is beginning to hit its stride. but more importantly, certain genetic etiologies have been
Spurred by findings that are important to many disciplines, the found to predispose individuals to specific maladaptive beha-
past two decades have seen a growing interest in individuals viors, profiles of cognitive–linguistic strengths and weaknesses,
with intellectual disabilities. Correspondingly, there has been a and medical or other conditions. Across many disciplines,
rapid expansion in our nation’s disability-related service and researchers have begun to appreciate that individual genetic
training structures, the inauguration of several new journals, conditions may constitute model systems that may help in
and the start of many research and clinical networks. In future understanding specific cognitive processes, psychiatric diag-
years, we expect even greater numbers of sophisticated, inter- noses, or medical conditions.
disciplinary research projects and increased services to these
individuals, all performed by professionals who have received
academic, research, and clinical training focused on persons Defining and Classifying Intellectual Disability
with intellectual disabilities and their families.
To appreciate these new developments, one needs only to Formerly referred to as ‘mental retardation,’ intellectual disabil-
juxtapose present advances with those in prior years. Until the ity has a long history of debate as to how it is best defined and
early 1960s, the field of intellectual disabilities was in its classified. Here, we discuss the main system used to diagnose
infancy. Few studies existed, few professionals were trained in individuals with intellectual disabilities, as well as the several
intellectual disabilities, and disability-related infrastructure ways in which professionals have classified these individuals.
was virtually nonexistent. But with the presidency of John
F. Kennedy (himself the brother of a woman with intellectual
Three-Pronged Definition
disabilities), a formal field began to take shape. The Kennedy
administration is generally credited with founding the National While the field continues to debate the appropriate way to
Institutes of Child Health and Human Development (NICHD), define an intellectual disability, at least in broad outline the
the United States’ main supporter of biobehavioral research definition of intellectual disabilities has remained relatively
in typical and atypical development. Similarly, the Kennedy stable over time. According to the Diagnostic and Statistical
Administration started the movement to develop University Manual of Mental Disorders, Fourth Edition-Text Revision (DSM-
Centers for Excellence in Developmental Disabilities to IV-TR), a three-pronged definition characterizes intellectual
help in service delivery, as well as programs to provide training disability: (1) having significantly subaverage intellectual func-
in intellectual disabilities to medical and nonmedical tioning that leads to (2) impairments in adaptive behavior, all
professionals. of which are (3) first manifested during the childhood years.

444
 Intellectual Disabilities 445

First, in order for a diagnosis of intellectual disabilities to be Another categorization is by the level of services that the
warranted, the individual must have subaverage intellectual individual needs. Technically, this system is not a level-
functioning. Subaverage intellectual functioning is operationa- of-impairment classification system, in that two individuals
lized as a score of 70 or below on an appropriately standar- with the same IQ score might have different service needs.
dized, individually administered IQ test. Still, particularly for those individuals at the moderate and
Second, individuals must show impairments in everyday severe-profound levels, a strong correlation exists between IQ
adaptive behavior. Thus, individuals with intellectual disabil- and service needs (correlations are weaker for individuals with
ities often have difficulties in performing age-appropriate skills IQs in the mild range). The support needs noted by this system
of daily living (i.e., caring for themselves and their environ- include: intermittent, limited, extensive, and pervasive. Inter-
ments); of communicating with others via expressive, receptive, mittent support refers to occasional support, whereas limited
or written communication; or of getting along with others and support refers to a day program or sheltered workshop. Exten-
following rules. This second criterion highlights that intellectual sive support refers to daily, ongoing support, whereas pervasive
disabilities are not solely related to intellectual deficits, but they support involves support for all activities of daily living (possi-
are also associated with concurrent deficits in everyday func- bly including nursing care).
tioning. To be diagnosed with intellectual disabilities, then,
individuals must also display impaired adaptive behavior. Classification: Etiological Approach
Third, individuals must also first exhibit deficits in both
First begun by Edward Zigler in the late 1960s, the second
intellectual and adaptive behaviors prior to 18 years of age.
approach to classification concerns the cause or etiology of
Thus, an intellectual disability is not an appropriate diagnosis
the person’s intellectual disabilities. In earlier years, researchers
for individuals showing deficits related to accidents, illnesses,
distinguished between those persons who had a clear cause of
or aging that originate during the adult years.
their intellectual disabilities from those who did not. Thus,
While most would agree with these three diagnostic criteria,
Zigler and others wrote of persons who showed ‘organic’ intel-
controversy abounds regarding how each is operationalized.
