Generalized neurodevelopmental disorder
Medical condition
Intellectual disability (ID), also known as general learning disability (in the United Kingdom), and formerly mental retardation (in the United States), is a generalized neurodevelopmental disorder characterized by significant impairment in intellectual and adaptive functioning that is first apparent during childhood. Children with intellectual disabilities typically have an intelligence quotient (IQ) below 70 and deficits in at least two adaptive behaviors that affect everyday living. According to the DSM-5, intellectual functions include reasoning, problem solving, planning, abstract thinking, judgment, academic learning, and learning from experience. Deficits in these functions must be confirmed by clinical evaluation and individualized standard IQ testing. On the other hand, adaptive behaviors include the social, developmental, and practical skills people learn to perform tasks in their everyday lives. Deficits in adaptive functioning often compromise an individual's independence and ability to meet their social responsibility.
Intellectual disability is subdivided into syndromic intellectual disability, in which intellectual deficits associated with other medical and behavioral signs and symptoms are present, and non-syndromic intellectual disability, in which intellectual deficits appear without other abnormalities. Down syndrome and fragile X syndrome are examples of syndromic intellectual disabilities.
Intellectual disability affects about 2–3% of the general population. Seventy-five to ninety percent of the affected people have mild intellectual disability. Non-syndromic, or idiopathic cases account for 30–50% of these cases. About a quarter of cases are caused by a genetic disorder, and about 5% of cases are inherited. Cases of unknown cause affect about 95 million people as of 2013[ [update]].
Signs and symptoms.
Intellectual disability (ID) becomes apparent during childhood, and involves deficits in mental abilities, social skills, and core activities of daily living (ADLs) when compared to peers of the same age. There are often no physical signs of mild forms of ID, although there may be characteristic physical traits when it is associated with a genetic disorder (e.g. Down syndrome).
The level of impairment ranges in severity for each person. Some of the early signs can include:
In early childhood, mild ID (IQ 50–69) may not be obvious or identified until children begin school. Even when poor academic performance is recognized, it may take expert assessment to distinguish mild intellectual disability from specific learning disability or emotional/behavioral disorders. People with mild ID are capable of learning reading and mathematics skills to approximately the level of a typical child aged nine to twelve. They can learn self-care and practical skills, such as cooking or using the local transit system. As individuals with intellectual disabilities reach adulthood, many learn to live independently and maintain gainful employment. About 85% of people with ID are likely to have mild ID.
Moderate ID (IQ 35–49) is almost always apparent within the first years of life. Speech delays are particularly common signs of moderate ID. People with moderate intellectual disabilities need considerable support in school, at home, and in the community in order to fully participate. While their academic potential is limited, they can learn simple health and safety skills and to participate in simple activities. As adults, they may live with their parents, in a supportive group home, or even semi-independently with significant supportive services to help them, for example, to manage their finances. As adults, they may work in a sheltered workshop. About 10% of people with ID are likely to have moderate ID.
People with Severe ID (IQ 20–34), accounting for 3.5% of persons with ID, or Profound ID (IQ 19 or below), accounting for 1.5% of people with ID, need more intensive support and supervision for their entire lives. They may learn some ADLs, but an intellectual disability is considered severe or profound when individuals are unable to independently care for themselves without ongoing significant assistance from a caregiver throughout adulthood. Individuals with profound ID are completely dependent on others for all ADLs and to maintain their physical health and safety. They may be able to learn to participate in some of these activities to a limited degree.
Co-morbidity.
Autism and intellectual disability.
Intellectual disability and autism spectrum disorder (ASD) share clinical characteristics which can result in confusion while diagnosing. Overlapping these two disorders, while common, can be detrimental to a person's well-being. Those with ASD that hold symptoms of ID may be grouped into a co-diagnosis in which they are receiving treatment for a disorder they do not have. Likewise, those with ID that are mistaken to have ASD may be treated for symptoms of a disorder they do not have. Differentiating between these two disorders will allow clinicians to deliver or prescribe the appropriate treatments. Comorbidity between ID and ASD is very common; roughly 30% of those with ASD also have ID. Both ASD and ID require shortfalls in communication and social awareness as defining criteria.
In a study conducted in 2016 surveying 2816 cases, it was found that the top subsets that help differentiate between those with ID and ASD are, "impaired non-verbal social behavior and lack of social reciprocity, [...] restricted interests, strict adherence to routines, stereotyped and repetitive motor mannerisms, and preoccupation with parts of objects". Those with ASD tend to show more deficits in non-verbal social behavior such as body language and understanding social cues. In a study done in 2008 of 336 individuals with varying levels of ID, it was found that those with ID display fewer instances of repetitive or ritualistic behaviors. It also recognized that those with ASD, when compared to those with ID, were more likely to isolate themselves and make less eye contact. When it comes to classification ID and ASD have very different guidelines. ID has a standardized assessment called the Supports Intensity Scale (SIS); this measures severity on a system built around how much support an individual will need. While ASD also classifies severity by support needed, there is no standard assessment; clinicians are free to diagnose severity at their own judgment.
Epilepsy and intellectual disability.
