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Profound intellectual disability

MedGen UID:
43816
Concept ID:
C0020796
Finding; Mental or Behavioral Dysfunction
Synonym: Idiocy
SNOMED CT: Profound mental retardation (Intelligence Quotient below 20) (31216003); Profound intellectual disability (31216003); Profound intellectual development disorder (31216003); Profound mental retardation (I.Q. below 20) (31216003)

Definition

IQ below 20. [from PSY]

Professional guidelines

PubMed

Paulet A, Bennett-Ness C, Ageorges F, Trost D, Green A, Goudie D, Jewell R, Kraatari-Tiri M, Piard J, Coubes C, Lam W, Lynch SA, Groeschel S, Ramond F, Fluss J, Fagerberg C, Brasch Andersen C, Varvagiannis K, Kleefstra T, Gérard B, Fradin M, Vitobello A, Tenconi R, Denommé-Pichon AS, Vincent-Devulder A, Haack T, Marsh JA, Laulund LW, Grimmel M, Riess A, de Boer E, Padilla-Lopez S, Bakhtiari S, Ostendorf A, Zweier C, Smol T, Willems M, Faivre L, Scala M, Striano P, Bagnasco I, Koboldt D, Iascone M, Suerink M, Kruer MC, Levy J, Verloes A, Abbott CM, Ruaud L
Eur J Hum Genet 2024 Sep;32(9):1144-1149. Epub 2024 Feb 15 doi: 10.1038/s41431-024-01560-8. PMID: 38355961Free PMC Article
D'Onofrio G, Accogli A, Severino M, Caliskan H, Kokotović T, Blazekovic A, Jercic KG, Markovic S, Zigman T, Goran K, Barišić N, Duranovic V, Ban A, Borovecki F, Ramadža DP, Barić I, Fazeli W, Herkenrath P, Marini C, Vittorini R, Gowda V, Bouman A, Rocca C, Alkhawaja IA, Murtaza BN, Rehman MMU, Al Alam C, Nader G, Mancardi MM, Giacomini T, Srivastava S, Alvi JR, Tomoum H, Matricardi S, Iacomino M, Riva A, Scala M, Madia F, Pistorio A, Salpietro V, Minetti C, Rivière JB, Srour M, Efthymiou S, Maroofian R, Houlden H, Vernes SC, Zara F, Striano P, Nagy V
Hum Genet 2023 Jul;142(7):909-925. Epub 2023 May 14 doi: 10.1007/s00439-023-02552-2. PMID: 37183190Free PMC Article
Borlot F, de Almeida BI, Combe SL, Andrade DM, Filloux FM, Myers KA
Epilepsia 2019 Aug;60(8):1661-1669. Epub 2019 Jul 5 doi: 10.1111/epi.16273. PMID: 31273778

Recent clinical studies

Etiology

Schmetz A, Lüdecke HJ, Surowy H, Sivalingam S, Bruel AL, Caumes R, Charles P, Chatron N, Chrzanowska K, Codina-Solà M, Colson C, Cuscó I, Denommé-Pichon AS, Edery P, Faivre L, Green A, Heide S, Hsieh TC, Hustinx A, Kleinendorst L, Knopp C, Kraft F, Krawitz PM, Lasa-Aranzasti A, Lesca G, López-González V, Maraval J, Mignot C, Neuhann T, Netzer C, Oehl-Jaschkowitz B, Petit F, Philippe C, Posmyk R, Putoux A, Reis A, Sánchez-Soler MJ, Suh J, Tkemaladze T, Tran Mau Them F, Travessa A, Trujillano L, Valenzuela I, van Haelst MM, Vasileiou G, Vincent-Delorme C, Walther M, Verde P, Bramswig NC, Wieczorek D
Hum Genet 2024 Jan;143(1):71-84. Epub 2023 Dec 20 doi: 10.1007/s00439-023-02622-5. PMID: 38117302
Hamouda I, Rousseau MC, Beltran Anzola A, Aim MA, de Villemeur TB, Auquier P, Baumstarck K; EVAL-PLH group
Sci Rep 2022 Jul 22;12(1):12512. doi: 10.1038/s41598-022-16596-3. PMID: 35869128Free PMC Article
Eaton C, Tarver J, Shirazi A, Pearson E, Walker L, Bird M, Oliver C, Waite J
J Intellect Disabil Res 2021 Mar;65(3):211-229. Epub 2021 Jan 11 doi: 10.1111/jir.12807. PMID: 33426741
Bar C, Kuchenbuch M, Barcia G, Schneider A, Jennesson M, Le Guyader G, Lesca G, Mignot C, Montomoli M, Parrini E, Isnard H, Rolland A, Keren B, Afenjar A, Dorison N, Sadleir LG, Breuillard D, Levy R, Rio M, Dupont S, Negrin S, Danieli A, Scalais E, De Saint Martin A, El Chehadeh S, Chelly J, Poisson A, Lebre AS, Nica A, Odent S, Sekhara T, Brankovic V, Goldenberg A, Vrielynck P, Lederer D, Maurey H, Terrone G, Besmond C, Hubert L, Berquin P, Billette de Villemeur T, Isidor B, Freeman JL, Mefford HC, Myers CT, Howell KB, Rodríguez-Sacristán Cascajo A, Meyer P, Genevieve D, Guët A, Doummar D, Durigneux J, van Dooren MF, de Wit MCY, Gerard M, Marey I, Munnich A, Guerrini R, Scheffer IE, Kabashi E, Nabbout R
Epilepsia 2020 Nov;61(11):2461-2473. Epub 2020 Sep 21 doi: 10.1111/epi.16679. PMID: 32954514
Vehmas S, Curtis B
Camb Q Healthc Ethics 2017 Jul;26(3):505-516. doi: 10.1017/S0963180116001183. PMID: 28541179

Diagnosis

Schmetz A, Lüdecke HJ, Surowy H, Sivalingam S, Bruel AL, Caumes R, Charles P, Chatron N, Chrzanowska K, Codina-Solà M, Colson C, Cuscó I, Denommé-Pichon AS, Edery P, Faivre L, Green A, Heide S, Hsieh TC, Hustinx A, Kleinendorst L, Knopp C, Kraft F, Krawitz PM, Lasa-Aranzasti A, Lesca G, López-González V, Maraval J, Mignot C, Neuhann T, Netzer C, Oehl-Jaschkowitz B, Petit F, Philippe C, Posmyk R, Putoux A, Reis A, Sánchez-Soler MJ, Suh J, Tkemaladze T, Tran Mau Them F, Travessa A, Trujillano L, Valenzuela I, van Haelst MM, Vasileiou G, Vincent-Delorme C, Walther M, Verde P, Bramswig NC, Wieczorek D
Hum Genet 2024 Jan;143(1):71-84. Epub 2023 Dec 20 doi: 10.1007/s00439-023-02622-5. PMID: 38117302
Wirth T, Garone G, Kurian MA, Piton A, Millan F, Telegrafi A, Drouot N, Rudolf G, Chelly J, Marks W, Burglen L, Demailly D, Coubes P, Castro-Jimenez M, Joriot S, Ghoumid J, Belin J, Faucheux JM, Blumkin L, Hull M, Parnes M, Ravelli C, Poulen G, Calmels N, Nemeth AH, Smith M, Barnicoat A, Ewenczyk C, Méneret A, Roze E, Keren B, Mignot C, Beroud C, Acosta F Jr, Nowak C, Wilson WG, Steel D, Capuano A, Vidailhet M, Lin JP, Tranchant C, Cif L, Doummar D, Anheim M
Mov Disord 2022 Jul;37(7):1547-1554. Epub 2022 Jun 20 doi: 10.1002/mds.29074. PMID: 35722775Free PMC Article
Brunklaus A, Brünger T, Feng T, Fons C, Lehikoinen A, Panagiotakaki E, Vintan MA, Symonds J, Andrew J, Arzimanoglou A, Delima S, Gallois J, Hanrahan D, Lesca G, MacLeod S, Marjanovic D, McTague A, Nuñez-Enamorado N, Perez-Palma E, Scott Perry M, Pysden K, Russ-Hall SJ, Scheffer IE, Sully K, Syrbe S, Vaher U, Velayutham M, Vogt J, Weiss S, Wirrell E, Zuberi SM, Lal D, Møller RS, Mantegazza M, Cestèle S
Brain 2022 Nov 21;145(11):3816-3831. doi: 10.1093/brain/awac210. PMID: 35696452Free PMC Article
Ernst ME, Baugh EH, Thomas A, Bier L, Lippa N, Stong N, Mulhern MS, Kushary S, Akman CI, Heinzen EL, Yeh R, Bi W, Hanchard NA, Burrage LC, Leduc MS, Chong JSC, Bend R, Lyons MJ, Lee JA, Suwannarat P, Brilstra E, Simon M, Koopmans M, van Binsbergen E, Groepper D, Fleischer J, Nava C, Keren B, Mignot C, Mathieu S, Mancini GMS, Madan-Khetarpal S, Infante EM, Bluvstein J, Seeley A, Bachman K, Klee EW, Schultz-Rogers LE, Hasadsri L, Barnett S, Ellingson MS, Ferber MJ, Narayanan V, Ramsey K, Rauch A, Joset P, Steindl K, Sheehan T, Poduri A, Vasquez A, Ruivenkamp C, White SM, Pais L, Monaghan KG, Goldstein DB, Sands TT, Aggarwal V
Epilepsia 2021 Jul;62(7):e103-e109. Epub 2021 May 26 doi: 10.1111/epi.16931. PMID: 34041744Free PMC Article
Walton C, Kerr M
J Appl Res Intellect Disabil 2016 Sep;29(5):395-408. Epub 2015 Jun 22 doi: 10.1111/jar.12203. PMID: 26101049

Therapy

Lo Buono V, Torrisi M, Leonardi S, Pidalà A, Corallo F
Medicine (Baltimore) 2022 Nov 18;101(46):e31404. doi: 10.1097/MD.0000000000031404. PMID: 36401482Free PMC Article
Monaghan R, O'Dwyer M, Luus R, Mulryan N, McCallion P, McCarron M, Henman MC
Epilepsy Behav 2021 Sep;122:108191. Epub 2021 Jul 12 doi: 10.1016/j.yebeh.2021.108191. PMID: 34265622
Mazza MG, Rossetti A, Crespi G, Clerici M
J Appl Res Intellect Disabil 2020 Mar;33(2):126-138. Epub 2019 Aug 20 doi: 10.1111/jar.12654. PMID: 31430018
D'Alessandro P, Giuglietti M, Baglioni A, Verdolini N, Murgia N, Piccirilli M, Elisei S
Psychiatr Danub 2017 Sep;29(Suppl 3):399-404. PMID: 28953798
Chan JSL, Chien WT
J Intellect Disabil Res 2017 Jun;61(6):532-548. Epub 2017 Apr 7 doi: 10.1111/jir.12377. PMID: 28387017

Prognosis

Thomas Q, Motta M, Gautier T, Zaki MS, Ciolfi A, Paccaud J, Girodon F, Boespflug-Tanguy O, Besnard T, Kerkhof J, McConkey H, Masson A, Denommé-Pichon AS, Cogné B, Trochu E, Vignard V, El It F, Rodan LH, Alkhateeb MA, Jamra RA, Duplomb L, Tisserant E, Duffourd Y, Bruel AL, Jackson A, Banka S, McEntagart M, Saggar A, Gleeson JG, Sievert D, Bae H, Lee BH, Kwon K, Seo GH, Lee H, Saeed A, Anjum N, Cheema H, Alawbathani S, Khan I, Pinto-Basto J, Teoh J, Wong J, Sahari UBM, Houlden H, Zhelcheska K, Pannetier M, Awad MA, Lesieur-Sebellin M, Barcia G, Amiel J, Delanne J, Philippe C, Faivre L, Odent S, Bertoli-Avella A, Thauvin C, Sadikovic B, Reversade B, Maroofian R, Govin J, Tartaglia M, Vitobello A
Am J Hum Genet 2022 Oct 6;109(10):1909-1922. Epub 2022 Aug 30 doi: 10.1016/j.ajhg.2022.08.008. PMID: 36044892Free PMC Article
Stamberger H, Crosiers D, Balagura G, Bonardi CM, Basu A, Cantalupo G, Chiesa V, Christensen J, Dalla Bernardina B, Ellis CA, Furia F, Gardiner F, Giron C, Guerrini R, Klein KM, Korff C, Krijtova H, Leffler M, Lerche H, Lesca G, Lewis-Smith D, Marini C, Marjanovic D, Mazzola L, McKeown Ruggiero S, Mochel F, Ramond F, Reif PS, Richard-Mornas A, Rosenow F, Schropp C, Thomas RH, Vignoli A, Weber Y, Palmer E, Helbig I, Scheffer IE, Striano P, Møller RS, Gardella E, Weckhuysen S
Neurology 2022 Jul 19;99(3):e221-e233. Epub 2022 Jun 3 doi: 10.1212/WNL.0000000000200715. PMID: 35851549Free PMC Article
Bar C, Kuchenbuch M, Barcia G, Schneider A, Jennesson M, Le Guyader G, Lesca G, Mignot C, Montomoli M, Parrini E, Isnard H, Rolland A, Keren B, Afenjar A, Dorison N, Sadleir LG, Breuillard D, Levy R, Rio M, Dupont S, Negrin S, Danieli A, Scalais E, De Saint Martin A, El Chehadeh S, Chelly J, Poisson A, Lebre AS, Nica A, Odent S, Sekhara T, Brankovic V, Goldenberg A, Vrielynck P, Lederer D, Maurey H, Terrone G, Besmond C, Hubert L, Berquin P, Billette de Villemeur T, Isidor B, Freeman JL, Mefford HC, Myers CT, Howell KB, Rodríguez-Sacristán Cascajo A, Meyer P, Genevieve D, Guët A, Doummar D, Durigneux J, van Dooren MF, de Wit MCY, Gerard M, Marey I, Munnich A, Guerrini R, Scheffer IE, Kabashi E, Nabbout R
Epilepsia 2020 Nov;61(11):2461-2473. Epub 2020 Sep 21 doi: 10.1111/epi.16679. PMID: 32954514
Bellinger DC, O'Leary K, Rainis H, Gibb HJ
Environ Res 2016 May;147:159-63. Epub 2016 Feb 11 doi: 10.1016/j.envres.2015.10.006. PMID: 26874048
Van Balkom ID, Vuijk PJ, Franssens M, Hoek HW, Hennekam RC
Dev Med Child Neurol 2012 Oct;54(10):925-31. Epub 2012 Jun 19 doi: 10.1111/j.1469-8749.2012.04339.x. PMID: 22712893

