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Childhood disintegrative disorder

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Childhood disintegrative disorder
Other namesHeller syndrome,
disintegrative psychosis,
dementia infantilis
SpecialtyPsychiatry,pediatrics
Usual onset3-4 years of age
Differential diagnosisregressive autism,Rett syndrome,lead poisoning,mercury poisoning,HIV infection,brain tumor,certainseizure disorders(e.g.Landau-Kleffner syndrome), and someneurodegenerative diseases(e.g.variant Creutzfeldt-Jakob disease)[1][2]

Childhood disintegrative disorder(CDD), also known asHeller's syndromeanddisintegrative psychosis,is a rare condition characterized by late onset ofdevelopmentaldelays—or severe and sudden reversals—inlanguage (receptive and expressive),social engagement,bowel and bladder, play andmotor skills.[3][4]Researchers have not been successful in finding a cause for thedisorder.[3][4]CDD has some similarities toautismand is sometimes considered alow-functioningform of it.[5][6][4]In May 2013, CDD, along with other sub-types of PDD (Asperger's syndrome,Classic autism,andPDD-NOS), was fused into a single diagnostic term called "autism spectrum disorder"under the newDSM-5manual.[7]

CDD was originally described by Austrian educator Theodor Heller (1869–1938) in 1908, 35 years beforeLeo KannerandHans Aspergerdescribedautism.Heller had previously used the namedementia infantilisfor the syndrome.[8]

An apparent period of fairly normal development is often noted before a regression in skills or a series of regressions in skills.[9][4]The age at which this regression can occur varies;[10]after three years of normal development is typical.[11]The regression, known as aprodrome,can be so dramatic that the child may be aware of it, and may in its beginning even ask, vocally, what is happening to them.[4][12]Some children describe or appear to be reacting tohallucinations,but the most obvious symptom is that skills apparently attained are lost.[4]

Manychildrenare already somewhat delayed when the disorder becomes apparent, but these delays are not always obvious in young children.[4]This has been described by many writers as a devastating condition, affecting both the family and the individual's future.[4]As is the case with allpervasive developmental disordercategories, there is considerable controversy about the right treatment for CDD.[4]

Signs and symptoms

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CDD is a rare condition, with only 1.7 cases per 100,000.[13][14][15]

A child affected with childhood disintegrative disorder shows normal development. Up until this point, the child has developed normally in the areas of language skills, social skills, comprehension skills, and has maintained those skills for about two years.[16][17]However, between the ages of two and 10, skills acquired are lost almost completely in at least two of the following six functional areas:[18]

Lack of normal function or impairment also occurs in at least two of the following three areas:[19]

In her bookThinking in Pictures,Temple Grandinargues that compared to "Kanner's classic autism" and to Asperger syndrome, CDD is characterized with more severesensory processing disorderbut less severecognitiveproblems.[20]She also argues that compared to most autistic individuals, persons with CDD have more severespeech pathologyand they usually do not respond well tostimulants.[20]

Causes

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All of the causes of childhood disintegrative disorder are still unknown.[3][4]Sometimes CDD surfaces abruptly within days or weeks, while in other cases it develops over a longer period of time. AMayo Clinicreport indicates: "Comprehensive medical and neurological examinations in children diagnosed with childhood disintegrative disorder seldom uncover an underlying medical or neurological cause. Although the occurrence ofepilepsyis higher in children with childhood disintegrative disorder, experts don't know whether epilepsy plays a role in causing the disorder. "[21]

CDD, especially in cases of later age of onset, has also been associated with certain other conditions, particularly the following:[11]

  • Lipid storage diseases:In this condition, a toxic buildup of excess fats (lipids) takes place in the brain and nervous system.
  • Subacute sclerosing panencephalitis:Chronic infection of the brain by a form of themeasles viruscauses subacute sclerosing panencephalitis. This condition leads to brain inflammation and the death of nerve cells.
  • Tuberous sclerosis(TSC): TSC is a genetic disorder. In this disorder, tumors may grow in the brain and other vital organs like kidneys, heart, eyes, lungs, and skin. In this condition, noncancerous (benign)tumors,hamartomas,grow in the brain.
  • Leukodystrophy:In this condition, themyelin sheathdoes not develop in a normal way, causingwhite matterin the brain to eventually fail and disintegrate.