lectual disabilities, or disabilities that could be traced to any of
In addition to the general criticisms leveled at IQ tests, all agree
hundreds of pre-, peri-, or postnatal causes. This ‘organic’
that few differences exist between individuals slightly below
group was contrasted with a group of individuals whose intel-
versus slightly above IQ 70 (the IQ cut-off line for intellectual
lectual disabilities could not be attributed to any clear causes.
disabilities). Similarly, professionals debate which specific
This latter group of individuals, called by a wide variety of
skills should constitute adaptive behavior; even the field’s
names (familial, cultural–familial, sociocultural familial),
main professional organization, the American Association on
have also been the subject of different theories with regard to
Intellectual and Developmental Disabilities, has changed the
the cause of their intellectual disabilities.
numbers and names of adaptive domains in subsequent defini-
Over the past two decades, the group with ‘organic’ intellec-
tional manuals. Concerns also exist regarding appropriate mea-
tual disabilities has been further divided into those with differ-
sures of adaptive behavior, the relation between adaptive and
ent genetic causes. At the last count, over 1000 different genetic
intellectual functioning, and the potentially limited opportu-
causes of intellectual disabilities were identified, and many show
nities that certain individuals have to develop adaptive skills.
etiology-related behaviors as well as medical and other charac-
Still, while each issue has received varying attention over the
teristics. Such personal characteristics, in turn, have proven
years, most would agree that the combination of subaverage
extremely interesting to professionals in a variety of disciplines.
intellectual functioning, concomitant deficits in adaptive
behavior, and onset during the childhood years, all character-
ize those with intellectual disabilities. Genetic Etiology

Before providing examples of three genetic etiologies, we first

Classification: Level-of-Impairment Approach
provide some background. First, a genetic disorder predisposes
Just as most would agree to the three-pronged definition of individuals to show a particular behavior, although not every
intellectual disabilities, so too do most agree that individuals individual with that disorder necessarily exhibits the behavior.
with intellectual disabilities differ one from another. The prob- Thus, not every person with Prader–Willi syndrome engages in
lem, however, concerns how best to differentiate within this hyperphagia (overeating), and not every person with Williams
population. syndrome has relatively strong linguistic and weak visuospatial
A first perspective has been referred to as a level- skills. Second, not every genetic condition is associated with its
of-impairment approach. That is, individuals with intellectual own unique outcome; indeed, several disorders may show a
disabilities are differentiated by their level or degree of intellec- single behavioral characteristic or set of characteristics. Finally,
tual impairment. The most common level-of-impairment there are many etiology-related outcomes. Some involve a
categorization is by IQ score: mild (IQ between 50 and 69), specific pattern of cognitive, linguistic, or adaptive strengths
moderate (IQ between 35 and 49), and severe/profound (IQ and weaknesses; others pertain to certain times during devel-
between 0 and 34) levels of functioning. Another categoriza- opment when rapid or slowed development occurs; still others
tion – most often used by the school system – differentiates predispose individuals to displaying one or more maladaptive
students into those who are Trainably Mentally Handicapped behaviors. In addition to such behavioral outcomes, different
(TMH) or Educably Mentally Handicapped (EMH); this cate- genetic syndromes also predispose children and adults to spe-
gorization would then direct the placement and instruction for cific physical characteristics and medical problems (e.g., heart
the individual. disease, leukemia, Alzheimer’s disease).
446 Intellectual Disabilities

With this brief background, we now discuss three genetic As individuals with Down syndrome reach adulthood, they
syndromes: Down syndrome, Prader–Willi syndrome, and may become more vulnerable to depressive disorders. Approx-
Williams syndrome. We have chosen to highlight these imately 6–11% of adults with Down syndrome are thought to
because each syndrome features a fair amount of behavioral experience depression and affective disorders, including char-
research, and is associated with several interesting behavioral acteristic features such as being passive, apathetic, and with-
characteristics. Each in its own way helps to make intellectual drawn. Adult depression may originate in adolescence and
disabilities interesting to researchers in various areas of psy- early adulthood and may be confused with the early signs of
chology, psychiatry, and other behavioral sciences. Alzheimer’s disease, which often has a similar presentation.
