Childhood disintegrative disorder

Childhood disintegrative disorder (CDD), also known as Heller's syndrome and disintegrative psychosis, is a rare condition characterized by late onset of developmental delays—or severe and sudden reversals—in language (receptive and expressive), social engagement, bowel and bladder, play and motor skills.[3][4] Researchers have not been successful in finding a cause for the disorder.[3][4] CDD has some similarities to autism and is sometimes considered a low-functioning form of it.[5][6][4] In May 2013, CDD, along with other sub-types of PDD (Asperger's syndrome, Classic autism, and PDD-NOS), was fused into a single diagnostic term called "autism spectrum disorder" under the new DSM-5 manual.[7]

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, certain seizure disorders (e.g. Landau-Kleffner syndrome), and some neurodegenerative diseases (e.g.variant Creutzfeldt-Jakob disease)[1][2]

CDD was originally described by Austrian educator Theodor Heller (1869–1938) in 1908, 35 years before Leo Kanner and Hans Asperger described autism. Heller had previously used the name dementia infantilis for 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 a 'prodrome,' 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 to hallucinations, but the most obvious symptom is that skills apparently attained are lost.[4]

Many children are 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 all pervasive developmental disorder categories, there is considerable controversy about the right treatment for CDD.[4]

Signs and symptoms

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 book, Thinking in Pictures, Temple Grandin argues that compared to "Kanner's classic autism" and to Asperger syndrome, CDD is characterized with more severe sensory processing disorder but less severe cognitive problems.[20] She also argues that compared to most autistic individuals, persons with CDD have more severe speech pathology and they usually do not respond well to stimulants.[20]

Causes

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. A Mayo Clinic report indicates: "Comprehensive medical and neurological examinations in children diagnosed with childhood disintegrative disorder seldom uncover an underlying medical or neurological cause. Although the occurrence of epilepsy is 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 the measles virus causes 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, the myelin sheath does not develop in a normal way, causing white matter in the brain to eventually fail and disintegrate.

Treatment

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 for Autism spectrum disorders by the American 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. Antipsychotic medications are used to treat severe behavior problems like aggressive stance and repetitive behavior patterns. Anticonvulsant medications are used to control seizures.

References
  1. Dr.Mary Lowthe. "Childhood Disintegrative Disorder".
  2. Hiroshi Kurita (2011). Textbook of Autism Spectrum Disorders. American Psychiatric Pub. p. 102. ISBN 9781585623419.
  3. Charan, Sri Hari (January–April 2012). "Childhood disintegrative disorder". Journal of Pediatric Neurosciences. 7 (1): 55–57. doi:10.4103/1817-1745.97627. PMC 3401658. PMID 22837782. 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. "Definitions for childhood disintegrative disorder". Definitions.net. STANDS4. Archived from the original on 25 September 2022. Retrieved 25 September 2022. 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. pp. 407–18. doi:10.1016/B978-0-444-52002-9.00023-1. ISBN 9780444520029. PMC 3848246. PMID 22608634. {{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. PMID 22427366. S2CID 12331005.
  7. "Childhood Disintegrative Disorder (Heller's Syndrome)".
  8. Mouridsen SE (June 2003). "Childhood disintegrative disorder". Brain Dev. 25 (4): 225–8. doi:10.1016/s0387-7604(02)00228-0. PMID 12767450. S2CID 25420772.
  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. PMID 15362172.
  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. PMID 10660829.
  11. Malhotra S, Gupta N (December 1999). "Childhood disintegrative disorder". J Autism Dev Disord. 29 (6): 491–8. doi:10.1023/A:1022247903401. PMID 10638461. S2CID 189899310.
  12. Dobbs, David (6 July 2016). "The most terrifying childhood condition you've never heard of". Spectrum. Simons Foundation Autism Research Initiative. Archived from the original on 25 September 2022. Retrieved 25 September 2022. 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. PMID 12083281. S2CID 145771483.
  14. Fombonne E (June 2009). "Epidemiology of pervasive developmental disorders". Pediatr. Res. 65 (6): 591–8. doi:10.1203/PDR.0b013e31819e7203. PMID 19218885.
  15. Fombonne, Eric (2002). "Prevalence of Childhood Disintegrative Disorder". Autism. 6 (2): 149–157. doi:10.1177/1362361302006002002. PMID 12083281. S2CID 145771483.
  16. Charan, Sri Hari (2012). "Childhood disintegrative disorder". Journal of Pediatric Neurosciences. 7 (1): 55–57. doi:10.4103/1817-1745.97627. ISSN 1817-1745. PMC 3401658. PMID 22837782.
  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. ISSN 1573-3432. PMID 10638461. S2CID 189899310.
  18. "A Guide to Childhood Disintegrative Disorder". Elemy. Archived from the original on 25 September 2022. Retrieved 25 September 2022.
  19. Braaten, Ellen, ed. (29 January 2018). The SAGE Encyclopedia of Intellectual and Developmental Disorders. Vol. 2. SAGE Publications. ISBN 9781506353296. 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. Grandin, Temple (7 September 2008). Thinking in Pictures. Bloomsbury Publishing. ISBN 9781408807309. Retrieved 25 September 2022.
  21. Childhood Disintegrative Disorder - Causes Archived September 29, 2007, at the Wayback Machine
  22. Mughal, Saba; Faizy, Rubina M.; Saadabadi, Abdolreza (January 2022). Autism Spectrum Disorder. Treasure Island, Florida: StatPearls Publishing. PMID 30247851. Archived from 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. ISSN 0031-4005. PMID 17967921.
  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. PMC 5704147. PMID 29072583.
  26. "Applied Behavioral Strategies - Getting to Know ABA". Archived from the original on 2015-10-07. Retrieved 2015-12-16.

Further reading
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