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, 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.[1][2] In May 2013, the term CDD, along with other types of autism, was fused into a single diagnostic term called "autism spectrum disorder" under the new DSM-5 manual.[3] Therefore, CDD is now also called "regressive autism", being that this term can now refer to any type of autism spectrum disorder that involves regression, including CDD.[4]
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.[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.
Contents
1Signs and symptoms
2Causes
3Treatment
4References
5Further reading
6External links
Signs and symptoms
CDD is a rare condition, with only 1.7 cases per 100,000.[9][10][11]
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"[citation needed] comparable to other children of the same age. However, between the ages of 2 and 10, skills acquired are lost almost completely in at least two of the following six functional areas:
Expressive language skills (being able to produce speech and communicate a message)
Receptive language skills (comprehension of language - listening and understanding what is communicated)
Social skills and self care skills
Control over bowel and bladder
Play skills
Motor skills
Lack of normal function or impairment also occurs in at least two of the following three areas:
Social interaction
Communication
Repetitive behavior and interest patterns
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. She also argues that compared to most individuals suffering from 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."[12]
CDD, especially in cases of later age of onset, has also been associated with certain other conditions, particularly the following:[8]
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 disintegrate.
Treatment
This section does not cite any sources. Please help improve this section by adding citations to reliable sources. Unsourced material may be challenged and removed.(February 2013) (Learn how and when to remove this template message)
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: The main aim of Applied Behavior Analysis (ABA) 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
^McPartland J, Volkmar FR (2012). Autism and related disorders. Handb Clin Neurol. Handbook of Clinical Neurology. 106. pp. 407–18. doi:10.1016/B978-0-444-52002-9.00023-1. ISBN 9780444520029. PMC 3848246. PMID 22608634.
^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.
^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.
^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.
^ abMalhotra S, Gupta N (December 1999). "Childhood disintegrative disorder". J Autism Dev Disord. 29 (6): 491–8. doi:10.1023/A:1022247903401. PMID 10638461.
^Fombone E (June 2002). "Prevalence of childhood disintegrative disorder". Autism. 6 (2): 149–57. doi:10.1177/1362361302006002002. PMID 12083281.
^Fombonne E (June 2009). "Epidemiology of pervasive developmental disorders". Pediatr. Res. 65 (6): 591–8. doi:10.1203/PDR.0b013e31819e7203. PMID 19218885.
^Fombonne, Eric (2002). "Prevalence of Childhood Disintegrative Disorder". Autism. 6 (2): 149–157. doi:10.1177/1362361302006002002. PMID 12083281.
^Childhood Disintegrative Disorder - Causes Archived September 29, 2007, at the Wayback Machine
Further reading
Maurice, Catherine (1993). Let me hear your voice: a family's triumph over autism. New York: Knopf. ISBN 978-0-679-40863-5. OCLC 26633221.
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. doi:10.1007/s10803-012-1559-z. PMID 22677931.
Dobbs, David (2016). "The most terrifying childhood condition you've never heard of." Spectrum 6 July 2016, accessed 6 May 2017 at https://en.wikipedia.org/wiki/Childhood_disintegrative_disorder?action=edit&oldid=778841596&wteswitched=1
External links
Classification
D
ICD-10: F84.2–F84.3
ICD-9-CM: 299.10–299.11
DiseasesDB: 33210
External resources
MedlinePlus: 001535
eMedicine: article/916515
Patient UK:
Childhood disintegrative disorder
Orphanet: 168782
NIH/Medline
v
t
e
Pervasive developmental disorders and autism spectrum (F84, 299)
Main
Causes
Comorbid conditions
Epidemiology
Heritability
Societal and cultural aspects
Medical model
Therapies
Diagnoses
Autism spectrum (High-functioning autism
Classic Autism
Asperger syndrome
Pervasive developmental disorder not otherwise specified
… and most prominent features of Alzheimer disease (AD) dementia and most other forms of dementia. A prominent exception is frontotemporal dementia (FTD), in which memory may be relatively preserved in relation …
… pathologic aspects of specific dementia syndromes and the treatment, risk factors, and prevention of dementia are discussed separately: Most dementia is caused by a neurodegenerative …
…clinical progression of dementia . Scores range from 1 to 7, with higher scores indicating greater dementia severity. Stage 7 of the GDS provides a useful description of advanced dementia: profound memory deficits …
…disorders in patients with dementia. The evaluation and treatment of other neuropsychiatric symptoms of dementia are reviewed separately. Both the normal aging process and dementia can be associated with …
… cerebrovascular disease is sufficient to cause vascular dementia. It is important to recognize that vascular dementia is a syndrome, not a disease. Vascular dementia may be caused by any cerebrovascular or cardiovascular …
English Journal
Case report: an unexpected link between partial deletion of the SHANK3 gene and Heller's dementia infantilis, a rare subtype of autism spectrum disorder.
Philippe A, Craus Y, Rio M, Bahi-Buisson N, Boddaert N, Malan V, Bonnefont JP, Robel L.
BMC psychiatry. 2015 Oct;15()256.
Deletions and mutations involving the SHANK3 gene lead to a nonspecific clinical presentation with moderate to profound intellectual disability, severely delayed or absent speech, and autism spectrum disorders (ASD). Better knowledge of the clinical spectrum of SHANK3 haploinsufficiency is useful to
Revisiting regression in autism: Heller's dementia infantilis. Includes a translation of Über Dementia Infantilis.
Westphal A, Schelinski S, Volkmar F, Pelphrey K.
Journal of autism and developmental disorders. 2013 Feb;43(2)265-71.
Theodor Heller first described a severe regression of adaptive function in normally developing children, something he termed dementia infantilis, over one 100 years ago. Dementia infantilis is most closely related to the modern diagnosis, childhood disintegrative disorder. We translate Heller's pape
In 1908 a Viennese remedial educator Theodor Heller described six children under the name of dementia infantilis who had insidiously developed a severe mental regression between the 3rd and 4th years of life after normal mental development. Neuropathological and other medical conditions are sometime
Dementia infantilis explanation free. What is Dementia infantilis? Meaning of Dementia infantilis medical term. What does Dementia infantilis mean? Dementia infantilis | definition of Dementia infantilis by Medical dictionary ? ...
Abstract Theodor Heller first described a severe regression of adaptive function in normally developing children, something he termed dementia infantilis, over one 100 years ago. Dementia infantilis is most closely related to the modern ...
A condition occurring in 3- and 4-year-old children that is characterized by unequivocally normal development in the first several years of life, followed by a marked developmental regression (a child who previously had been speaking in ...