Intellectual Disability in Children - Ma... T, Outcomes, and Prevention - UpToDate

3/13/2019 Intellectual disability in children: Management, outcomes, and prevention - UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2019 UpToDate, Inc. and/or its affiliates. All Rights Reserved.
Intellectual disability in children: Management, outcomes,
and prevention
Author: Penelope Pivalizza, MD
Section Editors: Marc C Patterson, MD, FRACP, Helen V Firth, DM, FRCP, DCH, Carolyn Bridgemohan, MD
Deputy Editor: Carrie Armsby, MD, MPH
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Feb 2019. | This topic last updated: Jul 26, 2018.
INTRODUCTION
Intellectual disability (ID) is a neurodevelopmental disorder with multiple etiologies. It is characterized
by deficits in both intellectual and adaptive functioning of varying severity presenting before 18 years
of age [1]. ID encompasses a broad spectrum of functioning, disability, and strengths. ID is an
important public health issue because of its prevalence, its impact on affected individuals, and the
need for extensive support services. ID affects approximately 1 percent of the population. Its
management requires early diagnosis and intervention, including access to health care and
appropriate supports.
The management, outcomes, and prevention of ID in children are discussed here. Other aspects of ID
are discussed separately:
(See "Intellectual disability in children: Definition, diagnosis, and assessment of needs".)
(See "Intellectual disability in children: Evaluation for a cause".)
(See "Primary care of the adult with intellectual and developmental disabilities".)
TERMINOLOGY
The following terms are used throughout this topic and are described in greater detail in a separate
topic review (see "Intellectual disability in children: Definition, diagnosis, and assessment of needs",
section on 'Definitions'):
https://www.uptodate.com/contents/intellectual-disability-in-children-management-outcomes-and-prevention/print?search=intellectual%20disability&topicRef=6172… 1/32
Intellectual disability – Intellectual disability (ID) is a neurodevelopmental disorder that begins in
childhood and is characterized by limitations in both intelligence and adaptive skills, with at least
one of three adaptive domains (conceptual, social, and practical) affected (table 1). The term
replaces the older term of "mental retardation."
Global developmental delay – Global developmental delay (GDD) is the preferred term to
describe intellectual and adaptive impairment in children <5 years old who fail to meet expected
developmental milestones in several areas of intellectual functioning. Not all children with GDD
will meet criteria for ID as they grow older. The term ID usually is applied to children ≥5 years old,
when the clinical severity of impairment is more reliably assessed.
MANAGEMENT
Overview — The overall goals of the management of ID are to:
Lessen the effects of disability
Prevent, halt, or limit deterioration in the child
Strengthen, improve, and optimize functioning in the child's everyday circumstances (eg, home,
school, community, and vocational settings)
To accomplish these goals, multiple supports are generally necessary. Interventions should be
evidencebased, begin early, and be sustained. Goals should be individualized, appropriate, and
achievable. Individualized supports should improve the functioning and lessen the disability of the
person with ID.
The approach should be collaborative and multidisciplinary. Optimal use of available resources,
coordinated continuity in care, monitoring of progress, and matching interventions to the unique and
changing needs of individuals over time are key components. Interventions may need to be prioritized
for efficacy and benefits to an individual in their everyday settings, and changed according to differing
lifetime needs. For example, an emphasis should be placed on vocational goals during adolescence,
and vocational professionals should be part of the multidisciplinary team. Ethical principles and legal
considerations are also important in guiding management.
There is no universallyaccepted standardized protocol or practice guideline for the comprehensive
management of children and adolescents with ID.
General considerations — General considerations in caring for children with ID differ somewhat
from caring for typicallydeveloping children. They include the following:
Effective communication strategies – Effective communication strategies are essential when
interacting with children with ID. This is relevant across all ages and settings. Communication
should be clear, concise, concrete (rather than abstract), and at the level of the child's language
understanding. Unnecessary details or superfluous words should be omitted; children with verbal
deficits (eg, Down syndrome) often have difficulty filtering out what is irrelevant [2]. Where verbal
functions are limited, communication can be enhanced by handson demonstration, the use of
visual adjuncts, or visualspatial learning. Information should be simply, meaningfully, and
respectfully shared at the functional level of the child or adolescent, especially in matters that
require consent. Preschool levels of functioning are compatible with surrogate consent; primary
school level of functioning enables partial participation and assent, but not full consent [3].
Encouraging independent functioning – Strategies to gradually increase personal
responsibility and independent functioning are needed [4]. Greater independent functioning
eventually results in better vocational, employment, and placement results; advocacy, caregiver
resourcefulness, and governing policies and systems can improve independent functioning in
children with ID [2]. Involved decisionmaking should be increased according to individual
capability and desire to do so [3].
Support for family members – It is important for health care providers to provide support and
counseling to families of children with ID. Support services for affected family members can help
address psychosocial stress, depression, helplessness, anxiety, and the need for respite. Levels
of psychosocial stress and optimism vary among families who have a child with ID or global
developmental delay (GDD). Families of severely impaired children with multiple special needs
frequently have the greatest need for psychosocial support; families of children with Down
syndrome report less stress and more optimism [2]. It may be helpful to connect families with
supportive parent groups and other resources in their community; often this is accomplished with
the help of a social worker. Examples of organizations that can help inform, support, and
advocate for individuals with ID include The Arc, FamilytoFamily Health Information and
Education Centers, Autism Society of America, and support groups corresponding to specific
genetic syndromes or conditions (eg, Fragile X, Down syndrome). Such organizations and other
forms of community participation can help support positive social relationships for a child with ID
and his or her family. Positive, warmly nurturing, patiently encouraging parenting styles are more
helpful to support child resilience than negative parenting; urging achievable independence and
task completion is advised.
Advocating on behalf of the child – Parents and providers are frequently effective in
advocating for a child with ID, such as motivating for necessary services to advance functioning.
For providers, this is an ethical responsibility of taking care of vulnerable special needs children.
By definition, ID requires ongoing support [1]; children with ID are able to make progress
provided they receive appropriate supports. Advocacy (eg, by parents) and caregiver
resourcefulness are factors that improve independent outcomes in children with ID [2].
Multidisciplinary collaboration – In the United States, the approach to caring for children with
ID typically involves multiple providers collaboratively contributing to the assessment of ongoing
needs and recommendations for targeted interventions and support in their area of expertise. The
medical home and the pivotal role of parents or family are important and nevertheless
emphasized.
Preventative screening and routine care — General preventative screening and routine health care
maintenance interventions should be no less than those of typically developing children. There are no
anticipatory guidance protocols that comprehensively cover the needs of all children and adolescents
with ID; anticipatory guidance should address agespecific as well as functional levelspecific
preventative health risks and needs. (See "Screening tests in children and adolescents" and
"Guidelines for adolescent preventive services".)
Longitudinal developmental surveillance — Effective longitudinal developmental surveillance in ID
expands upon standard pediatric surveillance and initial diagnosis of ID. Various providers can
contribute to this, including developmental pediatricians, neurodevelopmental neurologists, and/or
clinical psychologists.
Longitudinal surveillance is coupled with direct interventions during the course of a child's life so as to
continue to advance and strengthen functional progress. IDspecific concerns of the parent, provider,
and individual are taken into consideration. The individual's functional profile (relative strengths,
impairments, challenges), developmental trajectory (progress, plateau, stagnation, or skill loss), and
ongoing management plan can be sensitively discussed with the family, and appropriate targeted
supports put in place. The management plan requires collaborative agreement and application.
Goals may need adjusting to optimally strengthen and advance functioning. The child's environmental
may need to be modified. Interventional strategies should be flexible and responsive towards the
needs of the individual and their family [4].
Serial standardized testing assessments generates a curve of the individual's progress over time
which helps guide interventions. For example, nonverbal and visual learning strengths may be paired
with interventions aimed at improving language weakness. Identification of difficulties with certain
motor skills (eg, eyehand coordination, fine or gross motor skills) may prompt additional specific
supports to address these issues.
If there are multiple conditions contributing to impairments in a child with ID, comprehensive testing
can better inform interventional strategies. If deficits involve only a single domain, a narrower focus is
usually appropriate.
The tool(s) used for testing should include lower level skills with reasonable accuracy. The choice of a
specific tool is individualized based on the child's age, language, functional level, disability profile, and
affected domains. Specific tools are discussed in greater detail in separate topics (table 2). (See
"Intellectual disability in children: Definition, diagnosis, and assessment of needs", section on
'Adaptive function' and "Developmentalbehavioral surveillance and screening in primary care".)
The progress of children with ID is often compared with typical normative curves of children without a
disability, but progress may also be compared with causespecific normative developmental
milestones or outcome curves for individuals with ID as they become available (eg, in a studied
population of individuals with Down syndrome) [5].
Early interventions for ID — Early application of evidencebased interventions optimizes functioning
and progress of infants and children with ID. Most individuals with ID require a broad range of
interventions specific to their needs and functional deficits. Examples include:
Speech and language (S/L) therapy – Most individuals with ID have some level of language
based impairment, which can impact their conceptual adaptive functioning and other daily living
functions and activities. S/L therapy can advance a child's language, speech, articulation, and
communication functioning and it advances other skill gains. (See "Evaluation and treatment of
speech and language disorders in children".)
Occupational therapy (OT) – OT can advance functional gains in eyehand coordination and
other motor skills.
Physical therapy (PT) – PT can improve mobility and other motor skill functions, and provide
postural support and other adjuncts for affected individuals.
Hearing and vision services – Services for hearing and vision are important to optimize
functioning and improve everyday participation. (See "Vision screening and assessment in infants
and children" and "Hearing loss in children: Treatment".)
Consultation with a dietician – Consultation with a dietician may be necessary in some cases to
address malnutritionspecific dietary needs (eg, in metabolic or endocrine disorders), or to
evaluate the child's nutritional status and ensure a wellbalanced diet. Evidence is lacking to
support alternative dietary treatments or supplements. In particular, high doses of fatsoluble
vitamins should be avoided because excessive doses can have toxic effects. (See "Dietary
recommendations for toddlers, preschool, and schoolage children" and "Poor weight gain in
children older than two years of age".)
Early education assessment and services – In the United States and many other countries, early
education services are widely applied to advance educationallypertinent development, behavior,
and learning. (See 'Early intervention' below and 'Special education' below.)
Functional living skills – Functional living skills should be included from a young age. Help with
problemsolving and planning are usually necessary. In the classroom, strategies may include
preferential seating (eg, seating the child close to the teacher), providing additional time,
redirecting to stay on task, minimizing distraction, using interests and strengths to advance
weaker functions, reducing complex tasks into discrete sequential steps, using observational and
multisensory learning approaches (eg, visual presentation and handson demonstration),
applying checks for understanding, and reinforcing and generalizing learning. Children and
adolescents with ID are less likely to ask for help or let others know when they do not understand
[2].
Social support – Interventions that focus on improving social participation are important for
children with ID. This can improve independent functioning and enhance quality of life. Compared
with typicallydeveloping peers, young children with ID tend to have less frequent play with other
children, fewer friends, poorer quality friendships, and reduced levels of social participation [6,7].
Social supports may be provided in educational and other settings [2]. Community integration
interventions can improve friendship development and inclusion in recreation with typical peers.
Participation can be enhanced by selecting activities appropriate to the ability and interest of the
child, and including individualized mentoring along with peer interactions [8,9].
Behavioral interventions – Behavioral interventions may be aimed at addressing difficult
behaviors or specific comorbid conditions (eg, attention deficit hyperactivity disorder, autism
spectrum disorder, anxiety, depression, sleep problems). Behavioral interventions can improve
behaviorrelated functioning, performance, and participation in everyday activities and during
other service interventions. Interventions that lack a sufficient evidence base are advised against
(eg, sensory treatments) [10]. (See 'Behavior intervention' below.)
Treatment of specific causes of ID — Most causes of ID do not have a specific treatment or
intervention. However, if a treatable underlying cause of ID is identified (eg, congenital
hypothyroidism, treatable inborn error of metabolism), the child should be promptly treated to prevent
further disability. (See "Intellectual disability in children: Evaluation for a cause" and "Treatment and
prognosis of congenital hypothyroidism" and "Overview of phenylketonuria", section on
'Management'.)
A curated list of treatable diseases that can cause ID is available at www.treatableid.org [11].
The American Academy of Pediatrics offers a "tool kit" for management of children with fetal alcohol
spectrum disorder (FASD), including a list of evidencebased intervention programs specific to FASD
[12]. (See "Fetal alcohol spectrum disorder: Management and prognosis".)
Other impairing coexisting conditions should also be treated when appropriate. (See 'Associated
conditions' below.)
Associated conditions — Children with ID are at increased risk for associated conditions, including
other neurodevelopmental disorders, mental health problems, and medical and physical conditions
(table 3).
Neurodevelopmental disorders and mental health problems — Disorders such as autism
spectrum disorder, attention deficit hyperactivity disorder (ADHD), depression, and anxiety are
common in children and adolescents with ID and can adversely impact quality of life, adaptation, and
individual functioning. Selfinjurious behaviors may present in stress, depression, anxiety, or as a
medication sideeffect (eg, of sedativehypnotic and neuroleptic medication). Repetitive or self
