Mental retardation/Intellectual disability: Classification of Intellectual

disability

(Profound, Severe, moderate and mild),

Clinical Features, Epidemiology, Comorbidity, Etiology, Course and Prognosis

Intellectual disability can be caused by various environmental and genetic factors that lead to a
combination of cognitive and social impairments. The American Association on Intellectual and
Developmental Disability (AAIDD) defines intellectual disability as a disability characterized by
significant limitations in both intellectual functioning (reasoning, learning, and problem-solving)
and in adaptive behavior (conceptual, social, and practical skills) that emerges before the age of
18 years. Wide acceptance of this deϧnition has led to the international consensus that an
assessment of both social adaptation and intelligence quotient (IQ) is necessary to determine the
level of intellectual disability.

In the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5), various
levels of severity of intellectual disability are determined on the basis of adaptive functioning,
not on IQ scores. DSM-5 has adopted this change in emphasis from prior diagnostic manuals
because adaptive functioning determines the level of support that is required. Furthermore, IQ
scores are less valid in the lower portions of the IQ range. Making a determination of severity
level of intellectual disability, according to DSM-5, includes assessment of functioning in a
conceptual domain (e.g., academic skills), a social domain (e.g., relationships), and a practical
domain (e.g., personal hygiene).

The AAIDD promotes a view of intellectual disability as a functional interaction between an

individual and the environment, rather than a static designation of a person’s limitations. Within
this conceptual framework, a child or adolescent with intellectual disability is determined to need
intermittent, limited, extensive, or pervasive “environmental support” with respect to a specific
set of adaptive function domains. These include communication, self-care, home living, social or
interpersonal skills, use of community resources, self-direction, functional academic skills, work,
leisure, health, and safety.
CLASSIFICATION

DSM-5 criteria for intellectual disability include significantly subaverage general intellectual
functioning associated with concurrent impairment in adaptive behavior, manifested before the
age of 18. The diagnosis is made independent of coexisting physical or mental disorders.
Mental retardation (F70–F79) ICD-10

A condition of arrested or incomplete development of the mind, which is especially characterized

by impairment of skills manifested during the developmental period, skills which contribute to
the overall level of intelligence, i.e. cognitive, language, motor and social abilities. Retardation
can occur with or without any other mental or physical condition. Degrees of mental retardation
are conventionally estimated by standardized intelligence tests. These can be supplemented by
scales assessing social adaptation in a given environment. These measures provide an
approximate indication of the degree of mental retardation. The diagnosis will also depend on the
overall assessment of intellectual functioning by a skilled diagnostician. Intellectual abilities and
social adaptation may change over time, and, however poor, may improve as a result of training
and rehabilitation. Diagnosis should be based on the current levels of functioning. Use additional
code, if desired, to identify associated conditions such as autism, other developmental disorders,
epilepsy, conduct disorders or severe physical handicap.

The following fourth-character subdivisions are for use with categories F70–F79 to identify the
extent of impairment of behaviour:

.0 With the statement of no, or minimal, impairment of behaviour

.1 Significant impairment of behaviour requiring attention or treatment

.8 Other impairments of behaviour

.9 Without mention of impairment of behaviour

F70 Mild mental retardation

Approximate IQ range of 50 to 69 (in adults, mental age from 9 to under 12 years). Likely to
result in some learning difficulties in school. Many adults will be able to work and maintain
good social relationships and contribute to society. Incl.: feeble-mindedness mild mental
subnormality

F71 Moderate mental retardation

Approximate IQ range of 35 to 49 (in adults, mental age from 6 to under 9 years). Likely to
result in marked developmental delays in childhood but most can learn to develop some degree
of independence in self-care and acquire adequate communication and academic skills. Adults
will need varying degrees of support to live and work in the community. Incl.: moderate
mental subnormality
F72 Severe mental retardation

Approximate IQ range of 20 to 34 (in adults, mental age from 3 to under 6 years). Likely to
result in continuous need of support. Incl.: severe mental subnormality

F73 Profound mental retardation

IQ under 20 (in adults, mental age below self-care, continence, communication and Incl.:
profound mental subnormality

Other mental retardation

Unspecified mental retardation Incl.: mental: • deficiency NOS • subnormality NOS

DEGREES OF SEVERITY OF INTELLECTUAL DISABILITY

The severity levels of intellectual disability are expressed in DSM-5 as mild, moderate, severe,
and profound.