Recent research has also described a change during adoles-
cence among some individuals with Down syndrome, who
Down Syndrome
became increasingly underactive and overweight. Likewise, as
The most common genetic–chromosomal disorder causing compared with their behavior during childhood, some adoles-
intellectual disability, Down syndrome occurs in about 1 per cents may exhibit increasingly secretive tendencies and prefer-
800 births. Caused in most cases by the presence of an extra ences to be alone. Even compared with other young adults with
twenty-first chromosome, Down syndrome is associated with intellectual disabilities, young adults with Down syndrome
distinct physical and behavioral characteristics. These indivi- may benefit from increased activity and engagement in com-
duals are often affected by poor muscle tone, congenital heart munity life. Negative physical and behavioral changes among
defects, thyroid problems, vision and hearing problems, leuke- adolescents with Down syndrome warrant attention, as they
mia, and other health issues. By age 40, all persons with Down may anticipate depressive symptoms in adulthood.
syndrome are thought to have the plaques and tangles charac-
teristic of Alzheimer’s disease, even though the dementia nor-
Prader–Willi Syndrome
mally associated with Alzheimer’s disease appears only in
some adults (usually by age 50). Although life spans have Occurring in about 1 per 15 000 births, Prader–Willi syndrome
risen rapidly over the past few decades, the median age of results from two distinct causes. Most cases (70%) result from a
death for individuals with Down syndrome is now about deletion on the long arm of the paternal chromosome 15. The
60 years (some 15–20 years less than the life expectancies remaining individuals diagnosed with Prader–Willi syndrome
found in the general population). result from maternal uniparental disomy of chromosome 15,
Generally associated with intellectual disability in the mild in which both chromosome 15s are contributed by the mother.
to moderate range, individuals with Down syndrome also dem- Common to both causes is the absence of the paternal con-
onstrate a specific behavioral profile. Most persons with the syn- tribution to chromosome 15. Individuals diagnosed with
drome show relative strengths in visual (as opposed to auditory) Prader–Willi syndrome are often categorized in the mild
short-term memory, with relative weakness in several linguistic range of intellectual disability (IQ scores averaging around
tasks. With only a few exceptions, most children and adults with 70), and show relative strengths in daily living skills.
Down syndrome struggle with expressive language and articula- The most notable behavioral characteristic of Prader–Willi
tion, as well as with consistent use of grammatical morphemes syndrome is hyperphagia, or compulsive overeating. Hyper-
(e.g., ‘-s’ for plural, ‘-ed’ for past) and other aspects of grammar. phagia, which begins in most children sometime between
In terms of personality, individuals with Down syndrome 2 and 6 years and continues throughout life, is thought to
are often described as being cheerful, friendly, and eager to involve the hypothalamus and is characterized by incomplete
please. However, this commonly accepted description does not satiation (hence the motto of the Prader–Willi Syndrome Asso-
include other characteristics also frequently observed among ciation of the United States: “Always hungry, never full”).
individuals with Down syndrome. For example, some children Behaviors associated with hyperphagia include food-seeking,
and adults have been described as active, distractible, and foraging, and hoarding. If left unchecked, individuals with
stubborn. As with all behavioral profiles, variation exists and Prader–Willi syndrome become morbidly obese, and some
some individuals may exhibit these personality characteristics, adults can weigh 300 pounds or more. As might be predicted,
while others may not. obese individuals with Prader–Willi also experience heart dis-
Although maladaptive behaviors are found at lower rates ease, diabetes, and sleep apnea. Dietary interventions are
than among others with intellectual disabilities, researchers critical to avoid these health risks and to decrease the preva-
nevertheless estimate that 13–15% of children with Down lence of early death related to obesity.
syndrome engage in maladaptive behaviors, such as stubborn- Beyond hyperphagia, individuals with Prader–Willi syn-
ness, defiance, and aggression. Also, children with Down syn- drome also often display high rates of other maladaptive beha-
drome have been found to exhibit repetitive, compulsive-like viors. Many engage in tantrums and aggressive behavior, and
behaviors in more extreme forms and more frequently than may be described as emotionally labile and stubborn. Indivi-
typically developing children. However, like typical children, duals with Prader–Willi are also often described as underactive
the frequency of these behaviors decreases as age increases. and lethargic, further contributing to health concerns.
Also, individuals with Down syndrome may be affected by These individuals also show high rates of obsessive and
attention-deficit hyperactivity disorder (ADHD), oppositional compulsive behaviors. Although such obsessions and com-
and conduct disorders, and anxiety disorders. Such psychiatric pulsions often relate to food, individuals with Prader–Willi
conditions typically manifest during childhood and adoles- syndrome also engage in obsessive–compulsive behaviors unre-
cence; researchers generally report higher prevalence rates lated to food, such as hoarding objects, performing rituals, and
among adolescent populations. obsessive cleaning. Similar to their food-related obsessions,
 Intellectual Disabilities 447

other obsessive and compulsive behaviors typically emerge dur- and maintaining friendships. Furthermore, their indiscrimi-
ing the preschool years. The tendency toward these behaviors nately social behaviors may make these individuals more vul-
among individuals with Prader–Willi syndrome indicates the nerable to exploitation.