stimulating behaviors are more common in more severely impaired children with ID. Some youth with
ID are at increased risk of suicidal ideation and substance abuse compared with typically developing
peers. The increased behavioral and emotional problems in children with ID may not necessarily meet
standard diagnostic criteria for a disorder, but may nevertheless be troublesome [13]. The detection
and treatment of depression in children is particularly important, as depression can persist and impact
functioning into adulthood. Depression occurs more frequently in ID due to Down syndrome than in ID
due to other causes and frequently needs sustained followup [2]. Disorderspecific surveillance and
interventions are necessary for neurodevelopmental disorders, with referral as needed. (See
"Intellectual disability in children: Definition, diagnosis, and assessment of needs", section on 'Other
neurodevelopmental disorders and mental health problems'.)
Interventions include family counseling, education, behavior interventions designed to address a
specific type of neurodevelopmental disorder in the child (eg, ADHD or autism spectrum disorder),
and psychopharmacologic therapy (where this is recommended standard of care in children) [14].
Interventions should be appropriate to the child's developmental level of functioning and diagnoses.
Although most children with ID can be successfully helped by appropriate behavioral approaches and
environmental manipulation, some require pharmacologic treatment. Psychopharmacologic therapy is
more likely to be used in children who have a comorbid neurodevelopmental disorder or whose ID is
severe. Appropriate use of medication, when indicated, can improve the child's mental health,
participation, and functioning, and secondarily reduce their burden of care. However, medications are
generally started only after initial adequate appropriate behavioral intervention, so as to minimize the
risk and unnecessary use of medication [15]. Because the evidence base for making general
psychopharmacologic recommendations in young children with ID is relatively limited, a carefully
considered therapeutic trial may be indicated to assess whether a therapy is successful in an
individual patient.
The use of medications should be part of a clear comprehensive psychosocial treatment plan that
includes behavioral baseline functioning, identified target behaviors, goals, and evidencebased
intervention appropriate to the diagnosis and target behaviors [16,17]. A child psychiatrist, child
neurologist, or developmental pediatrician can help guide individual psychopharmacologic
management, especially where there are multiple comorbid disorders. Prior to initiating
pharmacotherapy, the clinician and caregivers should agree upon measurable goals and target
behaviors. In addition, patients with irritability, aggression, selfinjury, or disruptive behavior should be
evaluated for other possible causes, including underlying medical or psychiatric disorders or abuse.
Potential adverse effects must be weighed against possible benefits in treatment decisions.
Medications that control behaviors can have negative effects on attention, concentration, learning,
and quality of life, and can cause a temporary or persistent drugrelated movement disorder [18]. The
safest, most effective treatment at lowest possible dose is aimed for, beginning with a low initial dose,
and then slowly increasing ("start low, go slow"), monitoring the patient's response before making
dose adjustments. Ideally, medication regimes should be simple, and use the fewest possible doses in
children with ID. Idiosyncratic responses to medication are possible. For example, patients with ID
may have increased sensitivity to the disinhibiting effects of sedativehypnotics, and children with
Down syndrome may be especially sensitive to anticholinergic medication [16].
Pharmacotherapy for specific disorders are discussed in separate topic reviews:
(See "Autism spectrum disorder in children and adolescents: Pharmacologic interventions".)
(See "Attention deficit hyperactivity disorder in children and adolescents: Treatment with
medications".)
(See "Pharmacotherapy for anxiety disorders in children and adolescents".)
(See "Pediatric unipolar depression and pharmacotherapy: Choosing a medication".)
(See "Pediatric bipolar disorder and pharmacotherapy: General principles".)
Child abuse and neglect — Vigilant surveillance, sensitivity, and a high index of suspicion should
be maintained so as to detect, prevent, and address manipulation, abuse, and/or neglect of children
with ID [19,20]. The child's cognitive and social impairment, literal thinking, inadequate knowledge,
and misunderstanding of the expectations and emotions of others may contribute to the cycle of
abuse. Abuse may precipitate further behavioral disorder in the child (eg, conduct disorder, post
traumatic stress disorder, substance abuse), and those who suffer abuse are at increased risk of
becoming a perpetrator. Children with ID have a fivefold increased risk of sexual abuse; adolescent
females are at greatest risk [20]. Tools such as the Family Psychosocial Screen may help screen
families for concerns of abuse and maternal depression. (See "Child neglect: Evaluation and
management" and "Physical child abuse: Recognition".)
Medical and physical conditions — Numerous medical and physical conditions are commonly
associated with ID (table 3). Care should be taken to promptly identify and treat these conditions (if
warranted). (See "Intellectual disability in children: Definition, diagnosis, and assessment of needs",
section on 'Medical and physical conditions'.)
Environmental neurotoxic exposure — Neurotoxic environmental exposure can cause
measurable declines in intellectual functioning [21]. The most common environmental neurotoxin is
lead, which can harm cognitive function at low and high levels of exposure [22]. In the United States,
lead surveillance is recommended for all children with ID. This is discussed separately. (See
"Screening tests in children and adolescents", section on 'Lead poisoning' and "Intellectual disability in
children: Evaluation for a cause", section on 'Lead screening'.)
Sleep disorders — Sleep disorders are common in children with ID and can impact cognitive
performance, memory, learning, attention, and behavior. Sleep problems tend to be more severe in
children with ID compared with typical peers. Screening and intervening for sleep problems can
improve both individual as well as family functioning. Behavioral interventions should be individualized
with a goal of encouraging appropriate sleep hygiene, providing a supportive routine, and avoiding
inadvertent reinforcement of undesirable behaviors. Interventions need consistent application. Sleep
problems in children with ID tend to improve gradually with appropriate intervention and they may
escalate before improving. (See "Behavioral sleep problems in children".)
While most sleep issues in children with ID can be managed in the primary medical home, more
severe cases may need to referral to a sleep specialist. Sleep disorders can be more complex in the
setting of an underlying genetic syndrome causing ID. For example, children with SmithMagenis
syndrome may have insomnia, and those with PraderWilli or Down syndrome are at risk for
obstructive sleep apnea. These children usually require formal evaluation by a sleep specialist with
polysomnography. (See "Evaluation of suspected obstructive sleep apnea in children" and
"Microdeletion syndromes (chromosomes 12 to 22)", section on '17p11.2 deletion syndrome (Smith
Magenis syndrome)' and "Clinical features, diagnosis, and treatment of PraderWilli syndrome".)
Behavior intervention — Interventions for problem or disordered behavior can improve participation
and functioning in children with ID. Most children with ID benefit from interventions to improve
socialization skills and behavioral functioning. These should be individualized with the child's interests
in mind. They should be applied consistently to support optimal thinking, expression, behavior, and
adaptive functioning. Secure emotional attachment, parental warmth, and communicative social
interactions are relevant early goals.
The function served by a child's problem behavior (eg, an expression of frustration or need), its
antecedent triggers, and the reaction of others (such as inadvertent reinforcement of undesirable
behavior) should be considered. Desirable behaviors should be positively reinforced, and restraining
or punitive aversive approaches should be avoided [23]. Interventions should be appropriate to the
child's level of functioning (particularly language functioning). Specific techniques may include the
following:
Reducing identified triggers of problem behaviors. This may include modifying the child's
environment (eg, giving a child more space when an interfering behavior occurs in the context of
close proximity or reducing noise when noise is the trigger).
Reinforcing acceptable behaviors by rewarding the child with positive attention ("time in"
attention) or a desired reinforcer such as an appropriate treat or a preferred activity.
"Ignoring" misbehaviors so that a child no longer uses them to get attention (provided the
behaviors are not dangerous to the child or another person).
Redirecting the child's attention to preempt the expression of a problem behavior or extinguish it
early (eg, draw the child's attention to something of interest when a problem behavior appears
imminent).
Introducing appropriate behaviors that are incompatible with the problem behavior (eg, place the
child's hands in an appropriate position or activity that replaces their use in a problem behavior).
Promptly removing the child from an activity when a targeted problem behavior occurs (the child
is placed calmly and safely in "time out").
Various strategies for reducing problem behaviors can be helpful in schoolage children. A school
psychologist can assist with behavioral difficulties in the child's educational setting. Applied behavioral
analysis can improve staying on task and help to extinguish unwanted misbehaviors that challenge
participation in various settings [2]. Group psychotherapy may be helpful for children at school ages
with sufficient communication and intellectual functioning to meaningfully participate (a minimum of six
to seven years of ageequivalent intellectual functioning is usually necessary). In general, a group
intervention should be part of a comprehensive treatment program and involve a trained therapist.
Techniques need to be adapted to the child's level of functioning. Group therapy is most applicable to
adolescents and is particularly useful in social skills training.
Children with higher levels of selfesteem and independence tend to have fewer problem behaviors.
Transition interventions for youth should include problem solving, planning, issues of sexuality,
knowledge of rights, issues of transition into adult life, and preparation for independent community
living. (See 'Transition planning' below.)
Residential setting — In the United States, most individuals with ID live in integrated community
settings rather than in specialized residential care [24]. The community setting can be successful if
appropriate individualized health care, supports, and services are provided [25]. However, families of
children with ID and challenging behaviors can experience considerable stress [26]. Highquality
interventions are costly in both communitybased as well as specialized residential settings [27].
Residential programs or day support services can offer a structured setting that helps to manage
challenging behaviors, promotes participation in activities, provides individualized supports, and
permits respite for family [26]. Placement decisions are influenced by the age of the individual; their
abilities and functional profile; the nature and extent of needed supports; and environmental factors.
Deinstitutionalization has improved the lives of many individuals with ID. However, there appears to
be an increased mortality when individuals with ID are transferred from institutional to community care
[28,29]. Thus, necessary services, equipment, environmental modifications, caregiver training, and
readiness may need to be considered prior to transferring a child with ID and chronic health
conditions [30,31].
Transition planning — Transition planning refers to anticipating and facilitating the transition from
childhood to adulthood and promoting selfsufficiency. Included in this is the transition from pediatric
care services to adult services. For children with ID, transition planning should begin at or by age 12
years [32,33]. The Individuals with Disabilities Education Act (IDEA) mandates that affected students
have a transition plan by 16 years of age; however, earlier transition and vocational planning is
encouraged well before this age.
Early individualized discussions and interventions gradually prepare the child and family. These may
address issues of vocation, independent living/functioning, independent decisionmaking or
guardianship, care coordination, sexuality, reproduction, life expectancy, health insurance, eligibility
for adult communitybased services, medical management by different specialists, advance directives,
and communication preferences of the child or youth with ID. The child or youth with ID should be
involved as much as possible in the development of the plan and the decisionmaking process; his or
her level of understanding should be assessed and documented. Student advocacy and self
determination should be encouraged. The family should also be included. Youth with ID and autism
have greater identified needs for support after school, and are less likely to take an active leadership
role compared with those without autism [34].
A transition plan should be documented by age 14 years and reviewed annually thereafter [32]. A
transition plan focuses on interventions needed to attain transition readiness and accomplish
successful transition. It may include assessment of transition readiness, setting realistic goals, and
planning anticipated timelines. It is used to implement and monitor the transition process until 18
years of age, or the time of actual transition, which may be later.
Transition services may help individuals adjust from school to adult settings with occupational and
other activities, and prepare young people with ID for anticipated changes. Interventions may help
individuals to advocate for themselves. Supports may include a job coach, assistance with transport,
legal advocacy, supported employment, social security disability benefits and income, or assisted
living.
Transitionrelated resources for professionals include educational guide books, evaluation tools, and
checklists. Such tools are available at the following sites:
Carolina Health and Transition Project (CHAT)
Jacksonville Health and Transition Services (JaxHATS)
University of Wisconsin Waisman Center – Including a pocket health guide
National Health Care Transition Center – Got Transition
National Center for Medical Home Implementation – Transitions page
When the youth actually transitions to adult services and providers, the health record, a
comprehensive summary of medications, and the individualized collaborative care plan should
accompany the individual with ID. It is particularly important to indicate which specific communication
strategies are most effective for the individual.
Transition and posttransition functioning may be impacted by factors such as availability and access
to services, opportunities for housing and employment, access to transport, and the individual's family
system [35]. Other health conditions may also have a significant impact, and frequently make
transition more difficult. It is not unusual for individuals with chronic health conditions to experience a
decline in health after transition [36]. (See "Children and youth with special health care needs",
section on 'Transition planning'.)
In the United States, one initiative for improving the quality of transition and postsecondary programs
is the Transition Programs for Students with Intellectual Disabilities (TPSID) Program. Interventions
aim to advance intellectual and adaptive functioning, improve selfadvocacy, incorporate occupational
experience, and provide vocational skills. Additional information is provided through the United States
department of education website.
LEGISLATIVE MANDATES
In the United States, federal legislation supports a range of services for children with ID. The
Individuals with Disabilities Education Act (IDEA) provides for early intervention and special education
for children with disabilities from birth to 21 years of age. According to the Rehabilitation,
Comprehensive Services, and Developmental Disabilities Amendments of 1975 (PL 95602 of 1978),
individuals are considered to have disabilities if they have mental and/or physical impairment
occurring before 22 years of age that results in substantial functional limitation in at least three areas
