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Introduction
IDD)
IDD is the first disorder to appear under the major category of Neurodevelopmental
Disorders. This category is new to the DSM and is shared by seven disorders that have
childhood onset, and involve cognitive impairments (specific or general/global) that
significantly impact functioning in a major area, such as school or employment, and is
persistent across the life span. The other disorders in this category include: communication
disorders (language disorder, speech sound disorder, childhood-onset fluency
disorder/stuttering, social/pragmatic communication disorder); autism spectrum disorder
(ASD); attention-deficit/hyperactivity disorder; specific learning disorder; motor disorders
(developmental coordination disorder, stereotypic movement disorder, tic disorders) and other
neurodevelopmental disorders (other specified, or unspecified neurodevelopmental disorder).
The DSM-5 (APA, 2013) presents three main criteria for a diagnosis of IDD, as
follows:
1. Deficits in intellectual functioning (reasoning, problem solving, learning, etc.) that have
been verified by clinical observations and individual assessments;
2. Deficits in adaptive functioning resulting in an inability to meet developmental expectations
Significantly Subnormal Intellectual Functioning
obtained on a standard and well-recognized instrument that has been developed specifically to
assess intelligence (e.g., Wechsler Intelligence Scale for Children: WISC-V; Stanford–Binet 5 th
Edition).
Severity Conceptual Domain Social Domain Practical Domain
Mild In very young children differences There may be delays in Individuals may be able
may not be easily recognized, but social engagement and to manage self-care,
may begin to be noticeable by school communication and some recreational outlets,
age, especially delays in acquiring children may miss subtle and jobs that are highly
core academic skills. Adults may social cues, or not structured.
experience problems with recognize risky situations. Management of
organization, money management There may be problems financial affairs, or
and other activities relying on with emotion and behavior making
functional academics. regulation. important decisions
(e.g.,
health care) likely will
require support.
Moderat Increased supports may be Making and keeping friends Training and practice
e needed to enhance academic skills, may be problematic due can
especially in areas requiring to language delays and be successful in
reasoning involving money or time. immature social skills. acquiring
Adult skill levels are likely to plateau Caregivers will be needed skills in self-care,
academically at an elementary to provide ongoing although
school level and assistance will be support for decision monitoring may be
required to manage day-to-day making in day to day needed. Ongoing
Severity Conceptual Domain Social Domain Practical Domain
Severe Ongoing support will be Individuals may be able All activities of self-
needed, due to limitations in to communicate using care and
understanding time, money, or simple speech and daily living will require
numbers. gestures; however, speech support and
is often limited. Speech supervision.
augmentation may be Some may exhibit
required. maladaptive and self
injurious behaviors.
Profound Extensive support is required due to Social engagement is Extensive support is
comorbid motor and sensory challenging due to required due to the
impairments, and limited ability to comorbid motor and complex nature of
comprehend written or spoken sensory impairments accompanying
language. and very limited ability comorbid
to understand speech or impairments.
gestures. Extensive
support
needed.
Assessment of Intellectual and Adaptive Functioning
The instruments have been developed to provide normative information
concerning the individual’s intellectual level (IQ) relative to similar-aged peers,
and of adaptive functioning in the areas mentioned relative to developmental
expectations.
The DSM-5 has developed this special category for individuals under the age of 5
years who may be suspected of having IDD but cannot be assessed due to
circumstances (e.g., the child is too young). In this special case, the diagnosis of
global developmental delay will be given if the child does not achieve age-appropriate
developmental milestones in several areas of intellectual functioning. However, this is
only meant as a temporary diagnosis and is only valid for a period of time until
arrangements can be made for a full clinical assessment to be conducted.
The American Association on Intellectual and Developmental
Disabilities (AAIDD)
In 1992, the American Association on Mental Retardation (AAMR) made a landmark decision to
shift emphasis from the DSM focus on severity of disorder (mild, moderate, severe and
profound) to a focus on intensity of intervention required (intermittent, limited, extensive or
pervasive). Doing away with levels of dysfunction also placed greater emphasis on the
intensity of intervention required in order to assist individuals to have greater functioning
ability. The levels of intensity of intervention were listed as:
• Intermittent
• Limited
• Extensive
• Pervasive.