increased risk of obsessive–compulsive disorder (OCD) in this Recent research also highlights the tendency among indi-
population. viduals with Williams syndrome to become highly anxious,
While originating in childhood, obsessive–compulsive worried, fearful, and to have perseverative thoughts. Indeed,
behaviors typically intensify in adolescence and adulthood. compared to those with other types of intellectual disability,
Awareness of this trend should inform the administration of persons with Williams syndrome are more prone to experience
assessments and development of interventions to address vari- anxiety, and such fears and anxieties increase during adoles-
ous compulsions and maladaptive behaviors. Additionally, the cence. Many fears are about future events and health concerns.
severity of behavior among individuals with Prader–Willi syn- While many individuals with Williams syndrome experience
drome, particularly related to eating, often warrants highly some level of anxiety, it is unclear how this tendency relates to
restrictive interventions (e.g., dedicated ‘Prader–Willi-specific’ phobias and other psychiatric conditions.
group homes with locked kitchens). Understanding both the In addition to appropriate medication, interventions to
trajectory of these maladaptive behaviors and their typical address anxiety among individuals with Williams syndrome
levels of severity can help professionals effectively intervene might be developed based on the relative strengths of this
to benefit the physical and mental health of individuals with group. Given the high verbal abilities of most individuals,
Prader–Willi syndrome. interventions might prove particularly helpful when they
involve talking to a therapist or participating in group therapy
sessions. Finally, given most individuals’ affinity for music,
Williams Syndrome
therapies involving music may be particularly helpful in addres-
Williams syndrome results from a deletion on one of the sing anxiety and other behavioral issues experienced by many
chromosome 7s that includes the gene for elastin, a protein persons with Williams syndrome.
in connective tissue that supplies strength and elasticity to
the organs. Occurring in about 1 in 10 000 births, those with
Williams syndrome are often affected by health problems Dual Diagnosis
related to elastin insufficiency, such as cardiovascular disease.
Individuals with Williams syndrome are also typically affected Beyond the specific maladaptive behaviors and psychiatric
by hypercalcemia, as well as by musculoskeletal and renal conditions found in individuals with Down syndrome, Prader–
abnormalities. Individuals with Williams syndrome also Willi syndrome, and Williams syndrome, generally higher
often experience hyperacusis, or an oversensitivity to certain rates of maladaptive behavior–psychopathology occur within
sound frequencies. the overall population of those with intellectual disabilities.
Most notable to behavioral researchers has been the syn- Until recently, this statement was in itself controversial, in that
drome’s characteristic cognitive–linguistic profile. Most indivi- parents and advocates wanted to distinguish the interests and
duals with Williams syndrome have limited visuospatial skills needs of those with intellectual disabilities from those with
such that they have great difficulty in drawing and performing psychiatric diagnoses but who did not have intellectual dis-
visual tasks on IQ tests. However, they are generally able to abilities. Recently, however, the field has realized that it needs
recognize faces, as well as understand and respond to facial to pay greater attention to the amounts, types, and treatments
emotions. In addition, most individuals with Williams syn- needed for those who are ‘dually diagnosed,’ who have both
drome show relatively strong abilities in expressive language, intellectual disabilities and emotional/behavioral problems.
with well-developed vocabularies (often including atypical or The first issue relates to the sheer amount of mental health
low frequency words), use of relatively advanced syntax and issues among those with intellectual disabilities. At present,
semantics, and at times advanced prosody and story-telling skills. estimates vary widely, with the prevalence of comorbid intel-
Persons with Williams syndrome also often display an lectual disabilities and mental health issues estimated to be as
affinity for music. Although much has been made of musically low as 10% and as high as 70% in all individuals with intellec-
talented individuals with Williams syndrome, such individuals tual disabilities. Though imprecise, the median number, about
are relatively rare. In contrast, most individuals with the syn- 40%, indicates that many of these individuals have significant
drome are highly interested in music; these individuals seem to mental health concerns.
have exaggerated emotional responses to music that are cur- A second issue relates to the types of problems these indi-
rently being examined by researchers using functional MRI, viduals experience. It may, for example, be the case that per-
ERP, and other real-time brain measurements. sons with intellectual disabilities are at higher risk for certain
In terms of personality, individuals with Williams syn- kinds of psychopathology but not for others. Although this
drome are often described as unusually friendly, engaging, issue is difficult to resolve, so far most work has focused on
pleasant, and interpersonally sensitive. At the same time, how- several specific psychiatric diagnoses. For the sake of space, we
ever, many of these individuals are also described as impulsive, discuss here the diagnoses of autism spectrum disorder, anxi-
hyperactive, and inattentive, with a tendency to be socially ety, and depression.