of life activity [37].
Youth with ID and their families may need assistance in understanding the rights afforded to them by
federal laws, such as IDEA and the Affordable Care Act (ACA) and their application in the context of
other programs such as Medicaid, Title V, Supplemental Security Income (SSI), and Social Security
Disability Income (SSDI) [38]. Information is available on the United States department of Health and
Human Services website.
Early intervention — Early intervention is advised for children identified before three years of age
who have or are at risk of having disabilities, which are broadly defined. Infants and children are
eligible for early intervention services if they have an identified developmental delay or a condition
that puts them at risk (eg, Down syndrome, prematurity). Infants and children should be referred as
early as possible to the appropriate local agency. Referral can be made by a professional or
caregiver.
Early intervention provides comprehensive evaluation of the child's needs and functions. It provides
individualized multidisciplinary comprehensive services to support early learning and functional
progress. Early intervention services are usually provided in the child's home, but the location and
type of services can vary [39]. (See "Developmentalbehavioral surveillance and screening in primary
care", section on 'Early intervention or special education services'.)
Special education — Under IDEA, special education and related services can be provided for
children from 3 to 21 years of age. All children with disabilities are mandated to receive a
comprehensive education in the least restrictive environment, with priority given to the most severely
impaired children. IDEA requires the states to identify and evaluate eligible children and generate an
Individualized Education Plan (IEP) annually, with comprehensive, measurable, and appropriate
functional goals based on the child's current level of performance, and including specific services that
the school will provide for the child.
The legislation includes procedural safeguards with due process so that parents may advocate and
participate actively in their child's education. Requirements for the participation of physicians and the
extent of their participation vary among states. When it is not required by the state, parents may
choose to obtain a physician's evaluation.
Special education services offered by the school may include training in social skills, assistance in
selfhelp, vocational training, behavioral assistance, and academic program modifications. The
comprehensive educationallypertinent needs of the individual are considered; the intellectual quotient
(IQ) of the student should not solely direct decisionmaking. Children may be placed in a regular class
with supports or in a special education class, placed in resource classes, or an aide may also be
provided for the child.
Principles of least restrictive environment and inclusion must be applied to enable the child with ID to
participate with children who do not have a disability. Not all children can participate in regular
academic classes, but, depending on the extent of their disability, children may be included in many
other activities such as homeroom, physical education, lunch, recess, art, or music. Assistive
technology options can reduce the effect of the disability and improve functioning in communication,
daily living, mobility, communication, education, and vocational preparation. Other educational
settings are available for children who are unable to attend regular classes. These include a self
contained special education class with adaptive, selfhelp life skills training, a specialized day school,
or, in rare cases, a residential institutional placement.
According to the IDEA Improvement Act of 2004, an Individualized Transition Plan (ITP) must be
included in the student's annual IEP beginning at 16 years of age, although transition and vocational
planning is encouraged well before this. (See 'Transition planning' above.)
OUTCOME
Overview — ID is associated with lifelong impairments that have social, psychological, economic,
and other longterm consequences. The intelligence quotient (IQ) score is not an accurate measure of
longterm potential. The combination of adaptive and intellectual functioning in ID is more predictive of
outcomes than intellectual functioning or an IQ measure alone. The quality of life of individuals with ID
has improved considerably in settings where institutionalization is uncommon and support services
and employment opportunities are available. However, longterm outcomes among individuals with ID
suggest that there is a continued need to intervene for this population [36,40].
In general, individuals with ID are more likely to experience each of the following compared with
individuals without cognitive disabilities [2,36]:
Reduced overall extent of learning and education
Reduced levels of employment, occupational opportunity, and income, with disproportionately
reduced earnings
Increased longterm risk of poverty
Poorer, disparate longterm health outcomes
Reported functional outcomes in ID vary considerably. Studies often omit the most severely affected
individuals, resulting in more favorable estimates of outcomes [2].
Outcomes for children with ID depend on multiple factors, including:
Severity level – Individuals with mild ID generally have greater capacity to respond to
intervention than those with more severe ID. Thus, children with mild ID usually attain more
learning, speech and language, reading, interpersonal communication, and vocational skills than
individuals with severe ID. Other conditions that coexist with ID contribute to variable outcomes.
(See 'Outcomes according to severity level' below.)
Cause of ID – Different etiologies of ID can have distinct clinical profiles with a characteristic
course (eg, Rett syndrome) or range of functional outcomes (eg, Down syndrome, fragile X
syndrome, fetal alcohol syndrome). Establishing an accurate diagnosis helps with
prognostication. (See "Intellectual disability in children: Evaluation for a cause".)
Intellectual deficits may be distinctively worse in some causes of ID than in other causes of ID.
For example, individuals with Down syndrome have disproportionate deficits in executive
functioning, verbal working memory, and reaction times that exceed those of other individuals
with ID [2]. Such deficits may persist and can become more obvious over time, especially with
increased demands. However, outcomes can vary considerably even within the same etiology.
Most causes of ID do not result in progressive deterioration in function, though some genetic and
metabolic causes are expected to cause progressive worsening in function. In other cases,
cognitive deterioration can occur from another cause (eg, when a patient with ID has an
accidental brain injury or develops Alzheimer disease).
Newborn screening has been pivotal to improving outcomes in individuals with certain treatable
causes of ID (eg, inborn errors of metabolism) or concurrent impairing condition (eg, endocrine
abnormality). (See "Newborn screening".)
Coexisting conditions – Other comorbid conditions (eg, other developmental disabilities, mental
health problems, vision or hearing impairment, motor deficits, seizure disorder) may impair
progress, transition, and functional outcomes in individuals with ID. For example, in a child with
ID and cerebral palsy, gross motor impairment primarily determines mobility outcomes, while
cognitive impairment is important in determining the performance of daily living activities [41].
Persistent impairment due to autism impacts transition and posttransition outcomes. Behavioral
difficulties, substance abuse, obesity, depression, suicidal ideation and attempt, limited
understanding of risk and accidents, neglect, and abuse or harm perpetrated by others may also
impact the course and functioning of an individual with ID.
Environmental and social factors – Multiple environmental factors affect the natural history and
outcome of ID. Family, socioeconomic, and psychosocial factors may impact the child's course.
Social opportunities are important and caregiver support, expectations, attitudes, and motivation
can have an effect. Children with ID are more likely to be affected by poverty compared with
typically developing children incomes [6].
Access to services – The availability and provision of individualized supports (eg, early
intervention, educational, vocational, transition, and posttransition services) has a considerable
impact on outcomes for individuals with ID [37]. Deinstitutionalization has improved the lives of
many individuals with ID; however, specialized support needs have shifted into community
settings, which requires planning, financial provision, and comprehensive services. Appropriate
supports lessen the impact of disability and improve the functioning of individuals with ID. For
many children, developmental progress is manifested by stable, enduring gains in function, but
for others, the improvements are contingent upon sustaining support [1]. For youth with ID,
factors that impact the success of transitioning into adulthood include access, availability, and the
provision of needed interventions, the quality and extent of transition and postschool services,
necessary expertise in care, residential options, occupational opportunities, transport,
socioeconomic, and family factors [35]. Transition of individuals with ID is important to
subsequent progress and longterm functioning. (See 'Transition planning' above.)
Outcomes according to severity level — The level of severity in ID markedly influences short and
longterm outcomes. Severity is defined by the level of support needed, rather than by IQ scores
(table 4) [1]. (See "Intellectual disability in children: Definition, diagnosis, and assessment of needs",
section on 'Classification of severity'.)
Mild ID — Most affected children and adolescents have mild ID. Children with mild ID attain higher
levels of skills and progress at a faster pace than children with more severe ID. Children with mild ID
can be anticipated to gain skills at approximately onehalf to twothirds of the rate of children without
ID. Outcomes vary depending on coexisting conditions and the level of support provided.
Standardized test scores achieved by children with mild ID tend to decrease over time, compared with
typically developing children. However, some children with mild levels of global developmental delay
(GDD) who receive appropriate early intervention may make sufficient progress by five years of age
such that they may not meet criteria for ID. In addition, some children with ID can improve in their
adaptive functioning with appropriate individualized intervention, and no longer meet adaptive
impairment criteria required for an ID diagnosis.
Most children with mild ID need sustained academic supports to be able to learn and acquire age
typical conceptual skills, such as reading [1]. With appropriate supportive services, children with mild
ID usually accomplish reading at a third to sixth grade level by late adolescence [42]. However, some
adults with mild ID are functionally illiterate. Individuals with mild ID have difficulties grasping complex
information. Planning, problem solving, and money management need longterm support. Greater
gullibility, less mature judgment, and vulnerability to manipulation put children and adolescents with
mild ID at particular risk.
Children with mild ID have better social skills, communication, and social judgment than children with
more severe ID and they usually can relate to others, communicate in friendship, and display social
play and extracurricular interests. However, their interests and skill levels may be similar to those of
younger children. Social adaptive and communication outcomes vary depending on other coexisting
conditions. Children with autism have disproportionate impairment in social skills communication for
their level of cognitive functioning.
The longterm outcome of individuals with mild ID is related to their combined cognitive and adaptive
functioning. Most individuals with mild ID are able to accomplish independent daily living activities.
Many are able to live independently with provided supports, including decisionmaking for health,
nutrition, finances, or raising a family. Adults with mild ID can marry and become a parents, although
raising children is challenging.
Individuals with mild ID have the greatest potential for vocational placement, provided they receive
appropriate individualized services, and that other comorbidities aren't prohibitive. Some affected
adults can attain a simple regular position requiring straightforward occupational skills, or paid
employment in a competitive workplace, given appropriate supports. However, adults with low levels
of functional independence need sheltered employment and assisted living or a group home. In one
report of longterm followup of 52 individuals with mild ID followed from birth to adulthood, 73 percent
were married, 67 percent had a job, 62 percent had children, and 54 percent owned their own home
[43].
Moderate ID — Approximately 10 percent of individuals with ID have been estimated to have
moderate ID. Children with moderate ID are able to slowly make progress in skill development and
usually develop at approximately onethird to onehalf the rate of children without ID [42].
Schoolbased academic and social development gains are slow. Conceptual skills lag well behind
those of peers; substantial help or support is needed for conceptual tasks. Academic skills in adults
are typically at elementary level. Children with moderate ID learn to read essential words and signs
such as "stop," "exit," or "danger." With educational services, a child with moderate ID can learn to
read at a first to third grade level.
Simple spoken language and social friendships are possible, but significantly limited by social and
communication deficits. Social cues, social judgment, and social decisions need regular help and
support.
Longterm outcomes of moderate ID vary markedly, dependent on the presence and extent of
coexisting conditions and disorders that frequently complicate moderate ID. Most individuals with
moderate ID are capable of learning daily living activities and can achieve independent selfcare, with
sufficient teaching and support. Complex daily living tasks (eg, money management) need
considerable support. Most individuals are able to reside in a group home in a community setting with
support. The majority of adults with moderate ID need sheltered, supported, noncompetitive,
vocational positions with minimal communication and conceptual demands, although some are
capable of performing unskilled work. Few with moderate ID are able to marry or raise children.
Severe ID — Only 3 to 4 percent of individuals with ID are estimated to have severe ID. Children
with severe ID gain skills at a rate of approximately onequarter to onethird of that expected for
children without ID, and approximately half the pace of children with mild ID.
Individuals with severe ID have little understanding of written language or number, time, or money
concepts; caretakers provide extensive help in problem solving.
Individuals with severe ID derive benefit from healthy, socially supportive interactions with familiar
people who relate at the level of the individual's development. Speech development may be delayed
until four to five years of age, although some do not develop speech; individuals may use a basic
single word or phrase or gesture to communicate direct experience.
Most individuals with severe ID require training in social and selfhelp daily living skills in high school,
and may be trainable in some basic daily living activities, while requiring significant ongoing support
and supervision. Adults without prohibitive coexisting conditions may be able to participate in
sheltered vocational settings and, if not behaviorally disruptive, reside in a group home in the
community with appropriate supports. The majority of adults with severe ID need substantial
assistance with daily living and social skills. In general, severely affected individuals do not marry or
raise children [42]. Severe ID is associated with a reduced lifespan.
Profound ID — Approximately 1 to 2 percent of children with ID have profound impairment.
Individuals with profound ID may express themselves nonverbally and understand a simple
instruction, gesture, or emotional cue. However, outcomes are very variable, and can vary from total
dependence to some simple expression and social responsiveness; some individuals learn simple
selfcare in dressing or feeding.
Individuals with profound ID are usually dependent in all aspects of daily living and have pervasive
need of supports. Some affected individuals benefit from training in selfhelp skills, though gains are
very slow. Individuals may use objects in a goaldirected fashion in selfcare or simple recreation.
Most need high school classes in life skills and comprehensive assistance. Individuals with profound