At this time, the upper level for onset was also increased from 16 to 18 years of age. In 2007,
the American Association on Mental Retardation (AAMR) changed its name to the American
Association on Intellectual and Developmental Disabilities (AAIDD) to recognize how the
disorder was conceptualized internationally as intellectual disability or intellectual
developmental disorder (as it is recognized in the International Code of Diseases, ICD-10).
AAIDD continued to recognize the same three diagnostic criteria as the DSM-5, involving: IQ
cutoff for intellectual disability (70, plus or minus 5); adaptive deficits; and onset in the
When a diagnosis of IDD is made, AAIDD (2010) clearly focuses on the need to plan a
support system in order to reduce the gap between an individual’s capabilities and their
skills with the goal of promoting the most successful lifestyle possible. The AAIDD
continues to evolve how it defines an intellectual disability and the latest definition can be
found in the 11th Edition of the AAIDD Definition Manual (2010).
Although AIDD has supported an IQ range for IDD similar to that proposed by the DSM-5,
they have also more recently advocated for an IQ level that could potentially raise the
cutoff to 76.
Assessment of Intensity of Supports
When the definition of IDD was infused with the concept of intensity of
supports, it was soon recognized that the best way to determine the level
and nature of supports required was to develop a scale to measure
supports. Thompson and colleagues (2004) than developed Supports
Intensity Scale (SIS).
The SIS consists of six subscales that measure the nature and intensity of
supports needed in 49 different areas. The six subscales represent
domains of adaptive functioning and include:
1. Home living
2. Community living
3. Lifelong learning
4. Employment
5. Health and safety
IDEA (2004)
There has been considerable change in the way that disabilities have been defined over the
years, and the education system is no exception in this regard. Historically, IDEA has modified
its definition of IDD and the recommended levels of IQ functioning to align with definitions and
IQ levels suggested by AAIDD. Initially, criteria for IDD within the educational system involved
an IQ range of 55 to 80 for classification as either educable mentally retarded (EMR) or
educable mentally handicapped (EMH). Students with IQs in the 25 to 55 range traditionally
have been most commonly classified by the educational system as trainable mentally
retarded (TMR) or trainable mentally handicapped (TMH).
However, the suggested IQ level to serve as threshold for IDD has changed over the years and
has dropped from an initial consideration of 85 to its current cut-off score of an IQ of 70 (+5), in
most states. Although IDEA provides the general driving force for educational determination,
funds for special education programs are allocated by state codes that also set cutoff scores
that determine eligibility for programs. As a result, actual IQ ranges for IDD may vary from state
to state.
Studies that have looked at classification and placement procedures have found
considerably disparity in their results. For example, MacMillan and Forness (1998) suggest
that placement decisions may be made more on compliance issues (allotted placements per
category) than on predetermined criteria. In one sample in their California study, they found
that of 43 children, scoring below 75 on their IQ test, only 6 of the 43 were designated as
IDD. Fifty percent of children with IQ scores at this level were classified as Learning Disabled
(LD). When IDEA was reauthorized in 1990, the definition of IDD used by IDEA (1990) was in
agreement with definitions of IDD set by AAIDD at that time.
As a result, three key areas were deemed necessary for identification of having IDD:
1. Significant limitations in intellectual functioning;
2. Significant limitations in adaptive functioning;
3. Onset prior to 18 years of age.
Early Identification Procedures and Developmental Delay
Sections of IDEA (2004) that are concerned with early identification of children at the
beginning of the process (infants and toddlers) are covered under Part C). In Part C, Section
635, IDEA (2004) defines developmental delay as a delay of 35% or more in one of the
developmental areas or 25% or more delayed in two or more of the developmental areas.
There are five global areas of potential delay outlined in IDEA (2004, Sec. 632):
• Cognitive development
• Motor skills
• Communication skills
• Social or emotional development
• Adaptive functioning.
Included under adaptive functioning are those skills involved in daily living, such as self-help,
being able to dress or feed oneself and so forth. IDEA (2004) also mandates services for
infants and toddlers who have a diagnosed physical or mental condition that has a high
likelihood of resulting in a developmental delay.