disinhibited. Together, these behavioral characteristics often Individuals with intellectual disabilities who have autism
lead the individuals with Williams syndrome to be strikingly spectrum disorders (ASD) may have higher rates of behavioral
but indiscriminately social; as a result, many adolescents and problems. ASD is a spectrum of developmental disorders man-
adults with Williams syndrome have difficulties in developing ifested by impaired social interaction and communication,
448 Intellectual Disabilities

as well as stereotypic behaviors. Prevalence rates for the dual To address these problems, mental health professionals
diagnosis of intellectual disability and ASD range from 2% to have developed alternatives to typical psychiatric interviews.
41%. Compared to those with an intellectual disability alone, For example, the Psychiatric Assessment Schedule for Adults
individuals with both ASD and intellectual disability have with Developmental Disability was developed specifically to
higher levels of problem behavior, including stereotypy, assess individuals with intellectual disabilities. Additionally,
aggression, and self-injurious behavior. Additionally, indivi- the National Association for the Dually Diagnosed (NADD)
duals with ASD and intellectual disability (vs. those with intel- and American Psychiatric Association (APA) have recently
lectual disability alone) have higher rates of pica (eating adapted the criteria for diagnoses in the DSM-IV-TR, tailoring
nonedible items) and sleep disorders. them for use with individuals with intellectual disabilities.
As a second issue, individuals with intellectual disability are These adapted criteria are detailed in the Diagnostic Manual –
at a higher risk for anxiety. Prevalence of anxiety among indi- Intellectual Disability (DM-ID).
viduals with intellectual disabilities has been estimated to be as In addition to these assessment adaptations related to for-
low as 1.5% to as high as 24% of this population. As noted mal psychiatric diagnoses, other tools are available to assess
above, individuals with certain genetic conditions (vs. those the behavior of individuals with intellectual disabilities.
with other genetic conditions or with unknown etiology) may Checklists and screeners developed expressly for this purpose
be more likely to have heightened anxiety. For example, indivi- include the Aberrant Behavior Checklist, the Reiss Screen, and
duals with Williams syndrome are more likely to have anxiety, the Developmental Behavior Checklist. In addition, conduct-
including generalized anxiety or worry about the future. ing a Functional Behavioral Assessment (FBA) is an excellent
Like anxiety, depression is thought to be more prevalent way to observe the nature, duration, and functions of particular
among individuals with intellectual disabilities. Also similar to problem behaviors performed by an individual. Using the
the literature on anxiety, the prevalence estimates of depression FBA, professionals can identify patterns in an individual’s
within this population vary widely, ranging from 1.5% to 30% of problem behavior, and identify interventions that may effec-
all individuals. Though rates of depression are higher among tively decrease this behavior.
individuals with (vs. without) intellectual disabilities, prevalence
patterns mirror those of the general population: women are more
prone to depression than men, and adolescents/adults are more Interventions
likely to experience depression than children. Additionally, cer-
tain genetic disorders may be at a higher risk for depression. To address areas of concern for individuals with intellectual
Thus, individuals with Down syndrome seem more vulnerable disabilities, several interventions are available. These relate to
to depression, particularly during the adolescent and adult years. academics, social and adaptive behavior, mental health, and
family supports.
Assessment
Academic Interventions
For several reasons, mental health professionals have difficulty
in assessing psychopathology among individuals with intellec- Prior to 1975, individuals with intellectual disabilities were
tual disability. First, professionals have historically allowed the either not educated at all or educated in segregated settings
diagnosis of intellectual disability to overshadow a potential solely for individuals with intellectual disabilities. Since its
diagnosis of psychiatric disorders. For example, if an individual passage in 1975, however, the Individuals with Disabilities
with intellectual disabilities displays symptoms of depression, Education Act (IDEA) has mandated that schools provide ser-
anxiety, or a behavioral disorder, a clinician might simply vices to students with intellectual disabilities. Furthermore,
attribute these behaviors to the person’s intellectual disabil- IDEA requires that students with intellectual disabilities be
ities, rather than assessing the individual for a psychiatric educated in the least restrictive environment: to the maximum
diagnosis. In this way, diagnostic overshadowing can lead to extent appropriate, these students are educated with their peers
the lack of appropriate assessments and diagnoses for indivi- without disabilities. The most inclusive setting these students
duals with intellectual disabilities. could participate in is the general education classroom with
A second issue concerns the degree to which traditional supports and modifications.
