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{{DiseaseDisorder infobox |
  
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{{Infobox disease |
 
Name = Childhood disintegrative disorder |
  
Name = Childhood disintegrative disorder |
 
ICD10 = F84.2-F84.3 |
  
ICD10 = F84.2-F84.3 |
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ICD9 = 299.10-299.11 |
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ICD9 = {{ICD9|299.10}}-{{ICD9|299.11}} |
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MedlinePlus = 001535 |
 
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'''Childhood disintegrative disorder''' ('''CDD'''), also known as '''Heller's syndrome''' and '''disintegrative psychosis''', was originally described by Austrian educator Theodor Heller in 1908, 35 years before [[Leo Kanner]] and [[Hans Asperger]] described [[autism]], but it has not been officially recognised until recently. Heller had previously used the name ''dementia infantilis'' for the syndrome.<ref name="Mouridsen 2003">{{cite journal |author=Mouridsen SE |title=Childhood disintegrative disorder |journal=Brain Dev. |volume=25 |issue=4 |pages=225–8 |year=2003 |month=June |pmid=12767450 |doi= |url=}}</ref> CDD is a rare condition(1.7 cases per 100,000 condition).<ref name="Fombone 2002">{{cite journal |author=Fombone E |title=Prevalence of childhood disintegrative disorder |journal=Autism |volume=6 |issue=2 |pages=149–57 |year=2002 |month=June |pmid=12083281 |doi=10.1177/1362361302006002002 }}</ref><ref name="Fombonne 2009">{{cite journal |author=Fombonne E |title=Epidemiology of pervasive developmental disorders |journal=Pediatr. Res. |volume=65 |issue=6 |pages=591–8 |year=2009 |month=June |pmid=19218885 |doi=10.1203/PDR.0b013e31819e7203 |url=}}</ref> Characterized by late onset (>3 years of age) of [[Child development|developmental]] delays in [[language]], [[social function]], and [[motor skills]]. Researchers have not been successful in finding a cause for the [[mental illness|disorder]]. CDD has some similarity to [[autism]], and is sometimes considered a low-functioning form of it.<ref name="McPartland 2012">{{cite journal |author=McPartland J, Volkmar FR |title=Autism and related disorders |journal=Handb Clin Neurol |volume=106 |issue= |pages=407–18 |year=2012 |pmid=22608634 |doi=10.1016/B978-0-444-52002-9.00023-1 |url=}}</ref><ref name="Venkat 2012">{{cite journal |author=Venkat A, Jauch E, Russell WS, Crist CR, Farrell R |title=Care of the patient with an autism spectrum disorder by the general physician |journal=Postgrad Med J |volume=88 |issue=1042 |pages=472–81 |year=2012 |month=August |pmid=22427366 |doi=10.1136/postgradmedj-2011-130727 |url=}}</ref>
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'''Childhood disintegrative disorder''' ('''CDD''') is a rare condition characterized by late onset (>3 years of age) of [[development]]al delays in [[language]], [[social function]], and [[motor skills]]. Researchers have not been successful in finding a cause for the [[disorder]].
  
   
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An apparent period of fairly normal development is often noted before a regression in skills or a series of regressions in skills.<ref name="Rogers 2004">{{cite journal |author=Rogers SJ |title=Developmental regression in autism spectrum disorders |journal=Ment Retard Dev Disabil Res Rev |volume=10 |issue=2 |pages=139–43 |year=2004 |pmid=15362172 |doi=10.1002/mrdd.20027 |url=}}</ref> The age at which this regression can occur varies,<ref name="Hendry 2000">{{cite journal |author=Hendry CN |title=Childhood disintegrative disorder: should it be considered a distinct diagnosis? |journal=Clin Psychol Rev |volume=20 |issue=1 |pages=77–90 |year=2000 |month=January |pmid=10660829 |doi= 10.1016/S0272-7358(98)00094-4}}</ref> but typically after 3 years of normal development.<ref name="Malhotra 1999">{{cite journal |author=Malhotra S, Gupta N |title=Childhood disintegrative disorder |journal=J Autism Dev Disord |volume=29 |issue=6 |pages=491–8 |year=1999 |month=December |pmid=10638461 |doi= |url=}}</ref> The regression 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. Some children describe or appear to be reacting to [[hallucination]]s, but the most obvious symptom is that skills apparently attained are lost.
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CDD has some similarity to [[autism]], 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 illness becomes apparent, but these delays are not always obvious in young children.
  
   
  ⚫
Many [[child|children]] are already somewhat delayed when the disorder becomes apparent, but these delays are not always obvious in young children. 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.
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The age at which this regression can occur is defined variously, and can be from age 2-10 with the definition of this onset depending largely on the opinion.
 