Treatment

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Loss of language and skills related to social interaction and self-care are serious. The affected children face ongoing disabilities in certain areas and require long-term care. Treatment of CDD involves both behavior therapy, environmental therapy and medications.[22]

  • Behavior therapy:Applied behavior analysis(ABA) is considered to be the most effective form of treatment forautism spectrumdisorders by theAmerican Academy of Pediatrics.[23]The primary goal of ABA is to improve quality of life, and independence by teaching adaptive behaviors to children with autism,[24]and to diminish problematic behaviors like running away from home, or self-injury[25]by using positive or negative reinforcement to encourage or discourage behaviors over time.[26]
  • Environmental therapy:Sensory enrichment therapy uses enrichment of the sensory experience to improve symptoms in autism, many of which are common to CDD.
  • Medications:There are no medications available to directly treat CDD.Antipsychoticmedications are used to treat severe behavior problems like aggressive stance and repetitive behavior patterns.Anticonvulsantmedications are used to controlseizures.

References

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  1. ^Dr.Mary Lowthe (25 January 2021)."Childhood Disintegrative Disorder".
  2. ^Hiroshi Kurita (2011).Textbook of Autism Spectrum Disorders.American Psychiatric Pub. p. 102.ISBN9781585623419.
  3. ^abcCharan, Sri Hari (January–April 2012)."Childhood disintegrative disorder".Journal of Pediatric Neurosciences.7(1): 55–57.doi:10.4103/1817-1745.97627.PMC3401658.PMID22837782.Childhood disintegrative disorder (CDD), also known as Heller's syndrome and disintegrative psychosis, is a rare condition characterized by late onset (>3 years of age) of developmental delays in language, social function, and motor skills. [...] The cause of childhood disintegrative disorder is unknown.
  4. ^abcdefghij"Definitions for childhood disintegrative disorder".Definitions.net.STANDS4.Archivedfrom the original on 25 September 2022.Retrieved25 September2022.Childhood disintegrative disorder, also known as Heller's syndrome and disintegrative psychosis, is a rare condition characterized by late onset of developmental delays in language, social function, and motor skills. Researchers have not been successful in finding a cause for the disorder. CDD has some similarity to autism, and is sometimes considered a low-functioning form of it, but an apparent period of fairly normal development is often noted before a regression in skills or a series of regressions in skills. Many children are already somewhat delayed when the disorder becomes apparent, but these delays are not always obvious in young children. [...] The regression can be so dramatic, that the child may be aware of it, and in its beginning he may even ask, vocally, what is happening to him. Some children describe or appear to be reacting to hallucinations, but the most obvious symptom is that skills apparently attained are lost. This has been described by many writers as a devastating condition, affecting both the family and the individual's future. As is the case with all pervasive developmental disorder categories, there is considerable controversy about the right treatment for CDD.
  5. ^McPartland J, Volkmar FR (2012). "Autism and related disorders".Neurobiology of Psychiatric Disorders.Handbook of Clinical Neurology. Vol. 106. pp. 407–18.doi:10.1016/B978-0-444-52002-9.00023-1.ISBN9780444520029.PMC3848246.PMID22608634.{{cite book}}:|journal=ignored (help)
  6. ^Venkat A, Jauch E, Russell WS, Crist CR, Farrell R (August 2012). "Care of the patient with an autism by the general physician".Postgrad Med J.88(1042): 472–81.doi:10.1136/postgradmedj-2011-130727.PMID22427366.S2CID12331005.
  7. ^"Childhood Disintegrative Disorder (Heller's Syndrome)".25 January 2021.