diagnoses apply to individuals with intellectual disabilities. Research overwhelmingly supports the inclusion of stu-
As described in the DSM-IV-TR and the ninth or tenth edition dents with intellectual disabilities in general education class-
of the International Classification of Diseases (ICD), diagnoses rooms. When educated in inclusive settings (as opposed to
are often based on information gathered during psychiatric segregated settings), students with disabilities have higher
interviews. When interviewing persons with intellectual dis- levels of academic achievement and social competence. Fur-
abilities, professionals have raised concerns about the validity thermore, there have been no negative effects upon the instruc-
of such interview responses for several reasons. First, persons tional time of students without disabilities; these students also
with intellectual disabilities often display an acquiescence bias, benefit from inclusive schooling as it allows them to take on
telling the interviewer what he or she wants to hear. Second, different academic roles (e.g., peer tutor) for their classmates
some individuals with intellectual disabilities have difficulty with disabilities.
with expressive language and with communicating abstract While prior to the 1997 reauthorization of IDEA most
thoughts and feelings. Finally, some individuals may struggle instruction for students with intellectual disabilities focused
with reporting accurate information about the nature and on functional skills, recently the instructional focus has been
duration of their symptoms. academic skills. Regarding reading, systematic prompting, and
 Intellectual Disabilities 449

fading have been found effective in teaching sight words to perspective-taking and poor social skills. Consequently, these
students with intellectual disabilities. These instructional individuals have difficulty recognizing and interpreting non-
developments have been critical to advancing the literacy of verbal and contextual cues. One way to address these social
these students. To give one example, students with Down syn- deficits is through social stories: by reading or hearing a story,
drome were historically not considered capable of learning to the individual learns the social cues specific to a certain situa-
read, with many doubting that these students could gain phone- tion and the ways in which he or she should respond. By
mic awareness (i.e., the ability to break down words into repeatedly reading the social story, the person with disabilities
sounds). Given appropriate instruction, however, many students can learn the appropriate social response to a specific scenario.
with Down syndrome can acquire phonemic awareness and,
eventually, literacy skills. Changes in instruction along with
Mental Health Interventions
inclusive education have advanced both the academic achieve-
ment and community integration of individuals with intellectual Treatment of dually diagnosed individuals may include phar-
disabilities. macological interventions as well as cognitive and behavioral
Another intervention used to support the academic achieve- therapies. As with all patients, professionals should exercise
ment of students with intellectual disabilities involves uni- caution in recommending pharmacological treatments for
versal design. The purpose of universal design is to deliver individuals with intellectual disabilities. There are, however,
instruction in multiple ways to ensure that all students can several issues of special relevance that must be considered.
access the material. The curriculum is designed to give multiple First, many mental health professionals do not receive ade-
representations of content, multiple options for expression, quate training in working with individuals with intellectual
and multiple choices for engagement. Instruction using univer- disabilities; beyond their training years, most only work with
sal design principles could include using a lower reading level, this population occasionally. This lack of training and experi-
using visual representation for words, and embedding auditory ence may result in diagnostic overshadowing, or in inappropri-
cues within text. These strategies can allow learners of all ability ate diagnoses. Insufficient training and experience can also lead
levels to access academic content. to inappropriate prescriptions for these individuals. Clearly,
mental health professionals should exercise caution in diagnos-
ing and treating persons with intellectual disabilities.
Social and Adaptive Skill Interventions
In addition to pharmacological interventions, individuals
In addition to academic interventions, there is also a need for who are dually diagnosed may benefit from cognitive behav-
interventions related to adaptive behavior and social skills. For ioral therapy. This type of therapy has been used to treat
example, an individual with an intellectual disability may have individuals with a variety of mental health issues, including
difficulty with self-determination: making choices and advo- depression and anxiety disorders. Using cognitive behavioral
cating for oneself. During childhood and into early adulthood, therapy to treat an individual with an anxiety disorder might
many choices are limited or unavailable to individuals with involve relaxation and mindfulness training. Additionally, it
intellectual disabilities; instead of choices, these individuals are might include gradual exposure to the object of fear or anxiety.
often given directives. For these reasons, it is important to Cognitive behavioral therapy alone, or paired with appropriate
explicitly teach individuals with intellectual disabilities about medication, offers potential for addressing the needs of
decision-making. One way to do this is through a task analysis. individuals with intellectual disabilities.