ID do not learn to read. Concurrent sensory and physical limitations are common, and coexisting
medical conditions may need additional equipment. Medical needs, coexisting conditions, and
behavioral characteristics impact placement decisions for individuals with profound ID [42].
Profound ID is associated with reduced life expectancy, though the extent depends on the cause,
complicating disorders, and the individual's mobility. In one report of 7060 people with profound ID, life
expectancy was considerably higher among individuals who were mobile (even if unable to walk) and
capable of eating with assistance compared with individuals who were immobile and/or dependent on
tube feeding (23 versus 4 to 8 years) [44].
PREVENTION
Most causes of ID cannot be prevented. Preventive strategies include:
Primary prevention – Primary prevention describes measures to prevent ID by addressing the
conditions that cause or contribute to ID. There are numerous examples of primary preventive
strategies for ID, some notable examples include:
• Reducing prenatal alcohol exposure (see "Alcohol intake and pregnancy")
• Ensuring adequate folic acid intake during pregnancy (see "Folic acid supplementation in
pregnancy")
• Reducing the risk of congenital infection (see "Overview of TORCH infections", section on
'Screening for TORCH infections')
• Reducing lead exposure in infants and children (see "Screening tests in children and
adolescents", section on 'Lead poisoning')
• Measures aimed at reducing poverty
For genetic forms of ID, appropriate genetic testing and counselling, including estimates of
recurrence risks, can facilitate informed decisionmaking about reproductive options in the future.
Preimplantation genetic diagnosis and prenatal diagnosis is possible for some forms of genetic
ID. (See "Preimplantation genetic testing".)
Secondary prevention – Secondary prevention is directed at treating an underlying condition to
minimize the risk of impairment in ID. Examples include:
• Dietary restriction or other interventions in children with treatable inborn errors of metabolism
(eg, phenylketonuria, galactosemia). (See "Overview of phenylketonuria" and "Galactosemia:
Management and complications".)
• Thyroid hormone replacement in a child with hypothyroidism. (See "Treatment and prognosis
of congenital hypothyroidism" and "Acquired hypothyroidism in childhood and adolescence",
section on 'Treatment and prognosis'.)
• Treatment of vision and hearing impairments that may be associated with ID. (See "Vision
screening and assessment in infants and children" and "Hearing loss in children:
Treatment".)
• Management of seizures and other coexisting medical disorders. (See "Seizures and
epilepsy in children: Initial treatment and monitoring".)
Tertiary prevention – Tertiary prevention is aimed at maximizing function and quality of life by
attempting to reduce functional disability. This approach includes all of the interventions
described in the preceding sections. (See 'Management' above.)
SUMMARY AND RECOMMENDATIONS
The overall goals of management of intellectual disability (ID) are to lessen the effects of disability
and improve functioning; to prevent or minimize further deterioration relative to peers; and to
promote optimal functioning in the child's home, school, vocational, and community settings.
Interventions should begin early and should be individualized and be sustained. The approach
should be collaborative and multidisciplinary. Depending on the individual's needs, intervention
may include speech and language therapy, occupational therapy, physical therapy and
rehabilitation, vision and hearing services, family counseling and support, behavioral intervention,
interventions to improve social participation, educational assistance, community integration,
independent functioning and life skills learning, advocacy, and other aids. Effective
communication approaches should be used. (See 'General considerations' above and 'Early
interventions for ID' above.)
For children with ID, longitudinal developmental surveillance expands upon standard pediatric
surveillance and initial diagnosis of ID. It is coupled with direct interventions during the course of
a child's life so as to continue to advance and strengthen functional progress. (See 'Longitudinal
developmental surveillance' above.)
Most causes of ID do not have a specific treatment or intervention. However, if a treatable
underlying cause of ID is identified (eg, congenital hypothyroidism, treatable inborn error of
metabolism), the child should be promptly treated to prevent further disability. (See 'Treatment of
specific causes of ID' above.)
Interventions for other neurodevelopmental or mental health disorders associated with ID include
family counseling, education, conditionspecific behavioral interventions, and
psychopharmacologic therapy. The use of medications should be part of a clear comprehensive
psychosocial treatment plan that includes appropriate behavioral intervention, considers risks and
benefits, identifies baseline behavioral functioning, target behaviors and treatment goals, and
provides evidencebased treatment with baseline and serial monitoring. (See
'Neurodevelopmental disorders and mental health problems' above.)
Behavior interventions are usually needed to improve social and behavioral functioning in
children with ID and to address behavioral difficulties. These should be individualized and applied
consistently. Desirable behaviors should be positively reinforced, and restraining or punitive
aversive approaches should be avoided. (See 'Behavior intervention' above.)
In the United States, federal legislation supports a range of services for children with ID. The
Individuals with Disabilities Education Act (IDEA) provides for early intervention and special
education for children with disabilities from birth to 21 years of age. All children with disabilities
are mandated to receive a comprehensive education in the least restrictive environment, using an
Individualized Education Plan (IEP), with measurable goals that are tailored to the individual's
needs and abilities. Transition planning discussion should begin at or by 12 years of age, with a
written transition plan in the child's record by 16 years of age. (See 'Legislative mandates' above
and 'Transition planning' above.)
The outcome for ID is variable and depends on multiple factors such as the cause and severity of
ID, the presence and severity of other associated conditions, environmental and social factors,
and access to appropriate individualized services. (See 'Outcome' above.)
Use of UpToDate is subject to the Subscription and License Agreement.
REFERENCES
1. American Psychiatric Association. Intellectual Disability (Intellectual Developmental Disorder). I
n: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, American Psychiatric Ass
ociation (Ed), Arlington, VA 2013. p.33.
2. Grieco J, Pulsifer M, Seligsohn K, et al. Down syndrome: Cognitive and behavioral functioning
across the lifespan. Am J Med Genet C Semin Med Genet 2015; 169:135.
3. Treatment decisions regarding infants, children and adolescents. Paediatr Child Health 2004;
9:99.
4. Tobon JI, Reid GJ, Brown JB. Continuity of Care in Children's Mental Health: Parent, Youth and
Provider Perspectives. Community Ment Health J 2015; 51:921.
5. Frank K, Esbensen AJ. Fine motor and selfcare milestones for individuals with Down syndrome
using a Retrospective Chart Review. J Intellect Disabil Res 2015; 59:719.
6. Phillips KL, Schieve LA, Visser S, et al. Prevalence and impact of unhealthy weight in a national
sample of US adolescents with autism and other learning and behavioral disabilities. Matern
Child Health J 2014; 18:1964.
7. Taheri A, Perry A, Minnes P. Examining the social participation of children and adolescents with
Intellectual Disabilities and Autism Spectrum Disorder in relation to peers. J Intellect Disabil Res
2016; 60:435.
8. Andrews J, Falkmer M, Girdler S. Community participation interventions for children and
adolescents with a neurodevelopmental intellectual disability: a systematic review. Disabil
Rehabil 2015; 37:825.
9. Adair B, Ullenhag A, Keen D, et al. The effect of interventions aimed at improving participation
outcomes for children with disabilities: a systematic review. Dev Med Child Neurol 2015;
57:1093.
10. Barton EE, Reichow B, Schnitz A, et al. A systematic review of sensorybased treatments for
children with disabilities. Res Dev Disabil 2015; 37:64.
11. van Karnebeek CD, Houben RF, Lafek M, et al. The treatable intellectual disability APP
www.treatableid.org: a digital tool to enhance diagnosis & care for rare diseases. Orphanet J
Rare Dis 2012; 7:47.
12. EvidenceBased Interventions Specific to Children With FASDs. Available at: https://www.aap.or
g/enus/advocacyandpolicy/aaphealthinitiatives/fetalalcoholspectrumdisorderstoolkit/Page
s/EvidenceBased.aspx (Accessed on January 10, 2017).
13. Weitzman C, Wegner L, Section on Developmental and Behavioral Pediatrics, et al. Promoting
optimal development: screening for behavioral and emotional problems. Pediatrics 2015;
135:384.
14. Szymanski L, King BH. Practice parameters for the assessment and treatment of children,
adolescents, and adults with mental retardation and comorbid mental disorders. American
Academy of Child and Adolescent Psychiatry Working Group on Quality Issues. J Am Acad
Child Adolesc Psychiatry 1999; 38:5S.
15. Tyrer P, Cooper SA, Hassiotis A. Drug treatments in people with intellectual disability and
challenging behaviour. BMJ 2014; 349:g4323.
16. Szymanski L, King BH. Summary of the Practice Parameters for the Assessment and Treatment
of Children, Adolescents, and Adults with Mental Retardation and Comorbid Mental Disorders.
American Academy of Child and Adolescent Psychiatry. J Am Acad Child Adolesc Psychiatry
1999; 38:1606.
17. Glover G, Bernard S, Branford D, et al. Use of medication for challenging behaviour in people
with intellectual disability. Br J Psychiatry 2014; 205:6.
18. Rodnitzky RL. Druginduced movement disorders in children and adolescents. Expert Opin Drug
Saf 2005; 4:91.
19. Christian CW, Committee on Child Abuse and Neglect, American Academy of Pediatrics. The
evaluation of suspected child physical abuse. Pediatrics 2015; 135:e1337.
20. Wissink IB, van Vugt E, Moonen X, et al. Sexual abuse involving children with an intellectual
disability (ID): a narrative review. Res Dev Disabil 2015; 36:20.
21. Rauh VA, Margolis AE. Research Review: Environmental exposures, neurodevelopment, and
child mental health new paradigms for the study of brain and behavioral effects. J Child
Psychol Psychiatry 2016; 57:775.
22. www.cdc.gov/nceh/lead/ACCLPP/activities.htm (Accessed on October 16, 2016).
23. Zuromski ES. The Management of Mental Retardation. In: Static Encephalopathies of Infancy a
nd Childhood, Miller G, Ramer JC (Eds), Raven Press, New York 1992. p.119.
24. McConkey R, Kelly F, Mannan H, Craig S. Moving from family care to residential and supported
accommodation: national, longitudinal study of people with intellectual disabilities. Am J Intellect
Dev Disabil 2011; 116:305.
25. Kozma A, Mansell J, BeadleBrown J. Outcomes in different residential settings for people with
intellectual disability: a systematic review. Am J Intellect Dev Disabil 2009; 114:193.
26. Brown RI, Geider S, Primrose A, Jokinen NS. Family life and the impact of previous and present
residential and day care support for children with major cognitive and behavioural challenges: a
dilemma for services and policy. J Intellect Disabil Res 2011; 55:904.
27. Mansell J, BeadleBrown J, Special Interest Research Group. Deinstitutionalisation and
community living: position statement of the Comparative Policy and Practice Special Interest
Research Group of the International Association for the Scientific Study of Intellectual
Disabilities. J Intellect Disabil Res 2010; 54:104.
28. Kastner T, Nathanson R, Friedman DL. Mortality among individuals with mental retardation living
in the community. Am J Ment Retard 1993; 98:285.
29. Shavelle R, Strauss D. Mortality of persons with developmental disabilities after transfer into
community care: a 1996 update. Am J Ment Retard 1999; 104:143.
30. Friedman SL, Kalichman MA, Council on Children with Disabilities, Council on Children with
Disabilities. Outofhome placement for children and adolescents with disabilities. Pediatrics
2014; 134:836.
31. AAP Policy Statement, AAP Policy, American Academy of Pediatrics. November 1, 2014. Volum
e 134, Issue 5. http://pediatrics.aappublications.org/site/aappolicy/index.xhtml (Accessed on No
vember 06, 2014).
32. American Academy of Pediatrics, American Academy of Family Physicians, American College
of Physicians, et al. Supporting the health care transition from adolescence to adulthood in the
medical home. Pediatrics 2011; 128:182.
33. Stein DS, Blum NJ, Barbaresi WJ. Developmental and behavioral disorders through the life
span. Pediatrics 2011; 128:364.
34. Shogren KA, Plotner AJ. Transition planning for students with intellectual disability, autism, or
other disabilities: data from the National Longitudinal Transition Study2. Intellect Dev Disabil
2012; 50:16.
35. Foley KR, Dyke P, Girdler S, et al. Young adults with intellectual disability transitioning from
school to postschool: a literature review framed within the ICF. Disabil Rehabil 2012; 34:1747.
36. Queirós FC, Wehby GL, Halpern CT. Developmental disabilities and socioeconomic outcomes in
young adulthood. Public Health Rep 2015; 130:213.
37. Shevell M, Ashwal S, Donley D, et al. Practice parameter: evaluation of the child with global
developmental delay: report of the Quality Standards Subcommittee of the American Academy
of Neurology and The Practice Committee of the Child Neurology Society. Neurology 2003;
60:367.
38. The Patient Protection and Affordable Care Act. Available at: http://www.gpo.gov/fdsys/pkg/PLA
W111publ148/pdf/PLAW111publ148.pdf (Accessed on September 21, 2016).
39. Palfrey, JS. Legislation for the education of children with disabilities. in: DevelopmentalBehavior
al Pediatrics, 4th ed, Levine, MD, Carey WB, Crocker, AC (Eds), Saunders Elsevier, Philadelphi
a 2009. p.972.
40. Balogh R, McMorris CA, Lunsky Y, et al. Organising healthcare services for persons with an
intellectual disability. Cochrane Database Syst Rev 2016; 4:CD007492.
41. Vos RC, Becher JG, Ketelaar M, et al. Developmental trajectories of daily activities in children
and adolescents with cerebral palsy. Pediatrics 2013; 132:e915.
42. Accardo PM, Capute AJ. Mental Retardation. In: Developmental Disabilities in Infancy and Child
hood, 2nd, Capute AJ, Accardo PM (Eds), Paul Brookes, Baltimore 1996. p.211.
43. Hall I, Strydom A, Richards M, et al. Social outcomes in adulthood of children with intellectual
impairment: evidence from a birth cohort. J Intellect Disabil Res 2005; 49:171.
44. Eyman RK, Grossman HJ, Chaney RH, Call TL. The life expectancy of profoundly handicapped
people with mental retardation. N Engl J Med 1990; 323:584.
Topic 6191 Version 24.0
GRAPHICS
Adaptive skills used to define and determine severity of intellectual disability
Adaptive
Skills
domain
Conceptual These skills include language, reading, and writing (literacy); money, time, and number concepts
(mathematics); reasoning; memory; selfdirection; and judgment in novel situations.
Social These skills include interpersonal social communication, empathy, ability to relate to peers as friends,
social problemsolving, social responsibility, and selfesteem. Gullibility, the ability to follow rules, and
avoiding victimization may also be included.
Practical These skills include activities of personal care or daily living, such as eating, dressing, mobility, and
toileting. Additional skills may include following a schedule or routine, using a telephone, managing
money, preparing meals, occupational skills, and abilities in transportation/travel, health care, and
safety.
A diagnosis of intellectual disability (ID) requires impaired intellectual AND adaptive functioning in at least one of these
domains. Impairment in ID generally affects participation in multiple settings (home, community, and/or school) and
requires support. The severity of ID is defined according to the level of adaptive impairment and the level of supports
needed.
Adapted from the following sources:
1. American Psychiatric Association. Intellectual Disability (Intellectual Developmental Disorder). In: Diagnostic and
Statistical Manual of Mental Disorders, Fifth Edition, American Psychiatric Association.
2. American Association of Intellectual and Developmental Disabilities (AAIDD), Definition of Intellectual Disability,
available at: http://aaidd.org/intellectualdisability/definition (Accessed on July 17, 2018).
Graphic 90174 Version 2.0
Examples of instruments commonly used in the assessment of children and
adolescents with suspected intellectual disability (ID) or global developmental delay
(GDD)
Examples of commonly used instruments Applicable ages
Infants and young children <2 years
Cognitive and adaptive testing
Bayley Scales of Infant and Toddler Development, 3 rd Edition (BSIDIII)* 1 to 42 months