IDD: Developmental and Associated Features
Today the majority of children and youth with IDD would fall within the
mild level of impairment. As such, these children will most likely reside
and attend schools in their local community, unless they are transported
to different schools for special programs that are not available in their
home schools. The features of IDD vary widely depending on the
severity, associated personality and behavioral characteristics and the
developmental level. While some children may experience
developmental delays and in a sense, catch up with increased maturity
and stimulation/enrichment, others with diagnosable IDD may vary only
within a limited range of development. As was noted earlier, some
individuals with IDD live a relatively normal life despite cognitive
limitations due to their adequate adaptive skills. Associated features
that may become more obvious over time are delays in achieving
milestones in areas that are key to developmental progress. Examples
include delays in the following global areas:
• Gross motor skills (sitting independently, crawling, walking);
• Fine motor skills (drawing, printing, coloring);
• Communication skills (late speech development, trouble talking);
• Cognitive skill development (problems understanding, problem
solving, remembering and transferring information from one situation to
another);
Comorbidity Individuals with IDD are three to four times more likely to have a
comorbid disorder, than individuals in the population at large. In
addition, comorbid disorders associated with IDD are often more
complex and more difficult to diagnose and treat, because disorder
features may be modified by the presence of IDD (APA, 2013).
7,500 births.
Most individuals with WS have mild to moderate intellectual impairments and have
relatively well developed verbal skills (verbal IQ) compared with very poor performance
skills (performance IQ). Those with WS demonstrate strengths in language (concrete and
practical language rather than abstract) and significant weaknesses in areas of visual
cognition and visuospatial functioning, with the exception of facial recognition which
remains intact.
The personality of those with WS is remarkable in that they are highly gregarious and
driven to social engagement.
Bellugi and colleagues (2007) address the issue that despite being driven to engage
socially, children with WS often exhibit problems maintaining relationships with peers.
They speculate that the disconnect between social-perceptual abilities and social-
expressive behaviors may be found in the fact that although children with WS are
socially fearless, they are also endowed with nonsocial anxiety that may undermine
their ability to relate to others.
Fragile X
This is the most common inherited cause of IDD in the mild to moderate levels and occurs when
there is a change or mutation in a gene called the Fragile X Mental Retardation 1 (FMR1) gene
that is responsible for producing a protein necessary for normal brain development. If only mild
changes in the gene are present, there may be few symptoms; however, greater change in the
gene produces increased symptoms of fragile X syndrome.
The symptoms of fragile X, which can vary relative to the degree of change in the gene, include:
intellectual deficits, and possible physical characteristics (longer ears, faces and jaws). There
may also be challenging behaviors (fearfulness, anxiety), and males may tend to be inattentive
or aggressive, while females may appear withdrawn and shy. Language problems are also often
evident and children may exhibit heightened sensitivity to sound, touch and bright light (National
Institute of Child Health & Development: NICHD). A number of children with Fragile X will also
have comorbid autism.
Phenylketonuria (PKU)
Babies born to mothers who use cocaine can suffer a wide range of side effects after birth,
including physical defects and brain dysfunction in hemorrhages and seizures.
Mothers who are addicted to crack often give birth to infants who suffer from low birth
weight and damage to the central nervous system.
Fetal Alcohol Syndrome (FAS)
Approximately 33% of all babies born to mothers who are heavy consumers of alcohol
will be born with fetal alcohol syndrome (FAS). Clinical features of FAS include:
central nervous system dysfunction (intellectual disabilities, hyperactivity, irritability);
impaired motor coordination; and over activity.
Physically, these children often evidence slow growth and unusual facial features,
including underdeveloped upper lip, flattened nose, or short and upturned, widely
spaced eyes, and small head. Although facial features become less pronounced with
age, cognitive deficits remain.
Risks and Protective Factors
Behavioral Interventions:
Behavioral programs have been very successful in targeting and
altering problematic social, emotional and behavioral concerns. The
reason for the success of the behavioral programs can be linked to the
programs’ focus on breaking down problem behaviors into component
parts (simplicity) and to systematically shaping behaviors into more
socially adaptive behaviors through contingency management.
Parent Training Programs:
Educational Programs:
There is continual and at times heated debate over whether children with
IDD are better served within special education programs or the regular
class (a practice referred to as mainstreaming or inclusion). It is possible
to find support for either side of the debate in research studies. Hocutt
(1996) reviewed over 100 studies from the past 25 years comparing
special education to regular class placement for children with mild IDD
and found that there was considerable variability in results.
Prevention and Early Intervention