A task analysis breaks down the steps involved in a specific act
into teachable units. By clearly delineating the steps needed to
Family Support
make a certain decision, the individual may be better able to
make decisions. In earlier times, all families of individuals with disabilities were
Another issue related to self-determination among indivi- considered to have high levels of problems. Since the mid-
duals with intellectual disabilities is safety and vulnerability. 1980s, however, researchers have appreciated that having
Compared to those without intellectual disabilities, individuals a child with disabilities simply involves an added stressor on
with intellectual disabilities are more likely to experience the family system. Like moving, changing jobs, having a child,
exploitation or victimization. During childhood, children or getting sick, such stressors can lead to either good or bad
with (vs. without) disabilities are four to ten times more likely effects on the family overall and on each member. Following
to experience physical and sexual abuse, and neglect. Similarly, this realization, researchers changed in their perception that all
adults with intellectual disabilities are twice as likely to experi- of these families were in trouble, to the view that some families
ence crimes committed against them. In response to such statis- were doing well, others poorly. Henceforth, the emphasis
tics, behavior skills training – teaching and modeling safety shifted to identifying those characteristics – of the child with
skills and then role-playing the learned skills – has proven an disabilities, the parents, siblings, or family as a whole – that
effective teaching method for this population. Such skills are might promote optimal family functioning and identifying
further developed and generalized when behavior skills train- those families in need of additional help.
ing sessions are combined with assessments in which the indi- In this vein, some supports seem needed by virtually all
vidual practices the safety skills in the community. families, others by only a few. Faced with the birth and early
Related to their deficits in choice-making and higher development of a child with intellectual disabilities, all families
likelihood of victimization, individuals with intellectual dis- require information about various social service systems, rang-
abilities also have trouble with social adaptation. Specifically, ing from early intervention programs to medical services. Later,
some individuals with intellectual disabilities have poor parents must learn about schools, individualized education
450 Intellectual Disabilities

program (IEP) meetings and federally guaranteed rights. As the appreciating just how often intellectual disabilities and mental
child reaches the end of their federally guaranteed public edu- health problems co-occur. Likewise, only now are questions
cation, parents need to learn about their state’s adult-service being asked about how best to match characteristics of these
system, and how to negotiate for their offspring. individuals and their families to current or future service-
In addition to learning about the service system, parents delivery systems.
and families also require specific knowledge of their child’s We are, in short, at an exciting time in the field of intellec-
condition. Such information is often provided by knowledge- tual disabilities. Over the past decade, growing numbers of
able local professionals, as well as by national, state, and local diverse professionals – from neuropsychologists and develop-
chapters of various parent support groups. Increasingly, such mental psycholinguists to pediatricians, cardiologists, and
information can be found via many different modalities, oncologists – have all been drawn to subgroups within this
including federal, organizational, and parent-group websites. population. Service systems and training structures have also
In addition to education about services and their child’s grown and become more sensitive to the characteristics and
condition, many parents value the chance to meet and talk with needs of these individuals. Although we clearly have a long way
parents of children with similar problems. Parent support to go, individuals with intellectual disabilities and their
groups are especially effective for new parents, who realize that families are increasingly the focus of research, clinical-outreach,
they are not alone in parenting a child with a specific condition. and training efforts.
Parents also begin to see older children with the condition and
realize that their child may be capable of more than they had
imagined. Parent groups may also empower parents to advocate See also: Academic Achievement; Anxiety Disorders; Autism and
for their child with school, medical, and social service personnel. Pervasive Developmental Disorders; Behavior Analysis; Behavioral
One also must consider the needs of siblings. In contrast to Genetics; Childhood Mental Disorders; Cognitive Behavior Therapy;
the thinking from earlier years, most siblings of individuals Developmental Psychopathology; Phobias.
with intellectual disabilities fare well. Adverse outcomes are
nonexistent, affect only a few nondisabled siblings, or show
only ‘small effects’ in group-difference studies. Although posi-
tive outcomes have been less-often studied, many individuals Further Reading
benefit from being the sibling of a person with a disability. Baer DM, Wolf MM, and Risley TR (1968) Some current dimensions of applied behavior
Such siblings consider themselves to have grown because of analysis. Journal of Applied Behavior Analysis 1: 91–97.
this experience, and siblings often remark that they now better Batshaw ML, Pellegrino L, and Roizen NJ (2007) Children with Disabilities, 6th edn.
appreciate differences among individuals, are open to new Baltimore, MD: Paul H. Brookes.