(2005)
Griffiths Scales of Child Development, 3 rd Edition (Griffiths III) ¶ (2015) Birth to 72 months
Adaptive testing
The Vineland Adaptive Behavior Scale, 3 rd Edition (VinelandIII) Δ (2016) Birth to beyond 21 years
The Adaptive Behavior Assessment System, 3 rd Edition (ABASIII) ◊ (2015) Birth to beyond 21 years
Preschool age
Intellectual testing
Wechsler Preschool and Primary Scale of Intelligence, 4 th Edition (WPPSIIV) 2 years, 6 months to 7 years, 7

(2012) months
StanfordBinet Intelligence Scales, 5 th Scales, 5 th Edition (SB5) (2003) 2 to beyond 21 years
Differential Abilities Scales, 2 nd Edition (DASII) (2007) 2 years, 6 months to 17 years
Kaufman Assessment Battery for Children, 2 nd Edition (KABCII) (2004) 3 to 18 years
Leiter International Performance Scale, 3 rd Edition (Leiter3) (2013) 3 to beyond 21 years
Adaptive testing
The Diagnostic Adaptive Behavior Scale (DABS) ◊ § 4 to 21 years
School age and beyond
Intellectual testing
Wechsler Preschool and Primary Scale of Intelligence, 4 th Edition (WPPSIIV) 2 years, 6 months to 7 years, 7

(2012) months
StanfordBinet Intelligence Scales, 5 th Scales, 5 th Edition (SB5) (2003) 2 to beyond 21 years
Wechsler Intelligence Scales for Children, 5 th Edition (WISCV) (2014) 6 to 16 years, 11 months
Differential Abilities Scales, 2 n d Edition (DASII) (2007) 2 years, 6 months to 17 years
Kaufman Assessment Battery for Children, 2 nd Edition (KABCII) (2004) 3 to 18 years
Leiter International Performance Scale, 3 rd Edition (Leiter3) (2013) 3 to beyond 21 years
Test of Nonverbal Intelligence, 4 th Edition (TONI 4) ￥ (2010) 6 to beyond 21 years
Wechsler Adult Intelligence Scale, 4 th Edition (WAISCIV) (2008) 16 to beyond 21 years
Adaptive testing
The Diagnostic Adaptive Behavior Scale (DABS) ◊ § 4 to 21 years
This table lists commonly used examples. The publication dates are per provider websites (last accessed June 2018).
* Standardized in the United States and United Kingdom; provides cognitive, language, and motor scores, and assesses social
emotional and adaptive behavior up to 42 months (3.5 years).
¶ Standardized in the United Kingdom; the 2015 version replaces older versions commonly used internationally; examines the
foundations of learning, language and communication, eye hand coordination (includes fine motor and visual perception),
gross motor, and personalsocialemotional (includes independent strengths and needs) up to 72 months (6 years).
Δ Most commonly used caregiverreported adaptive tool; available in both English and Spanish; the 2016 version replaces the
commonly used VinelandII (2006).
◊ These instruments have capacity to identify adaptive impairment in a single domain as defined by the Diagnostic and
Statistical Manual of Mental Disorders, Fifth Edition (DSM5).
§ The American Association on Intellectual and Developmental Disabilities (AAIDD) DABS provides conceptual, social, and
practical domain scores and a total score. (https://aaidd.org/dabs#.WxWt1u4vyM8).
￥ A test of nonverbal intelligence.
Conditions and disorders commonly associated with intellectual disability in children
Developmental and mental health disorders
Autism spectrum disorder
Attention deficit hyperactivity disorder
Depressive and anxiety disorders
Eating and feeding disorders (eg, pica and rumination disorder)
Learning disorders
Movement disorders (stereotypic movements, developmental coordination disorder)
Posttraumatic stress disorder
Selfinjurious behavior
Substance abuse
Medical and physical conditions
Cerebral palsy and other motor impairments
Congenital heart defects (eg, atrioventricular canal defects in children with Down syndrome)
Constipation
Dental caries
Endocrine abnormalities (eg, hypothyroidism, short stature)
Gastroesophageal reflux disease
Hearing loss
Lead poisoning
Obesity
Seizures
Sleep disorders (behavioral sleep problems and/or obstructive sleep apnea)
Undescended testes
Vision impairment (eg, strabismus, cataracts, refractive errors)
Other
Physical and sexual abuse
Typical adaptive needs and supports according to severity of intellectual disability
Severity level Adaptive skill domains
DSM5 AAIDD
Conceptual Social Practical
categories categories
Mild Intermittent Children require academic Social skills and personal Most individuals achieve

