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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.
See: This record
Not genetically inherited (Orphanet)
 
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

Autism Spectrum Disorder: Primary Care Principles.
Sanchack KE, Thomas CA
Am Fam Physician 2016 Dec 15;94(12):972-979. PMID: 28075089
Approach to autism spectrum disorder: Using the new DSM-V diagnostic criteria and the CanMEDS-FM framework.
Lee PF, Thomas RE, Lee PA
Can Fam Physician 2015 May;61(5):421-4. PMID: 25971758Free PMC Article

Recent clinical studies

Etiology

Developmental regression in children: Current and future directions.
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
Childhood disintegrative disorder and autism spectrum disorder: a systematic review.
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
Autism and related disorders.
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
Comparison of childhood disintegrative disorder and disintegrative psychosis not diagnosed as childhood disintegrative disorder.
Kurita H, Koyama T, Osada H
Psychiatry Clin Neurosci 2005 Apr;59(2):200-5. doi: 10.1111/j.1440-1819.2005.01358.x. PMID: 15823168
Childhood disintegrative disorder: issues for DSM-IV.
Volkmar FR
J Autism Dev Disord 1992 Dec;22(4):625-42. doi: 10.1007/BF01046331. PMID: 1483980

Diagnosis

Autism Spectrum Disorder: Primary Care Principles.
Sanchack KE, Thomas CA
Am Fam Physician 2016 Dec 15;94(12):972-979. PMID: 28075089
Autism and related disorders.
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
Epilepsy-aphasia syndromes.
Steinlein OK
Expert Rev Neurother 2009 Jun;9(6):825-33. doi: 10.1586/ern.09.49. PMID: 19496686
Childhood disintegrative disorder.
Mouridsen SE
Brain Dev 2003 Jun;25(4):225-8. doi: 10.1016/s0387-7604(02)00228-0. PMID: 12767450
Childhood disintegrative disorder.
Malhotra S, Gupta N
J Autism Dev Disord 1999 Dec;29(6):491-8. doi: 10.1023/a:1022247903401. PMID: 10638461

Therapy

Autism Spectrum Disorder: Primary Care Principles.
Sanchack KE, Thomas CA
Am Fam Physician 2016 Dec 15;94(12):972-979. PMID: 28075089
Brief report: childhood disintegrative disorder as a likely manifestation of vitamin B12 deficiency.
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
Care of the patient with an autism spectrum disorder by the general physician.
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
Corticosteroid treatment of behaviour, language and motor regression in childhood disintegrative disorder.
Mordekar SR, Prendergast M, Chattopadhyay AK, Baxter PS
Eur J Paediatr Neurol 2009 Jul;13(4):367-9. Epub 2008 Jul 14 doi: 10.1016/j.ejpn.2008.06.001. PMID: 18625572
Risperidone treatment of children and adolescents with pervasive developmental disorders: a prospective open-label study.
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

Childhood disintegrative disorder and autism spectrum disorder: a systematic review.
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
Autism Spectrum Disorder: Primary Care Principles.
Sanchack KE, Thomas CA
Am Fam Physician 2016 Dec 15;94(12):972-979. PMID: 28075089
Epilepsy-aphasia syndromes.
Steinlein OK
Expert Rev Neurother 2009 Jun;9(6):825-33. doi: 10.1586/ern.09.49. PMID: 19496686
Prevalence of childhood disintegrative disorder.
Fombone E
Autism 2002 Jun;6(2):149-57. doi: 10.1177/1362361302006002002. PMID: 12083281
Childhood disintegrative disorder: issues for DSM-IV.
Volkmar FR
J Autism Dev Disord 1992 Dec;22(4):625-42. doi: 10.1007/BF01046331. PMID: 1483980

Clinical prediction guides

Neurogenetic analysis of childhood disintegrative disorder.
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
A new structured interview for children with autism spectrum disorder based on the DSM-IV.
Hansakunachai T, Roongpraiwan R, Sombuntham T, Limprasert P, Ruangdaraganon N
J Med Assoc Thai 2014 Aug;97 Suppl 8:S7-14. PMID: 25518287
Autism, an overwhelming condition: history, etiopathogenesis, types, diagnosis, therapy and prognosis.
Amihăesei IC, Stefanachi E
Rev Med Chir Soc Med Nat Iasi 2013 Jul-Sep;117(3):654-61. PMID: 24502031
Childhood disintegrative disorder: distinction from autistic disorder and predictors of outcome.
Rosman NP, Bergia BM
J Child Neurol 2013 Dec;28(12):1587-98. Epub 2013 Jan 22 doi: 10.1177/0883073812472391. PMID: 23340080
Childhood autism rating scale--Tokyo version for screening pervasive developmental disorders.
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

Childhood disintegrative disorder and autism spectrum disorder: a systematic review.
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
Addressing sequelae of developmental regression associated with developmental disabilities: A systematic review of behavioral and educational intervention studies.
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
Approach to autism spectrum disorder: Using the new DSM-V diagnostic criteria and the CanMEDS-FM framework.
Lee PF, Thomas RE, Lee PA
Can Fam Physician 2015 May;61(5):421-4. PMID: 25971758Free PMC Article

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