Browder DM, Spooner F, Wakeman S, Trela K, and Baker JN (2006) Aligning instruction
experiences, and appreciate life’s true meanings. with academic content standards: Finding the link. Research and Practice for
A final issue relates to the longevity of individuals with Persons with Severe Disabilities 31: 309–321.
intellectual disabilities. In most industrialized countries, indi- Dykens EM (1996) Direct effects of genetic mental retardation syndromes: Maladaptive
viduals with intellectual disabilities are living increasingly lon- behavior and psychopathology. International Review of Research in Mental
Retardation 22: 2–26.
ger lives, even as the majority (about 60%) of such individuals
Dykens EM (2000a) Annotation: Psychopathology in children with intellectual disability.
live their adult years in their parents’ home. The presumed Child Psychopathology and Psychiatry 41: 407–418.
future caregiver for most aging adults with intellectual disabil- Dykens EM, Hodapp RM, and Finucane B (2000b) Genetics and Mental Retardation
ities will thus be a nondisabled sibling, in most cases one of the Syndromes: A New Look at Behavior and Treatments. Baltimore: Paul H. Brookes.
female siblings. Many, maybe even most, of these women need Evans DW and Gray FL (2000) Compulsive-like behavior in individuals with Down
syndrome: Its relation to mental age level, adaptive and maladaptive behavior. Child
information about the needs of their sibling with a disability Development 71: 288–300.
and the various systems that might provide support. Unfortu- Fisher MH, Hodapp RM, and Dykens EM (2008) Child abuse among children with
nately, most state service systems are ill-equipped to handle the disabilities: What we know and what we need to know. International Review of
growing numbers of aging individuals with disabilities; sibling Research in Mental Retardation 35: 251–289.
Fletcher R, Loschen E, Stavrakaki C, and First M (eds.) (2007) Diagnostic
caretakers need information about how best to navigate this
Manual – Intellectual Disability (DM-ID): A Textbook of Diagnosis of Mental
system to secure needed services. Disorders in Persons with Intellectual Disability. Westchester, NY: National
Association of the Dually Diagnosed (NADD).
Freeman SFN and Alkin MC (2000) Academic and social attainments of children with
Conclusion mental retardation in general education and special education settings. Remedial
and Special Education 21: 3–26.
Greenspan S and Granfield JM (1992) Reconsidering the construct of mental
Historically an area of less interest or excitement, the popula- retardation: Implications of a model of social competence. American Journal on
tion with intellectual disabilities is increasingly gaining in Mental Retardation 96: 442–453.
importance within a wide variety of fields. Much of this interest Hodapp RM and Dykens EM (2009) Intellectual disabilities and child psychiatry:
Looking to the future. Journal of Child Psychology and Psychiatry 50: 99–107.
stems from connecting findings on those with different genetic
Hodapp RM and Ly TM (2005) Parenting children with developmental disabilities.
disorders to specific cognitive–linguistic, medical, or other In: Luster T and Okagaki L (eds.) Parenting: An Ecological Perspective, 2nd edn.,
conditions. Those with genetic disorders are increasingly pp. 177–201. Mahwah, NJ: Erlbaum.
serving as models for how specific behavioral or medical out- Individuals with Disabilities Education Act of 2004, 20 U.S.C. 1400 et seq.
comes might arise. Koegel LK, Koegel RL, and Dunlap G (1996) Positive Behavioral Support: Including
People with Difficult Behavior in the Community. Baltimore, MD: Paul H. Brookes.
Persons with intellectual disabilities and their families also Rojahn J and Meier LJ (2009) Epidemiology of mental illness and maladaptive behavior
have characteristics and needs that are only now being under- in intellectual disabilities. International Review of Research in Mental Retardation
stood. For example, over the past decade we are gradually 38: 239–287.
 Intellectual Disabilities 451

Seltzer MM, Patterson D, and Lott IT (eds.) (2007) Special issue on Down syndrome. http://idea.ed.gov/ – Individuals with Disabilities Education Act.
Mental Retardation and Developmental Disabilities Research Reviews 13: 197–289. http://www.nichcy.org/ – National Dissemination Center for Children with Disabilities.
http://www.ndsccenter.org/ – National Down Syndrome Congress.
http://www.ndss.org/ – National Down Syndrome Society.
Relevant Websites http://www.rarediseases.org/ – National Organization of Rare Disorders (NORD).
http://www.pwsausa.org/ – Prader–Willi Association, US.
www.aaidd.org – American Association for Intellectual and Developmental Disabilities. http://www.thearc.org/ – The Arc of the United States.
www.aucd.org – Association of University Centers on Disabilities. http://www.thenadd.org/ – The National Association for the Dually Diagnosed (NADD).
http://www.cec.sped.org/ – Council for Exceptional Children. http://www.williams-syndrome.org/ – The Williams Syndrome Association.