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Mental Retardation

MedGen UID:: 7544
•Concept ID:: C0025362
•: Mental or Behavioral Dysfunction

Synonym:

Mental retardation

Definition

A developmental disorder characterized by less than average intelligence and significant limitations in adaptive behavior with onset before the age of 18. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVMental Retardation

Neurodevelopmental disorder
- Developmental disorder
  - Neurodevelopmental disorder
    - Developmental disorder
      - Neurodevelopmental disorder
        Developmental disorder
        Mental Retardation
        ADNP-related multiple congenital anomalies - intellectual disability - autism spectrum disorder
        Alpha thalassemia-X-linked intellectual disability syndrome
        Borjeson-Forssman-Lehmann syndrome
        Christianson syndrome
        Intellectual disability, autosomal dominant 1
        Intellectual disability, autosomal dominant 5
        Intellectual disability, autosomal dominant 39
        Intellectual disability, autosomal recessive 34
        Intellectual disability, X-linked 1
        Mental Retardation, Autosomal Dominant 40
        Mental Retardation, X-linked 102
        Mental Retardation, X-linked 21/34
        Renpenning syndrome
        Hamel cerebro-palato-cardiac syndrome
        X-linked intellectual disability, Golabi-Ito-hall type
        X-linked intellectual disability, Porteous type
        X-linked intellectual disability, Sutherland-Haan type
        Severe intellectual disability-progressive spastic diplegia syndrome
        Syndromic X-linked intellectual disability Lubs type
        X-linked intellectual disability Cabezas type

Professional guidelines

PubMed

Joubert syndrome: Molecular basis and treatment.

Spahiu L, Behluli E, Grajçevci-Uka V, Liehr T, Temaj G
J Mother Child 2022 Mar 1;26(1):118-123. Epub 2023 Feb 22 doi: 10.34763/jmotherandchild.20222601.d-22-00034. PMID: 36803942 Free PMC Article

Mental retardation: management and prognosis.

Johnson CP, Walker WO Jr
Pediatr Rev 2006 Jul;27(7):249-55; quiz 256. doi: 10.1542/pir.27-7-249. PMID: 16815993

Sialorrhea: a management challenge.

Hockstein NG, Samadi DS, Gendron K, Handler SD
Am Fam Physician 2004 Jun 1;69(11):2628-34. PMID: 15202698

See all (703)

Recent clinical studies

Etiology

Do comorbidities among patients with mental retardation differ across various age groups?

Singh S, Singh LK, Sahu M, Tikka SK
Asian J Psychiatr 2019 Jan;39:12-14. Epub 2018 Nov 13 doi: 10.1016/j.ajp.2018.11.001. PMID: 30466056

Neurobehavioural evidence for the involvement of the FMR1 gene in female carriers of fragile X syndrome.

Kraan CM, Hocking DR, Bradshaw JL, Fielding J, Cohen J, Georgiou-Karistianis N, Cornish KM
Neurosci Biobehav Rev 2013 Mar;37(3):522-47. Epub 2013 Jan 23 doi: 10.1016/j.neubiorev.2013.01.010. PMID: 23352653

X-linked mental retardation.

Lisik MZ, Sieron AL
Med Sci Monit 2008 Nov;14(11):RA221-9. PMID: 18971887

Outpatient psychotherapy for adults with mental retardation and concomitant psychopathology: research and clinical imperatives.

Nezu CM, Nezu AM
J Consult Clin Psychol 1994 Feb;62(1):34-42. doi: 10.1037//0022-006x.62.1.34. PMID: 8034826

Folie à deux and mental retardation: review and case report.

Ghaziuddin M
Can J Psychiatry 1991 Feb;36(1):48-9. doi: 10.1177/070674379103600110. PMID: 2029684

See all (7010)

Diagnosis

Advances in understanding fragile X syndrome and related disorders.

Rooms L, Kooy RF
Curr Opin Pediatr 2011 Dec;23(6):601-6. doi: 10.1097/MOP.0b013e32834c7f1a. PMID: 22001764

Mental retardation.

Crausman B
Psychosomatics 1966 Jan-Feb;7(1):19-20. doi: 10.1016/S0033-3182(66)72196-3. PMID: 5905730

GENETIC COUNSELING.

PRIEST JH
J Am Med Womens Assoc 1964 Nov;19:961-3. PMID: 14216379

BRACHMANN/DE LANGE SYNDROME.

OPITZ JM, SEGAL AT, LEHRKE R, NADLER H
Lancet 1964 Nov 7;2(7367):1019. doi: 10.1016/s0140-6736(64)90980-8. PMID: 14201663

THE AUTISTIC CHILD.

SODDY K
Practitioner 1964 Apr;192:525-33. PMID: 14143327

See all (8683)

Therapy

Efficacy and Safety of Fenfluramine for the Treatment of Seizures Associated With Lennox-Gastaut Syndrome: A Randomized Clinical Trial.

Knupp KG, Scheffer IE, Ceulemans B, Sullivan JE, Nickels KC, Lagae L, Guerrini R, Zuberi SM, Nabbout R, Riney K, Shore S, Agarwal A, Lock M, Farfel GM, Galer BS, Gammaitoni AR, Davis R, Gil-Nagel A
JAMA Neurol 2022 Jun 1;79(6):554-564. doi: 10.1001/jamaneurol.2022.0829. PMID: 35499850 Free PMC Article

Congenital Hypothyroidism.