● Mild intellectual disability represents approximately 85 percent of persons with

intellectual disability. Children with mild intellectual disability often are not identified
until the first or second grade, when academic demands increase. By late adolescence,
they often acquire academic skills at approximately a sixth-grade level. Specific causes
for intellectual disability are often unidentified in this group. Many adults with mild
intellectual disability can live independently with appropriate support and raise their own
families. IQ for this level of adaptive function may typically range from 50 to 70.
● Moderate intellectual disability represents about 10 percent of persons with intellectual
disability. Most children with moderate intellectual disability acquire language and can
communicate adequately during early childhood. They are challenged academically and
often are not able to achieve above a second to third-grade level. During adolescence,
socialization difficulties often set these persons apart, and a great deal of social and
vocational support is beneficial. As adults, individuals with moderate intellectual
disability may be able to perform semi-skilled work under appropriate supervision. IQ for
this level of adaptive function may typically range from 35 to 50.
● Severe intellectual disability represents about 4 percent of individuals with intellectual
disability. They may be able to develop communication skills in childhood and often can
learn to count as well as recognize words that are critical to functioning. In this group, the
cause for intellectual disability is more likely to be identified than in milder forms of
intellectual disability. In adulthood, persons with severe intellectual disability may adapt
 well to supervised living situations, such as group homes, and may be able to perform
work-related tasks under supervision. IQ in individuals with this level of adaptive
function may typically range from 20 to 35.
● Profound intellectual disability constitutes approximately 1 to 2 percent of individuals
with intellectual disability. Most individuals with profound intellectual disability have
identiϧable causes for their condition. Children with profound intellectual disability may
be taught some self-care skills and learn to communicate their needs given the
appropriate training. IQ in individuals with this level of adaptive function may typically
be less than 20.

EPIDEMIOLOGY

The majority of population-based prevalence estimates for intellectual disability in developing

countries range from 10 to 15 per 1,000 children. The prevalence of intellectual disability at any
one time is estimated to range from 1 to 3 percent of the population in Western societies. The
incidence of intellectual disability is difficult to accurately calculate because mild disabilities
may be unrecognized until middle childhood. In some cases, even when intellectual function is
limited, social adaptive skills may not be challenged until late childhood or early adolescence,
and the diagnosis is not made until that time. The highest incidence of intellectual disability is
reported in school-age children, with the peak at ages 10 to 14 years. Intellectual disability is
about 1.5 times more common among males than females.

Prevalence

Epidemiological surveys indicate that up to two-thirds of children and adults with intellectual
disability have comorbid psychiatric disorders, and this rate is several times higher than that in
community samples without intellectual disability. The prevalence of psychopathology appears
to be correlated with the severity of intellectual disability; the more severe the intellectual
disability, the higher the risk for coexisting psychiatric disorders.
WRITE IN REFERENCE TO THE NPC CURVE.

ETIOLOGY

Etiological factors in intellectual disability can be genetic, developmental, environmental, or a

combination.
Genetic causes include chromosomal and inherited conditions; developmental and
environmental factors include prenatal exposure to infections and toxins; and environmental or
acquired factors include prenatal trauma (e.g., prematurity) and sociocultural factors. The
severity of intellectual disability may be related to the timing and duration of a given trauma as
well as to the degree of exposure to the central nervous system (CNS). In about three fourths of
persons diagnosed with severe intellectual disability, the etiology is known, whereas the etiology
is apparent in up to half of those diagnosed with mild intellectual disability. Among
chromosomal disorders, Down syndrome and fragile X syndrome are the most common
disorders that usually produce at least moderate intellectual disability. A prototype of a metabolic
disorder associated with intellectual disability is phenylketonuria (PKU). Deprivation of
nutrition, nurturance, and social stimulation can potentially contribute to the development of at
least mild forms of intellectual disability. Current knowledge suggests that genetic,
environmental, biological, and psychosocial factors work additively in the emergence of
intellectual disability.

