childhood disintegrative disorder

小児崩壊性障害

WordNet

1. bring disorder to (同)disarray
2. a physical condition in which there is a disturbance of normal functioning; "the doctor prescribed some medicine for the disorder"; "everyone gets stomach upsets from time to time" (同)upset
3. a disturbance of the peace or of public order
4. the state of a child between infancy and adolescence (同)puerility
5. the time of persons life when they are a child
6. tending to cause breakup into constituent elements or parts
7. not arranged in order (同)unordered

PrepTutorEJDIC

1. 〈U〉『無秩序』,混乱,乱雑(confusion) / 《しばしば複数形で》(社会的・政治的な)粉争,騒動 / 〈C〉(肉体的・精神的な)不調,異常,障害 / …‘の'秩序を乱す / 〈心身〉‘に'異常を起こさせる
2. 『幼時』,子供時代

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/07/05 15:40:21」(JST)

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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

The childhood disintegrative disorder (CDD), 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.

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.

An apparent period of fairly normal development is often noted before a regression in skills or a series of regressions in skills. The age at which this regression can occur varies, but typically after 3 years of normal development. 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 her/him. 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

CDD is a rare condition, with only 1.7 cases per 100,000.

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:

• 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 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."

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

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

Further reading

• Maurice, Catherine (1993). Let me hear your voice: a family's triumph over autism. New York: Knopf. ISBN 0-679-40863-0. 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. 

External links

• NIH/Medline
• Yale Child Study Center
• Merck

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. 自閉症スペクトラム障害：用語、疫学および病因 autism spectrum disorder terminology epidemiology and pathogenesis
• 2. 自閉症スペクトラム障害：診断 autism spectrum disorder diagnosis
• 3. 自閉症スペクトラム障害：臨床的特徴 autism spectrum disorder clinical features
• 4. 自閉症スペクトラム障害：プライマリケアにおけるサーベイランスおよびスクリーニング autism spectrum disorder surveillance and screening in primary care
• 5. 小児および思春期における自閉症スペクトラム障害：行動介入および教育的介入 autism spectrum disorder in children and adolescents behavioral and educational interventions

English Journal

• Autism Spectrum Disorder: Primary Care Principles.

• Sanchack KE1, Thomas CA2.
• American family physician.Am Fam Physician.2016 Dec 15;94(12):972-979.
• Autism spectrum disorder is characterized by difficulty with social communication and restricted, repetitive patterns of behavior, interest, or activities. The Diagnostic and Statistical Manual of Mental Disorders, 5th ed., created an umbrella diagnosis that includes several previously separate cond
• PMID 28075089

• Trauma and Violence in Autism.

• Westphal A1.
• The journal of the American Academy of Psychiatry and the Law.J Am Acad Psychiatry Law.2016 Jun;44(2):198-9.
• Comorbidities of autism spectrum disorder are discussed as an introduction to the argument that, although ASD may modify presentation, it does not confer any protection against other disorder, including the negative effects of trauma (e.g., posttraumatic stress disorder). Dr. Im's hypotheses are dis
• PMID 27236175

• CSF N-glycan profile reveals sialylation deficiency in a patient with GM2 gangliosidosis presenting as childhood disintegrative disorder.

• Barone R1, Sturiale L2, Fiumara A3, Palmigiano A2, Bua RO2, Rizzo R1, Zappia M4, Garozzo D2.
• Autism research : official journal of the International Society for Autism Research.Autism Res.2016 Apr;9(4):423-8. doi: 10.1002/aur.1541. Epub 2015 Aug 19.
• Protein N-glycosylation consists in the synthesis and processing of the oligosaccharide moiety (N-glycan) linked to a protein and it serves several functions for the proper central nervous system (CNS) development and function. Previous experimental and clinical studies have shown the importance of
• PMID 26286102

Japanese Journal

• Aripiprazoleが有効であった小児期崩壊性障害(ICD-10)の1症例

• 精神医学 57(3), 211-218, 2015-03
• NAID 40020400857

• 思春期に重篤な高次脳機能障害を呈したてんかん例

• てんかん研究 26(1), 26-32, 2008-06-30
• NAID 110006827306

• Childhood disintegrative disorder

• Brain & development 25(4), 225-228, 2003-06-01
• NAID 10011591312

Related Links

• Childhood disintegrative disorder - Mayo Clinic

Childhood disintegrative disorder — Comprehensive overview covers symptoms, diagnosis and treatment of this rare developmental disorder. ... Childhood disintegrative disorder is also known as Heller's syndrome. It's a very rare ...

• Childhood disintegrative disorder: MedlinePlus Medical ...

Childhood disintegrative disorder is a condition in which children develop normally through age 3 or 4. Then, over a few months, they lose language, motor, social, and other skills that they already learned. Childhood ...

Related Pictures

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★リンクテーブル★

リンク元	「小児崩壊性障害」
関連記事	「disorder」「childhood」

「小児崩壊性障害」

　　[★]

英

childhood disintegrative disorder

関

発達障害

「disorder」

　　[★]

• 障害：個人的苦痛や機能の障害があるので「疾病」とは言えるものの、その背景にある臓器障害がもう一つはっきりしない場合に用いられる。(PSY.9)

• n.

• an untidy state; a lack of order or organization (⇔order)
• violent behaviour of large groups of people
• an illness that cause a part of the body to stop functioning correctly

• 注意

disease <> illness <> disorder

• vt.

• 乱す、乱雑にする。(人)の(心身の)調子を狂わせる。

• vi.

　 　 　 　

「childhood」

　　[★]

• n.

• 幼児期、小児期

関

infant、preschool、preschool period