supports to learn skills judgement are immature independence in daily
expected for age. Adults for age. The individual is living and personal care
may have difficulties with at risk of being activities; most are
functional academic skills manipulated by others employable in jobs
such as planning, (gullibility). requiring simple skills and
reading, and money are often able to live
management. independently. They
typically need support for
making decisions in
health care, nutrition,
shopping, finances, and
raising a family.
Moderate Limited For children, conceptual Successful friendships Most individuals are

and academic skills lag with family/friends are capable of personal care
well behind those of possible using simple activities with sufficient
peers. For adults, spoken language, but the teaching and support, and
academic skills are individual is limited by achieve independent self
typically attainable at an deficits in social and care with moderate
elementary level. communicative skills. supports, such as is
Complex tasks such as Social cues, social available in a group
money management judgment, and social and home. Adults may be
need substantial support. life decisions regularly employable in a
need support. supported environment.
Severe Extensive Individuals have little Individuals benefit from Individuals are trainable

understanding of written healthy supportive in some basic activities of
language, or number, interactions with daily living with
time, and money family/familiar people and significant ongoing
concepts. Caretakers may use very basic single support and supervision.
provide extensive words, phrases, or
supports for problem gestures pertinent to their
solving. direct experience.
Profound Pervasive Individuals may use Although understanding Individuals are typically