   
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==Signs and symptoms==
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Regression can be very sudden, and the child may even voice concern about what is happening, much to the parent's surprise. Some children describe or appear to be reacting to [[hallucination]]s, 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 PDD categories, there is considerable controversy around the right treatment for CDD.
  
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A child affected with childhood disintegrative disorder shows normal development and he/she acquires "normal development of age-appropriate verbal and nonverbal communication, social relationships, motor, play and self-care skills" comparable to other children of the same age. However, from between the age of 2 through the age of 10, skills acquired are lost almost completely in at least two of the following six functional areas:
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* Expressive language skills (being able to [[Speech production|produce speech]] and communicate a message)
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* Receptive language skills (comprehension of language - [[listening]] and [[Speech perception|understanding what is communicated]])
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* [[Social skills]] & [[self care]] skills
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* Control over [[bowel]] and [[urinary bladder|bladder]]
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* Play skills
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* [[Motor skill]]s
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Lack of normal function or impairment also occurs in at least two of the following three areas:
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* [[social relation|Social interaction]]
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* [[Communication]]
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* Repetitive behavior & interest patterns
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In her book, ''Thinking in Pictures'', [[Temple Grandin]] claims that comparing to "Kanner's classic autism" and to [[Asperger syndrome]], CDD is characterized with more severe [[sensory processing disorder]] but less severe [[cognition|cognitive]] problems. She also claims that comparing to most persons with autism, persons with CDD have more severe [[Speech-language pathology|speech pathology]] and they usually do not respond well to [[stimulants]].
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==Causes==
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All of the causes of childhood disintegrative disorder are still unknown. 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."<ref>[http://www.mayoclinic.com/health/childhood-disintegrative-disorder/DS00801/DSECTION=3 Childhood Disintegrative Disorder - Causes]</ref>
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CDD has also been associated with certain other conditions, particularly the following:
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* '''[[Lipid storage disorder|Lipid storage diseases]]''': In this condition, a toxic buildup of excess fats (lipids) takes place in the brain and nervous system.
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* '''[[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.
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* '''[[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]], [[hamartoma]]s, grow in the brain.
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==Treatment==
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{{unreferenced section|date=February 2013}}
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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.
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*'''Behavior therapy''': [[Applied behavior analysis| Applied Behavior Analysis (ABA)]] main aim is to systematically teach the child to relearn language, self-care and social skills. The treatment programs designed in this respect "use a system of rewards to reinforce desirable behaviors and discourage problem behavior." ABA programs may be designed by a board-certified specialist in behavior analysis called a "BCBA" (Board Certified Behavior Analyst), but ABA is also widely used by a number of other health care personnel from different fields like psychologists, speech therapists, physical therapists and occupational therapists with differing levels of expertise. Parents, teachers and caregivers are instructed to use these behavior therapy methods at all times.
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* '''Environmental Therapy''': Sensory Enrichment Therapy uses enrichment of the sensory experience to improve symptoms in autism, many of which are common to CDD.
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*'''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 [[seizure]]s.
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== References ==
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{{reflist|30em}}
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==Further reading==
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* {{cite book |author=Maurice, Catherine |title=Let me hear your voice: a family's triumph over autism |publisher=Knopf |location=New York |year=1993 |pages= |isbn=0-679-40863-0 |oclc=26633221 }}
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* {{cite journal |author=Westphal A, Schelinski S, Volkmar F, Pelphrey K |title=Revisiting regression in autism: Heller's dementia infantilis. Includes a translation of Über Dementia Infantilis |journal=J Autism Dev Disord |volume=43 |issue=2 |pages=265–71 |year=2013 |month=February |pmid=22677931 |doi=10.1007/s10803-012-1559-z |url=}}
   
 
==External links==
  
==External links==
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{{portal|Pervasive Developmental Disorders}}
 
* [http://www.nlm.nih.gov/medlineplus/ency/article/001535.htm NIH/Medline]
  
* [http://www.nlm.nih.gov/medlineplus/ency/article/001535.htm NIH/Medline]
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* [http://info.med.yale.edu/chldstdy/autism/cdd.html Yale]
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* [http://www.med.yale.edu/chldstdy/autism/cdd.html Yale Child Study Center]
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* [http://www.merck.com/mmhe/sec23/ch286/ch286e.html Merck]
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* [http://www.merck.com/mmhe/sec23/ch288777/ch288777c.html#sec23-ch288777-ch288777c-51 Merck]
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{{med-stub}}
  
