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Intellectual Disability (Intellectual Developmental Disorder)

Intellectual Disability (Intellectual Developmental Disorder) is a topic covered in the 5-Minute Clinical Consult.

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Basics

  • A global deficit in cognitive functioning evidenced by a significant difference in mental and chronologic ages (intelligence quotient [IQ]) and significantly impaired adaptive functioning (1)
  • Although cognitive issues typically have a pervasive impact, patients with ID display highly variable levels of functioning and subsequent service needs.
  • Evaluation and treatment plans must be tailored to individual needs.
  • The term mental retardation was deleted from the DSM-5 and replaced with “intellectual disability” (ID) or “intellectual developmental disorder.” The term “mental retardation” is pejorative and insensitive.

Description

  • ID is defined as an IQ ≤70 + 5 and a significant impairment in intellectual functioning, including verbal and nonverbal reasoning, planning, academic learning, problem solving, and experiential learning. Intellectual function is confirmed by IQ testing and clinical assessment (1).
  • A diagnosis of ID also requires deficits in adaptive functioning, such as communication, socialization, and independent living (1).
  • By definition, ID is a neurodevelopmental disorder that is typically present from birth or recognized during early childhood (1).
  • ID is currently subgrouped according to adaptive function and level of needed support: mild (typical development in some domains, mild impairment in others), moderate (skills are markedly behind same-age peers), severe (skills quite limited compared to peers), profound (limited awareness of concepts, language; dependent on others for adaptive functioning) (1).
  • The three most common causes of ID are Down syndrome, fragile X syndrome, and fetal alcohol syndrome (FAS).
  • If ID reflects a loss of previously acquired intellectual skills, consider a comorbid neurocognitive disorder.
  • Previous stereotypes of people with ID (e.g., always happy, poor prognosis, unable to function independently) have been refuted. People with ID are showing a level of functioning variability that parallels what is found in the non-ID population.
  • Under the Individuals with Disabilities Education Act (IDEA), caregivers should be encouraged to familiarize themselves with their child’s legal rights to facilitate coordination of services with the school district.
  • Under the Americans with Disabilities Act (1990), people with ID cannot be discriminated against based on their disability, and employers are legally required to ensure that appropriate accommodations are in place.

ALERT
For some causes of ID, prenatal testing is available.

Epidemiology

Incidence
  • 1 of 6 children (2)
  • Predominant sex: male > female: 1.6:1 for mild ID, 1.2:1 for severe ID (1)

Prevalence
In the United States, 1% of the general population. The prevalence of severe ID is 6/1,000 (1).

Etiology and Pathophysiology

  • Causes:
    • Maternal substance abuse (e.g., alcohol); FAS is a leading environmental cause of ID.
    • Maternal infections: TORCH viruses (toxoplasma, other infections, rubella, cytomegalovirus, and herpes simplex virus)
    • Down syndrome
    • Sex chromosome abnormalities: fragile X, Turner syndrome, Klinefelter syndrome
    • Autosomal dominant conditions: neurocutaneous syndromes (e.g., neurofibromatosis, tuberous sclerosis)
    • Autosomal recessive conditions:
      • Amino acid metabolism (e.g., phenylketonuria, maple syrup urine disease)
      • Carbohydrate metabolism (e.g., galactosemia, fructosuria)
      • Lipid metabolism
      • Tay-Sachs disease
      • Gaucher disease
      • Niemann-Pick disease; mucopolysaccharidosis
      • Purine metabolism (e.g., Lesch-Nyhan disease)
      • Other (e.g., Wilson disease)
  • Maternal use of prescription medications (e.g., Accutane, Dilantin)
  • Perinatal factors:
    • Prematurity
    • Birth injuries
    • Perinatal anoxia
  • Postnatal factors:
    • Childhood diseases (e.g., meningitis, encephalitis, hypothyroidism, seizure disorders)
    • Trauma (e.g., accidents, physical abuse, hypoxia)
    • Severe deprivation
    • Poisoning (e.g., lead, carbon monoxide, household products)

Genetics
A number of genetic and epigenetic causes are known, and more are under investigation (3).

Risk Factors

  • Maternal substance abuse during pregnancy
  • Maternal infection during pregnancy
  • For some causes, family history

General Prevention

  • Public health efforts to reduce alcohol and drug use by pregnant women
  • Prenatal folic acid supplementation

Commonly Associated Conditions

  • Seizures
  • Mood disorders
  • Behavioral disorders
  • Constipation

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Citation

Stephens, Mark B., et al., editors. "Intellectual Disability (Intellectual Developmental Disorder)." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816254/all/Intellectual_Disability__Intellectual_Developmental_Disorder_.
Intellectual Disability (Intellectual Developmental Disorder). In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816254/all/Intellectual_Disability__Intellectual_Developmental_Disorder_. Accessed March 26, 2019.
Intellectual Disability (Intellectual Developmental Disorder). (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816254/all/Intellectual_Disability__Intellectual_Developmental_Disorder_
Intellectual Disability (Intellectual Developmental Disorder) [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 March 26]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816254/all/Intellectual_Disability__Intellectual_Developmental_Disorder_.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Intellectual Disability (Intellectual Developmental Disorder) ID - 816254 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816254/all/Intellectual_Disability__Intellectual_Developmental_Disorder_ PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -