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Childhood disintegrative disorder

MedGen UID:
472967
Concept ID:
C0236791
Mental or Behavioral Dysfunction
Synonym: Childhood disintegrative disease
SNOMED CT: Childhood disintegrative disorder (71961003); Heller's syndrome (71961003); Dementia infantilis (71961003); Disintegrative psychosis (71961003); CDD - Childhood disintegrative disorder (71961003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0015681
Orphanet: ORPHA168782

Definition

Childhood disintergrative disorder is a rare pervasive developmental disorder with a disease onset before the age of three and characterized by a dramatic loss of behavioral and developmental functioning after atleast two years of normal development. Manifestations of the disease include loss of speech, incontinence, communication and social interaction problems, stereotypical autistic behaviors and dementia. [from ORDO]

Professional guidelines

PubMed

Sanchack KE, Thomas CA
Am Fam Physician 2016 Dec 15;94(12):972-979. PMID: 28075089
Lee PF, Thomas RE, Lee PA
Can Fam Physician 2015 May;61(5):421-4. PMID: 25971758Free PMC Article

Recent clinical studies

Etiology

Furley K, Mehra C, Goin-Kochel RP, Fahey MC, Hunter MF, Williams K, Absoud M
Cortex 2023 Dec;169:5-17. Epub 2023 Sep 27 doi: 10.1016/j.cortex.2023.09.001. PMID: 37839389
Mehra C, Sil A, Hedderly T, Kyriakopoulos M, Lim M, Turnbull J, Happe F, Baird G, Absoud M
Dev Med Child Neurol 2019 May;61(5):523-534. Epub 2018 Dec 13 doi: 10.1111/dmcn.14126. PMID: 30548847
Gupta AR, Westphal A, Yang DYJ, Sullivan CAW, Eilbott J, Zaidi S, Voos A, Vander Wyk BC, Ventola P, Waqar Z, Fernandez TV, Ercan-Sencicek AG, Walker MF, Choi M, Schneider A, Hedderly T, Baird G, Friedman H, Cordeaux C, Ristow A, Shic F, Volkmar FR, Pelphrey KA
Mol Autism 2017;8:19. Epub 2017 Apr 4 doi: 10.1186/s13229-017-0133-0. PMID: 28392909Free PMC Article
McPartland J, Volkmar FR
Handb Clin Neurol 2012;106:407-18. doi: 10.1016/B978-0-444-52002-9.00023-1. PMID: 22608634Free PMC Article
Volkmar FR
J Autism Dev Disord 1992 Dec;22(4):625-42. doi: 10.1007/BF01046331. PMID: 1483980

Diagnosis

Mehra C, Sil A, Hedderly T, Kyriakopoulos M, Lim M, Turnbull J, Happe F, Baird G, Absoud M
Dev Med Child Neurol 2019 May;61(5):523-534. Epub 2018 Dec 13 doi: 10.1111/dmcn.14126. PMID: 30548847
Sanchack KE, Thomas CA
Am Fam Physician 2016 Dec 15;94(12):972-979. PMID: 28075089
McPartland J, Volkmar FR
Handb Clin Neurol 2012;106:407-18. doi: 10.1016/B978-0-444-52002-9.00023-1. PMID: 22608634Free PMC Article
Mouridsen SE
Brain Dev 2003 Jun;25(4):225-8. doi: 10.1016/s0387-7604(02)00228-0. PMID: 12767450
Malhotra S, Gupta N
J Autism Dev Disord 1999 Dec;29(6):491-8. doi: 10.1023/a:1022247903401. PMID: 10638461

Therapy

Sanchack KE, Thomas CA
Am Fam Physician 2016 Dec 15;94(12):972-979. PMID: 28075089
Wattchow N, Lee HE, Brock P
Australas Psychiatry 2015 Feb;23(1):63-5. Epub 2014 Dec 17 doi: 10.1177/1039856214563844. PMID: 25520002
Malhotra S, Subodh BN, Parakh P, Lahariya S
J Autism Dev Disord 2013 Sep;43(9):2207-10. doi: 10.1007/s10803-013-1762-6. PMID: 23334842
Venkat A, Jauch E, Russell WS, Crist CR, Farrell R
Postgrad Med J 2012 Aug;88(1042):472-81. Epub 2012 Mar 16 doi: 10.1136/postgradmedj-2011-130727. PMID: 22427366
McDougle CJ, Holmes JP, Bronson MR, Anderson GM, Volkmar FR, Price LH, Cohen DJ
J Am Acad Child Adolesc Psychiatry 1997 May;36(5):685-93. doi: 10.1097/00004583-199705000-00020. PMID: 9136504

Prognosis

Mehra C, Sil A, Hedderly T, Kyriakopoulos M, Lim M, Turnbull J, Happe F, Baird G, Absoud M
Dev Med Child Neurol 2019 May;61(5):523-534. Epub 2018 Dec 13 doi: 10.1111/dmcn.14126. PMID: 30548847
Sanchack KE, Thomas CA
Am Fam Physician 2016 Dec 15;94(12):972-979. PMID: 28075089
Steinlein OK
Expert Rev Neurother 2009 Jun;9(6):825-33. doi: 10.1586/ern.09.49. PMID: 19496686
Fombone E
Autism 2002 Jun;6(2):149-57. doi: 10.1177/1362361302006002002. PMID: 12083281
Volkmar FR
J Autism Dev Disord 1992 Dec;22(4):625-42. doi: 10.1007/BF01046331. PMID: 1483980

Clinical prediction guides

Gupta AR, Westphal A, Yang DYJ, Sullivan CAW, Eilbott J, Zaidi S, Voos A, Vander Wyk BC, Ventola P, Waqar Z, Fernandez TV, Ercan-Sencicek AG, Walker MF, Choi M, Schneider A, Hedderly T, Baird G, Friedman H, Cordeaux C, Ristow A, Shic F, Volkmar FR, Pelphrey KA
Mol Autism 2017;8:19. Epub 2017 Apr 4 doi: 10.1186/s13229-017-0133-0. PMID: 28392909Free PMC Article
Hansakunachai T, Roongpraiwan R, Sombuntham T, Limprasert P, Ruangdaraganon N
J Med Assoc Thai 2014 Aug;97 Suppl 8:S7-14. PMID: 25518287
Amihăesei IC, Stefanachi E
Rev Med Chir Soc Med Nat Iasi 2013 Jul-Sep;117(3):654-61. PMID: 24502031
Rosman NP, Bergia BM
J Child Neurol 2013 Dec;28(12):1587-98. Epub 2013 Jan 22 doi: 10.1177/0883073812472391. PMID: 23340080
Tachimori H, Osada H, Kurita H
Psychiatry Clin Neurosci 2003 Feb;57(1):113-8. doi: 10.1046/j.1440-1819.2003.01087.x. PMID: 12519463

Recent systematic reviews

Mehra C, Sil A, Hedderly T, Kyriakopoulos M, Lim M, Turnbull J, Happe F, Baird G, Absoud M
Dev Med Child Neurol 2019 May;61(5):523-534. Epub 2018 Dec 13 doi: 10.1111/dmcn.14126. PMID: 30548847
Sigafoos J, O'Reilly MF, Ledbetter-Cho K, Lim N, Lancioni GE, Marschik PB
Neurosci Biobehav Rev 2019 Jan;96:56-71. Epub 2018 Nov 24 doi: 10.1016/j.neubiorev.2018.11.014. PMID: 30481529
Lee PF, Thomas RE, Lee PA
Can Fam Physician 2015 May;61(5):421-4. PMID: 25971758Free PMC Article

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