  8. ^Mouridsen SE (June 2003). "Childhood disintegrative disorder".Brain Dev.25(4): 225–8.doi:10.1016/s0387-7604(02)00228-0.PMID12767450.S2CID25420772.
  9. ^Rogers SJ (2004)."Developmental regression in autism spectrum disorders".Ment Retard Dev Disabil Res Rev.10(2): 139–43.doi:10.1002/mrdd.20027.PMID15362172.
  10. ^Hendry CN (January 2000). "Childhood disintegrative disorder: should it be considered a distinct diagnosis?".Clin Psychol Rev.20(1): 77–90.doi:10.1016/S0272-7358(98)00094-4.PMID10660829.
  11. ^abMalhotra S, Gupta N (December 1999). "Childhood disintegrative disorder".J Autism Dev Disord.29(6): 491–8.doi:10.1023/A:1022247903401.PMID10638461.S2CID189899310.
  12. ^Dobbs, David (6 July 2016)."The most terrifying childhood condition you've never heard of".Spectrum.Simons Foundation Autism Research Initiative.Archivedfrom the original on 25 September 2022.Retrieved25 September2022.During this anxiety-ridden prologue, known as a 'prodrome,' a child will often seem keenly aware that something is wrong.
  13. ^Fombone E (June 2002). "Prevalence of childhood disintegrative disorder".Autism.6(2): 149–57.doi:10.1177/1362361302006002002.PMID12083281.S2CID145771483.
  14. ^Fombonne E (June 2009)."Epidemiology of pervasive developmental disorders".Pediatr. Res.65(6): 591–8.doi:10.1203/PDR.0b013e31819e7203.PMID19218885.
  15. ^Fombonne, Eric (2002). "Prevalence of Childhood Disintegrative Disorder".Autism.6(2): 149–157.doi:10.1177/1362361302006002002.PMID12083281.S2CID145771483.
  16. ^Charan, Sri Hari (2012)."Childhood disintegrative disorder".Journal of Pediatric Neurosciences.7(1): 55–57.doi:10.4103/1817-1745.97627.ISSN1817-1745.PMC3401658.PMID22837782.
  17. ^Malhotra, Savita; Gupta, Nitin (1999-12-01). "Childhood Disintegrative Disorder".Journal of Autism and Developmental Disorders.29(6): 491–498.doi:10.1023/A:1022247903401.ISSN1573-3432.PMID10638461.S2CID189899310.
  18. ^"A Guide to Childhood Disintegrative Disorder".Elemy.Archivedfrom the original on 25 September 2022.Retrieved25 September2022.
  19. ^Braaten, Ellen, ed. (29 January 2018).The SAGE Encyclopedia of Intellectual and Developmental Disorders.Vol. 2. SAGE Publications.ISBN9781506353296.Lack of normal function or impairment also occurs in at least two of the following three areas: Social interaction, Communication, and Repetitive behavior and interest patterns.
  20. ^abGrandin, Temple (7 September 2008).Thinking in Pictures.Bloomsbury Publishing.ISBN9781408807309.Retrieved25 September2022.
  21. ^Childhood Disintegrative Disorder - CausesArchivedSeptember 29, 2007, at theWayback Machine
  22. ^Mughal, Saba; Faizy, Rubina M.; Saadabadi, Abdolreza (January 2022).Autism Spectrum Disorder.Treasure Island, Florida: StatPearls Publishing.PMID30247851.Archivedfrom the original on 25 September 2022 – via National Center for Biotechnology Information Bookshelf.Treatment of childhood disintegrative disorder requires behavior therapy, environmental therapy, and medications.
  23. ^Myers, Scott M.; Johnson, Chris Plauché (1 November 2007)."Management of Children With Autism Spectrum Disorders".Pediatrics.120(5): 1162–1182.doi:10.1542/peds.2007-2362.ISSN0031-4005.PMID17967921.
  24. ^Matson, Johnny; Hattier, Megan; Belva, Brian (January–March 2012). "Treating adaptive living skills of persons with autism using applied behavior analysis: A review".Research in Autism Spectrum Disorders.6(1): 271–276.doi:10.1016/j.rasd.2011.05.008.
  25. ^Summers, Jane; Sharami, Ali; Cali, Stefanie; D'Mello, Chantelle; Kako, Milena; Palikucin-Reljin, Andjelka; Savage, Melissa; Shaw, Olivia; Lunsky, Yona (November 2017)."Self-Injury in Autism Spectrum Disorder and Intellectual Disability: Exploring the Role of Reactivity to Pain and Sensory Input".Brain Sci.7(11): 140.doi:10.3390/brainsci7110140.PMC5704147.PMID29072583.
  26. ^"Applied Behavioral Strategies - Getting to Know ABA".Archivedfrom the original on 2015-10-07.Retrieved2015-12-16.

Further reading

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