objects in a goaldirected of symbolic dependent upon support
fashion for selfcare and communication is very for all activities of
recreation. limited, individuals may everyday living. Co
understand some occurring sensory or
gestures and emotional physical limitations are
cues, and can express common.
themselves nonverbally.
This table provides examples of typical adaptive needs and supports according to the severity of ID. The severity of ID
is defined by the level of adaptive impairment and the level of support needed. The American Psychiatric Association's
DSM5 categorizes adaptive impairment from mild to profound. [1] The AAIDD uses categories of intermittent, limited,
extensive, and pervasive. [2] Supports are wideranging and aim to optimize the functioning, participation, and
independence of the child in everyday environmental settings, including home, school, community. Profoundly
impaired children usually require pervasive support.
ID: intellectual disability; DSM5: Diagnostic and Statistical Manual, 5th Edition; AAIDD: American Association on Intellectual
and Developmental Disabilities.
Adapted from the following sources:
1. American Psychiatric Association. Intellectual Disability (Intellectual Developmental Disorder). In: Diagnostic and
Statistical Manual of Mental Disorders, Fifth Edition, American Psychiatric Association.
2. American Association of Intellectual and Developmental Disabilities (AAIDD). Definition of Intellectual Disability.
Available at: http://aaidd.org/intellectualdisability/definition (Accessed on July 17, 2018).
Contributor Disclosures
Penelope Pivalizza, MD Nothing to disclose Marc C Patterson, MD, FRACP Grant/Research/Clinical Trial
Support: Orphazyme [Niemann-Pick disease, type C (Arimoclomol)]; Shire [Metachromatic leukodystrophy
(Enzyme replacement therapy)]. Consultant/Advisory Boards: Actelion [Niemann-Pick C (Miglustat)]; Agios
[CGD]; Alexion [General lysosomal diseases, lysosomal acid lipase deficiency (Sebelipase alfa)]; Amicus [Fabry,
Gaucher, Pompe (Migalastat)]; Cerecor [Congenital disorders of glycosylation]; IntraBio [General lysosomal
diseases and ataxic disorders]; Novartis [Multiple sclerosis (Sphingolimod)]; Orphazyme [Niemann-Pick disease,
type C]; Shire [Metachromatic leukodystrophy]; Vtesse [Niemann-Pick disease, type C (Cyclodextrin)]. Equity
Ownership/Stock Options: IntraBio [General lysosomal diseases and ataxic disorders]. Other Financial Interest:
Sage [Honorarium as Editor-in-Chief of Journal of Child Neurology and Child Neurology Open]. Helen V Firth,
DM, FRCP, DCH Nothing to disclose Carolyn Bridgemohan, MD Nothing to disclose Carrie Armsby, MD,
MPH Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must conform to
UpToDate standards of evidence.
Conflict of interest policy

Intellectual Disability in Children - Ma... T, Outcomes, and Prevention - UpToDate

Hochgeladen von

Dokumentinformationen

Originaltitel

Copyright

Verfügbare Formate

Dieses Dokument teilen

Dokument teilen oder einbetten

Freigabeoptionen

Stufen Sie dieses Dokument als nützlich ein?

Sind diese Inhalte unangemessen?

Copyright:

Verfügbare Formate

Intellectual Disability in Children - Ma... T, Outcomes, and Prevention - UpToDate

Hochgeladen von

Copyright:

Verfügbare Formate

3/13/2019 Intellectual disability in children: Management, outcomes, and prevention - UpToDate

Bayley Scales of Infant and Toddler Development, 3 rd Edition (BSID­III)* 1 to 42 months

Griffiths Scales of Child Development, 3 rd Edition (Griffiths III) ¶ (2015) Birth to 72 months

The Vineland Adaptive Behavior Scale, 3 rd Edition (Vineland­III) Δ (2016) Birth to beyond 21 years

The Adaptive Behavior Assessment System, 3 rd Edition (ABAS­III) ◊ (2015) Birth to beyond 21 years

Wechsler Preschool and Primary Scale of Intelligence, 4 th Edition (WPPSI­IV) 2 years, 6 months to 7 years, 7

Stanford­Binet Intelligence Scales, 5 th Scales, 5 th Edition (SB­5) (2003) 2 to beyond 21 years

Differential Abilities Scales, 2 nd Edition (DAS­II) (2007) 2 years, 6 months to 17 years

Kaufman Assessment Battery for Children, 2 nd Edition (KABC­II) (2004) 3 to 18 years

Leiter International Performance Scale, 3 rd Edition (Leiter­3) (2013) 3 to beyond 21 years

The Vineland Adaptive Behavior Scale, 3 rd Edition (Vineland­III) Δ (2016) Birth to beyond 21 years

The Adaptive Behavior Assessment System, 3 rd Edition (ABAS­III) ◊ (2015) Birth to beyond 21 years

Wechsler Preschool and Primary Scale of Intelligence, 4 th Edition (WPPSI­IV) 2 years, 6 months to 7 years, 7

Stanford­Binet Intelligence Scales, 5 th Scales, 5 th Edition (SB­5) (2003) 2 to beyond 21 years

Wechsler Intelligence Scales for Children, 5 th Edition (WISC­V) (2014) 6 to 16 years, 11 months

Differential Abilities Scales, 2 n d Edition (DAS­II) (2007) 2 years, 6 months to 17 years

Kaufman Assessment Battery for Children, 2 nd Edition (KABC­II) (2004) 3 to 18 years

Leiter International Performance Scale, 3 rd Edition (Leiter­3) (2013) 3 to beyond 21 years

Test of Nonverbal Intelligence, 4 th Edition (TONI 4) ￥ (2010) 6 to beyond 21 years

Wechsler Adult Intelligence Scale, 4 th Edition (WAISC­IV) (2008) 16 to beyond 21 years

The Vineland Adaptive Behavior Scale, 3 rd Edition (Vineland­III) Δ (2016) Birth to beyond 21 years

The Adaptive Behavior Assessment System, 3 rd Edition (ABAS­III) ◊ (2015) Birth to beyond 21 years

Mild Intermittent Children require academic Social skills and personal Most individuals achieve

Moderate Limited For children, conceptual Successful friendships Most individuals are

Severe Extensive Individuals have little Individuals benefit from Individuals are trainable

Profound Pervasive Individuals may use Although understanding Individuals are typically

Conﬂict of interest policy

Das könnte Ihnen auch gefallen

Bayley Scales of Infant and Toddler Development, 3 rd Edition (BSIDIII)* 1 to 42 months

The Vineland Adaptive Behavior Scale, 3 rd Edition (VinelandIII) Δ (2016) Birth to beyond 21 years

The Adaptive Behavior Assessment System, 3 rd Edition (ABASIII) ◊ (2015) Birth to beyond 21 years

Wechsler Preschool and Primary Scale of Intelligence, 4 th Edition (WPPSIIV) 2 years, 6 months to 7 years, 7

StanfordBinet Intelligence Scales, 5 th Scales, 5 th Edition (SB5) (2003) 2 to beyond 21 years

Differential Abilities Scales, 2 nd Edition (DASII) (2007) 2 years, 6 months to 17 years

Kaufman Assessment Battery for Children, 2 nd Edition (KABCII) (2004) 3 to 18 years

Leiter International Performance Scale, 3 rd Edition (Leiter3) (2013) 3 to beyond 21 years

The Vineland Adaptive Behavior Scale, 3 rd Edition (VinelandIII) Δ (2016) Birth to beyond 21 years

The Adaptive Behavior Assessment System, 3 rd Edition (ABASIII) ◊ (2015) Birth to beyond 21 years

Wechsler Preschool and Primary Scale of Intelligence, 4 th Edition (WPPSIIV) 2 years, 6 months to 7 years, 7

StanfordBinet Intelligence Scales, 5 th Scales, 5 th Edition (SB5) (2003) 2 to beyond 21 years

Wechsler Intelligence Scales for Children, 5 th Edition (WISCV) (2014) 6 to 16 years, 11 months

Differential Abilities Scales, 2 n d Edition (DASII) (2007) 2 years, 6 months to 17 years

Kaufman Assessment Battery for Children, 2 nd Edition (KABCII) (2004) 3 to 18 years

Leiter International Performance Scale, 3 rd Edition (Leiter3) (2013) 3 to beyond 21 years

Wechsler Adult Intelligence Scale, 4 th Edition (WAISCIV) (2008) 16 to beyond 21 years

The Vineland Adaptive Behavior Scale, 3 rd Edition (VinelandIII) Δ (2016) Birth to beyond 21 years

The Adaptive Behavior Assessment System, 3 rd Edition (ABASIII) ◊ (2015) Birth to beyond 21 years