Acquired and Developmental Factors

Prenatal Period. Important prerequisites for the overall development of the fetus include the
mother’s physical, psychological, and nutritional health during pregnancy. Maternal chronic
illnesses and conditions affecting the normal development of the fetus’s CNS include
uncontrolled diabetes, anemia, emphysema, hypertension, and long-term use of alcohol and
narcotic substances. Maternal infections during pregnancy, especially viral infections, have been
known to cause fetal damage and intellectual disability. The extent of fetal damage depends on
such variables as the type of viral infection, the gestational age of the fetus, and the severity of
the illness.

Rubella (German measles). Rubella has replaced syphilis as the major cause of congenital
malformations and intellectual disability caused by maternal infection. The children of affected
mothers may show several abnormalities, including congenital heart disease, intellectual
disability, cataracts, deafness, microcephaly, and microphthalmia. Timing is crucial, because the
extent and frequency of the complications are inversely related to the duration of the pregnancy
at the time of maternal infection.

Cytomegalic Inclusion Disease. In many cases, cytomegalic inclusion disease remains dormant
in the mother. Some children are stillborn, and others have jaundice, microcephaly,
hepatosplenomegaly, and radiographic findings of intracerebral calcification. Children with
intellectual disability from the disease frequently have ce calcification, microcephaly, or
hydrocephalus.

Syphilis. Syphilis in pregnant women was once the main cause of various neuropathological
changes in their offspring, including intellectual disability. Today, the incidence of syphilitic
complications of pregnancy fluctuates with the incidence of syphilis in the general population.

Toxoplasmosis. Toxoplasmosis can be transmitted by the mother to the fetus. It causes mild or
severe intellectual disability and, in severe cases, hydrocephalus, seizures, microcephaly, and
chorioretinitis.

Herpes Simplex. The herpes simplex virus can be transmitted transplacentally, although the
most common mode of infection is during birth. Microcephaly, intellectual disability, intracranial
calcification, and ocular abnormalities may result.

Human Immunodeficiency Virus (HIV). Cognitive impairments are well known to be

associated with the transmission of HIV from mothers to their babies. HIV may have both direct
and indirect influences on the developing brain. A subset of infants born infected with HIV may
develop progressive encephalopathy, intellectual disabilities, and seizures within the first year of
life.

Fetal Alcohol Syndrome. Fetal alcohol syndrome (FAS) results from prenatal alcohol exposure
and can lead to a wide range of problems in the newborn. The typical phenotypic picture of a
child with FAS includes facial dysmorphism comprising hypertelorism, microcephaly, short
palpebral fissures, inner epicanthal folds, and a short, turned-up nose. Often, the affected
children have learning disorders and ADHD, and in some cases intellectual disability. Cardiac
defects are also frequent.
Prenatal Drug Exposure. Prenatal exposure to opioids, such as heroin, often results in infants
who are small for their gestational age, with a head circumference below the tenth percentile and
withdrawal symptoms that appear within the first 2 days of life. The withdrawal symptoms of
infants include irritability, hypertonia, tremor, vomiting, a high- pitched cry, and an abnormal
sleep pattern. Seizures are unusual, but the withdrawal syndrome can be life-threatening to
infants if it is untreated. Rather than a withdrawal reaction, the physiological and behavioral
abnormalities are a response to the cocaine, which may be excreted for up to a week postnatally.

Complications of Pregnancy. Toxemia of pregnancy and uncontrolled maternal diabetes present

hazards to the fetus and can potentially result in intellectual disability. Maternal malnutrition
during pregnancy often results in prematurity and other obstetrical complications. Vaginal
hemorrhage, placenta previa, premature separation of the placenta, and prolapse of the cord can
damage the fetal brain by causing anoxia.

Perinatal Period. Some evidence indicates that premature infants and infants with low birth
weight are at high risk for neurological and subtle intellectual impairments that may not be
apparent until their school years. Infants who sustain intracranial hemorrhages or show evidence
of cerebral ischemia are especially vulnerable to cognitive abnormalities. The degree of
neurodevelopmental impairment generally correlates with the severity of the intracranial
hemorrhage.

Socioeconomic deprivation can also affect the adaptive function of these vulnerable infants.
Early intervention may improve their cognitive, language, and perceptual abilities.

Acquired Childhood Disorders Infection.

Infection. The most serious infections affecting cerebral integrity are encephalitis and
meningitis. Measles encephalitis has been virtually eliminated by the universal use of measles
vaccine, and the incidence of other bacterial infections of the CNS has been markedly reduced
with antibacterial agents.

Head Trauma. The best-known causes of head injury in children that produces developmental
handicaps, including seizures, are motor vehicle accidents, but more head injuries are caused by
household accidents, such as falls from tables, open windows, and on stairways. Child
maltreatment is not infrequently implicated in head traumas or intracranial trauma such as
bleeding due to “shaken baby” syndrome.

Asphyxia. Brain damage due to asphyxia associated with near drowning is not an uncommon
cause of intellectual disability.
Long-term Exposures. Long-term exposure to lead is a well-established cause of compromised
intelligence and learning skills. Intracranial tumors of various types and origins, surgery, and
chemotherapy can also adversely affect brain function.

Environmental and Sociocultural Factors

Mild intellectual disability has been associated with significant deprivation of nutrition and
nurturance. Children who have endured these conditions are at risk for a host of psychiatric
disorders including mood disorders, posttraumatic stress disorder, and attentional and anxiety
disorders. Prenatal environment compromised by poor medical care and poor maternal nutrition
may be contributing factors in the development of mild intellectual disability. Teenage
pregnancies are at risk for mild intellectual disability in the baby due to the increased risk of
obstetrical complications, prematurity, and low birth weight.

CLINICAL FEATURES

Mild intellectual disability may not be recognized or diagnosed in a child until school challenges
the child’s social and communication skills. Cognitive deficits include poor ability to abstract
and egocentric thinking, both of which become more easily evident as a child reaches middle
childhood. Children with milder intellectual disabilities may function academically at the high
elementary level and may acquire vocational skills sufficient to support themselves in some
cases; however, social assimilation may be problematic. Communication deficits, poor
self-esteem, and dependence may further contribute to a relative lack of social spontaneity.

Moderate levels of intellectual disability are significantly more likely to be observed at a younger
age, since communication skills develop more slowly and social isolation may ensue in the
elementary school years. Academic achievement is usually limited to the middle-elementary
level. Children with moderate intellectual deficits benefit from individual attention focused on
the development of self-help skills. However, these children are aware of their deϧcits and often
feel alienated from their peers and frustrated by their limitations. They continue to require a
relatively high level of supervision but can become competent at occupational tasks in supportive
settings.

Severe intellectual disability is typically obvious in the preschool years; affected children have
minimal speech and impaired motor development. Some language development may occur in the
school-age years. By adolescence, if language has not improved significantly, poor, nonverbal
forms of communication may have evolved. Behavioral approaches are useful means to promote
some self-care, although those with severe intellectual disability generally need extensive
supervision. Children with profound intellectual disability require constant supervision and are
severely limited in both communication and motor skills. By adulthood, some speech
development may be present, and simple self-help skills may be acquired. Clinical features
frequently observed in populations with intellectual disability either in isolation or as part of a
mental disorder, include hyperactivity, low frustration tolerance, aggression, affective instability,
repetitive and stereotypic motor behaviors, and self-injurious behaviors. Self-injurious behaviors
occur more frequently and with greater intensity in more severe intellectual disability.

COURSE AND PROGNOSIS

Although the underlying intellectual impairment does not improve, in most cases of intellectual
disability, the level of adaptation increases with age and can be influenced positively by an
enriched and supportive environment. In general, persons with mild and moderate mental
intellectual disabilities have the most flexibility in adapting to various environmental conditions.
Comorbid psychiatric disorders negatively impact overall prognosis. When psychiatric disorders
are superimposed on intellectual disability, standard treatments for the comorbid mental
disorders are often beneficial; however, less robust responses and increased vulnerability to side
effects of psychopharmacologic agents are often the case.