Clinical prediction guides

Sandal S, Verma IC, Mahay SB, Dubey S, Sabharwal RK, Kulshrestha S, Saxena R, Suman P, Kumar P, Puri RD
Indian J Pediatr 2024 Jul;91(7):682-695. Epub 2023 Oct 7 doi: 10.1007/s12098-023-04820-5. PMID: 37804371
Stamberger H, Crosiers D, Balagura G, Bonardi CM, Basu A, Cantalupo G, Chiesa V, Christensen J, Dalla Bernardina B, Ellis CA, Furia F, Gardiner F, Giron C, Guerrini R, Klein KM, Korff C, Krijtova H, Leffler M, Lerche H, Lesca G, Lewis-Smith D, Marini C, Marjanovic D, Mazzola L, McKeown Ruggiero S, Mochel F, Ramond F, Reif PS, Richard-Mornas A, Rosenow F, Schropp C, Thomas RH, Vignoli A, Weber Y, Palmer E, Helbig I, Scheffer IE, Striano P, Møller RS, Gardella E, Weckhuysen S
Neurology 2022 Jul 19;99(3):e221-e233. Epub 2022 Jun 3 doi: 10.1212/WNL.0000000000200715. PMID: 35851549Free PMC Article
Wirth T, Garone G, Kurian MA, Piton A, Millan F, Telegrafi A, Drouot N, Rudolf G, Chelly J, Marks W, Burglen L, Demailly D, Coubes P, Castro-Jimenez M, Joriot S, Ghoumid J, Belin J, Faucheux JM, Blumkin L, Hull M, Parnes M, Ravelli C, Poulen G, Calmels N, Nemeth AH, Smith M, Barnicoat A, Ewenczyk C, Méneret A, Roze E, Keren B, Mignot C, Beroud C, Acosta F Jr, Nowak C, Wilson WG, Steel D, Capuano A, Vidailhet M, Lin JP, Tranchant C, Cif L, Doummar D, Anheim M
Mov Disord 2022 Jul;37(7):1547-1554. Epub 2022 Jun 20 doi: 10.1002/mds.29074. PMID: 35722775Free PMC Article
Zaman T, Helbig I, Božović IB, DeBrosse SD, Bergqvist AC, Wallis K, Medne L, Maver A, Peterlin B, Helbig KL, Zhang X, Goldberg EM
Ann Neurol 2018 Apr;83(4):703-717. Epub 2018 Mar 30 doi: 10.1002/ana.25188. PMID: 29466837Free PMC Article
Walton C, Kerr M
J Appl Res Intellect Disabil 2016 Sep;29(5):395-408. Epub 2015 Jun 22 doi: 10.1111/jar.12203. PMID: 26101049

Recent systematic reviews

Hanley E, Martin AM, Dalton C, Lehane E
J Intellect Disabil 2023 Dec;27(4):1107-1134. Epub 2022 Jul 18 doi: 10.1177/17446295221115914. PMID: 35850628Free PMC Article
Eaton C, Tarver J, Shirazi A, Pearson E, Walker L, Bird M, Oliver C, Waite J
J Intellect Disabil Res 2021 Mar;65(3):211-229. Epub 2021 Jan 11 doi: 10.1111/jir.12807. PMID: 33426741
Mazza MG, Rossetti A, Crespi G, Clerici M
J Appl Res Intellect Disabil 2020 Mar;33(2):126-138. Epub 2019 Aug 20 doi: 10.1111/jar.12654. PMID: 31430018
Bellinger DC, O'Leary K, Rainis H, Gibb HJ
Environ Res 2016 May;147:159-63. Epub 2016 Feb 11 doi: 10.1016/j.envres.2015.10.006. PMID: 26874048
Walton C, Kerr M
J Appl Res Intellect Disabil 2016 Sep;29(5):395-408. Epub 2015 Jun 22 doi: 10.1111/jar.12203. PMID: 26101049

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