Al-Qahtani M
J Matern Fetal Neonatal Med 2022 Oct;35(19):3761-3769. Epub 2020 Oct 28 doi: 10.1080/14767058.2020.1838480. PMID: 33115295

Wilson disease.

Aggarwal A, Bhatt M
Curr Opin Neurol 2020 Aug;33(4):534-542. doi: 10.1097/WCO.0000000000000837. PMID: 32657896

Pharmacotherapy for Fragile X Syndrome: Progress to Date.

Davenport MH, Schaefer TL, Friedmann KJ, Fitzpatrick SE, Erickson CA
Drugs 2016 Mar;76(4):431-45. doi: 10.1007/s40265-016-0542-y. PMID: 26858239

Fragile X syndrome: from gene discovery to therapy.

Heulens I, Kooy F
Front Biosci (Landmark Ed) 2011 Jan 1;16(4):1211-32. doi: 10.2741/3785. PMID: 21196228

See all (2497)

Prognosis

The epidemiology of drug-resistant epilepsy: A systematic review and meta-analysis.

Kalilani L, Sun X, Pelgrims B, Noack-Rink M, Villanueva V
Epilepsia 2018 Dec;59(12):2179-2193. Epub 2018 Nov 13 doi: 10.1111/epi.14596. PMID: 30426482

Congenital hypothyroidism.

Jain V, Agarwal R, Deorari AK, Paul VK
Indian J Pediatr 2008 Apr;75(4):363-7. Epub 2008 May 18 doi: 10.1007/s12098-008-0040-7. PMID: 18536892

Non-typhoid Salmonella meningitis.

Kavaliotis J, Tsiaousi A, Papavasiliou D, Kansouzidou A
Scand J Infect Dis 1994;26(4):403-5. doi: 10.3109/00365549409008612. PMID: 7984971

The Cohen syndrome.

Fryns JP, Van den Berghe H
J Genet Hum 1981 Dec;29(4):449-53. PMID: 7328419

SIGMOID VOLVULUS.

ABRAMS BL
Arch Surg 1963 Dec;87:1033-9. doi: 10.1001/archsurg.1963.01310180149025. PMID: 14063812

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Clinical prediction guides

Stratification of the risk of ovarian dysfunction by studying the complexity of intermediate and premutation alleles of the FMR1 gene.

Quilichini J, Perol S, Cuisset L, Grotto S, Fouveaut C, Barbot JC, Verebi C, Jordan P, Héron D, Molina-Gomes D, Pipiras E, Grynberg M, Catteau-Jonard S, Touraine P, Christin-Maître S, Plu-Bureau G, El Khattabi L, Bienvenu T
Am J Med Genet A 2024 Apr;194(4):e63479. Epub 2023 Nov 21 doi: 10.1002/ajmg.a.63479. PMID: 37987117

Change in Maladaptive Behavior Affects Intergenerational Relationships in Fragile X Syndrome.

Lorang E, Hong J, DaWalt LS, Mailick M
Am J Intellect Dev Disabil 2022 May 1;127(3):231-248. doi: 10.1352/1944-7558-127.3.231. PMID: 35443052 Free PMC Article

Current status of newborn screening: decision-making about the conditions to include in screening programs.

Watson MS
Ment Retard Dev Disabil Res Rev 2006;12(4):230-5. doi: 10.1002/mrdd.20127. PMID: 17183572

Developmental phonological disorders. I: A clinical profile.

Shriberg LD, Kwiatkowski J
J Speech Hear Res 1994 Oct;37(5):1100-26. doi: 10.1044/jshr.3705.1100. PMID: 7823556

Risk factors for developmental delay among infants and toddlers.

King EH, Logsdon DA, Schroeder SR
Child Health Care 1992 Winter;21(1):39-52. doi: 10.1207/s15326888chc2101_6. PMID: 10145594

See all (5162)

Recent systematic reviews

Calcium channelopathies and intellectual disability: a systematic review.

Kessi M, Chen B, Peng J, Yan F, Yang L, Yin F
Orphanet J Rare Dis 2021 May 13;16(1):219. doi: 10.1186/s13023-021-01850-0. PMID: 33985586 Free PMC Article

Raggi A, Plazzi G, Ferri R
J Nerv Ment Dis 2019 Feb;207(2):84-99. doi: 10.1097/NMD.0000000000000918. PMID: 30672873

The epidemiology of drug-resistant epilepsy: A systematic review and meta-analysis.

Kalilani L, Sun X, Pelgrims B, Noack-Rink M, Villanueva V
Epilepsia 2018 Dec;59(12):2179-2193. Epub 2018 Nov 13 doi: 10.1111/epi.14596. PMID: 30426482

Epidemiology of fragile X syndrome: a systematic review and meta-analysis.

Hunter J, Rivero-Arias O, Angelov A, Kim E, Fotheringham I, Leal J
Am J Med Genet A 2014 Jul;164A(7):1648-58. Epub 2014 Apr 3 doi: 10.1002/ajmg.a.36511. PMID: 24700618

Systematic review of central nervous system anomalies in incontinentia pigmenti.

Minić S, Trpinac D, Obradović M
Orphanet J Rare Dis 2013 Feb 13;8:25. doi: 10.1186/1750-1172-8-25. PMID: 23406512 Free PMC Article

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