Around 22% of individuals with ID suffer from epilepsy. The incidence of epilepsy is associated with level of ID; epilepsy affects around half of individuals with profound ID. Proper epilepsy management is particularly crucial in this population, as individuals are at increased risk of sudden unexpected death in epilepsy. Nonetheless, epilepsy management in the ID population can be challenging due to high levels of polypharmacy prescribing, drug interactions, and increased vulnerability to adverse effects. It is thought that 70% of individuals with ID are pharmaco-resistant, however only around 10% of individuals are prescribed Anti-Seizure Medications (ASMs) licensed for pharmaco-resistant epilepsy. Research shows that certain ASMs, including Levetiracetam and Brivaracetam, show similar efficacy and tolerability in individuals with ID as compared to those without. There is much ongoing research into epilepsy management in the ID population.
Causes.
Among children, the cause of intellectual disability is unknown for one-third to one-half of cases. About 5% of cases are inherited. Genetic defects that cause intellectual disability, but are not inherited, can be caused by accidents or mutations in genetic development. Examples of such accidents are development of an extra chromosome 18 (trisomy 18) and Down syndrome, which is the most common genetic cause. DiGeorge syndrome and fetal alcohol spectrum disorders are the next most common causes. Some other frequently observed causes include:
Diagnosis.
According to both the American Association on Intellectual and Developmental Disabilities and the American Psychiatric Association's "Diagnostic and Statistical Manual of Mental Disorders" (DSM-IV), three criteria must be met for a diagnosis of intellectual disability: significant limitation in general mental abilities (intellectual functioning), significant limitations in one or more areas of adaptive behavior across multiple environments (as measured by an adaptive behavior rating scale, i.e. communication, self-help skills, interpersonal skills, and more), and evidence that the limitations became apparent in childhood or adolescence (onset during developmental phase).
In general, people with intellectual disabilities have an IQ below 70, but clinical discretion may be necessary for individuals who have a somewhat higher IQ but severe impairment in adaptive functioning.
It is formally diagnosed by an assessment of IQ and adaptive behavior. A third condition requiring onset during the developmental period is used to distinguish intellectual disability from other conditions, such as traumatic brain injuries and dementias (including Alzheimer's disease).
Intelligence quotient.
The first English-language IQ test, the Stanford–Binet Intelligence Scales, was adapted from a test battery designed for school placement by Alfred Binet in France. Lewis Terman adapted Binet's test and promoted it as a test measuring "general intelligence". Terman's test was the first widely used mental test to report scores in "intelligence quotient" form ("mental age" divided by chronological age, multiplied by 100). Current tests are scored in "deviation IQ" form, with a performance level by a test-taker two standard deviations below the median score for the test-takers age group defined as IQ 70. Until the most recent revision of diagnostic standards, an IQ of 70 or below was a primary factor for intellectual disability diagnosis, and IQ scores were used to categorize degrees of intellectual disability.
Since the current diagnosis of intellectual disability is not based on IQ scores alone, but must also take into consideration a person's adaptive functioning, the diagnosis is not made rigidly. It encompasses intellectual scores, adaptive functioning scores from an adaptive behavior rating scale based on descriptions of known abilities provided by someone familiar with the person, and also the observations of the assessment examiner, who is able to find out directly from the person what they can understand, communicate, and such like. IQ assessment must be based on a current test. This enables a diagnosis to avoid the pitfall of the Flynn effect, which is a consequence of changes in population IQ test performance changing IQ test norms over time.
Distinction from other disabilities.
Clinically, intellectual disability is a subtype of cognitive deficit or disabilities affecting intellectual abilities, which is a broader concept and includes intellectual deficits that are too mild to properly qualify as intellectual disability, or too specific (as in specific learning disability), or acquired later in life through acquired brain injuries or neurodegenerative diseases like dementia. Cognitive deficits may appear at any age. Developmental disability is any disability that is due to problems with growth and development. This term encompasses many congenital medical conditions that have no mental or intellectual components, although it, too, is sometimes used as a euphemism for intellectual disability.
Limitations in more than one area.
Adaptive behavior, or adaptive functioning, refers to the skills needed to live independently (or at the minimally acceptable level for age). To assess adaptive behavior, professionals compare the functional abilities of a child to those of other children of similar age. To measure adaptive behavior, professionals use structured interviews, with which they systematically elicit information about persons' functioning in the community from people who know them well. There are many adaptive behavior scales, and accurate assessment of the quality of someone's adaptive behavior requires clinical judgment as well. Certain skills are important to adaptive behavior, such as:
Other specific skills can be critical to an individual's inclusion in the community and to develop appropriate social behaviors, as for example being aware of the different social expectations linked to the principal lifespan stages (i.e., childhood, adulthood, old age). The results of a Swiss study suggest that the performance of adults with ID in recognizing different lifespan stages is related to specific cognitive abilities and to the type of material used to test this performance.
Management.
By most definitions, intellectual disability is more accurately considered a "disability" rather than a "disease". Intellectual disability can be distinguished in many ways from mental illness, such as schizophrenia or depression. Currently, there is no "cure" for an established disability, though with appropriate support and teaching, most individuals can learn to do many things. Causes, such as congenital hypothyroidism, if detected early may be treated to prevent the development of an intellectual disability.
There are thousands of agencies around the world that provide assistance for people with developmental disabilities. They include state-run, for-profit, and non-profit, privately run agencies. Within one agency there could be departments that include fully staffed residential homes, day rehabilitation programs that approximate schools, workshops wherein people with disabilities can obtain jobs, programs that assist people with developmental disabilities in obtaining jobs in the community, programs that provide support for people with developmental disabilities who have their own apartments, programs that assist them with raising their children, and many more. There are also many agencies and programs for parents of children with developmental disabilities.
Beyond that, there are specific programs that people with developmental disabilities