   
 
{{Pervasive developmental disorders}}
  
{{Pervasive developmental disorders}}
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{{Autism resources}}
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[[Category:Childhood psychiatric disorders]]
  
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{{DEFAULTSORT:Childhood Disintegrative Disorder}}
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[[Category:Pervasive developmental disorders]]
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[[Category:Neurological disorders in children]]
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[[Category:Psychiatric diagnosis]]
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[[Category:Psychosis]]
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[[Category:Learning disabilities]]
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{{enWP|Childhood disintegrative disorder}}

Latest revision as of 01:53, 18 August 2013

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Childhood disintegrative disorder
Classification and external resources
ICD-10 F84.2-F84.3
ICD-9 299.10-299.11
MedlinePlus 001535

Childhood disintegrative disorder (CDD), also known as Heller's syndrome and disintegrative psychosis, was originally described by Austrian educator Theodor Heller in 1908, 35 years before Leo Kanner and Hans Asperger described autism, but it has not been officially recognised until recently. Heller had previously used the name dementia infantilis for the syndrome.[1] CDD is a rare condition(1.7 cases per 100,000 condition).[2][3] Characterized by late onset (>3 years of age) 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.[4][5]

An apparent period of fairly normal development is often noted before a regression in skills or a series of regressions in skills.[6] The age at which this regression can occur varies,[7] but typically after 3 years of normal development.[8] The regression 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. Some children describe or appear to be reacting to hallucinations, but the most obvious symptom is that skills apparently attained are lost.

Many children are already somewhat delayed when the disorder becomes apparent, but these delays are not always obvious in young children. 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.

Signs and symptoms

A child affected with childhood disintegrative disorder shows normal development and he/she acquires "normal development of age-appropriate verbal and nonverbal communication, social relationships, motor, play and self-care skills" comparable to other children of the same age. However, from between the age of 2 through the age of 10, skills acquired are lost almost completely in at least two of the following six functional areas:

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

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

Causes

All of the causes of childhood disintegrative disorder are still unknown. 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."[9]

CDD has also been associated with certain other conditions, particularly the following:

  • 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.

Treatment

Info icon This section does not cite any references or sources.
Please help improve this section by adding citations to reliable sources. (help, get involved!)
Unverifiable material may be challenged and removed.

This article has been tagged since February 2013.

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.

  • Behavior therapy: Applied Behavior Analysis (ABA) main aim is to systematically teach the child to relearn language, self-care and social skills. The treatment programs designed in this respect "use a system of rewards to reinforce desirable behaviors and discourage problem behavior." ABA programs may be designed by a board-certified specialist in behavior analysis called a "BCBA" (Board Certified Behavior Analyst), but ABA is also widely used by a number of other health care personnel from different fields like psychologists, speech therapists, physical therapists and occupational therapists with differing levels of expertise. Parents, teachers and caregivers are instructed to use these behavior therapy methods at all times.
  • 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. ↑ Mouridsen SE (June 2003). Childhood disintegrative disorder. Brain Dev. 25 (4): 225–8.
  2. ↑ Fombone E (June 2002). Prevalence of childhood disintegrative disorder. Autism 6 (2): 149–57.
  3. ↑ Fombonne E (June 2009). Epidemiology of pervasive developmental disorders. Pediatr. Res. 65 (6): 591–8.
  4. ↑ McPartland J, Volkmar FR (2012). Autism and related disorders. Handb Clin Neurol 106: 407–18.
  5. ↑ Venkat A, Jauch E, Russell WS, Crist CR, Farrell R (August 2012). Care of the patient with an autism spectrum disorder by the general physician. Postgrad Med J 88 (1042): 472–81.
  6. ↑ Rogers SJ (2004). Developmental regression in autism spectrum disorders. Ment Retard Dev Disabil Res Rev 10 (2): 139–43.
  7. ↑ Hendry CN (January 2000). Childhood disintegrative disorder: should it be considered a distinct diagnosis?. Clin Psychol Rev 20 (1): 77–90.
  8. ↑ Malhotra S, Gupta N (December 1999). Childhood disintegrative disorder. J Autism Dev Disord 29 (6): 491–8.
  9. ↑ Childhood Disintegrative Disorder - Causes

Further reading

  • Maurice, Catherine (1993). Let me hear your voice: a family's triumph over autism, New York: Knopf.
  • Westphal A, Schelinski S, Volkmar F, Pelphrey K (February 2013). Revisiting regression in autism: Heller's dementia infantilis. Includes a translation of Über Dementia Infantilis. J Autism Dev Disord 43 (2): 265–71.

External links

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