by anasthasia » Mon Jul 20, 2009 9:30 am
Hi!
I've found some information on the Internet, hope it helps.
DSM-IV:
299.10 Childhood Disintegrative Disorder
(A)
Apparently normal development for at least the first 2 years after birth as manifested by the presence of age-appropriate verbal and nonverbal communication, social relationships, play, and adaptive behavior.
(B)
Clinically significant loss of previously acquired skills (before age 10 years) in at least two of the following areas:
1. expressive or receptive language
2. social skills or adaptive behavior
3. bowel or bladder control
4. play
5. motor skills
(C)
Abnormalities of functioning in at least two of the following areas:
1. qualitative impairment in social interaction (e.g., impairment in nonverbal behaviors, failure to develop peer relationships, lack of social or emotional reciprocity)
2. qualitative impairments in communication (e.g., delay or lack of spoken language, inability to initiate or sustain a conversation, stereotyped and repetitive use of language, lack of varied make-believe play)
3. restricted, repetitive, and stereotyped patterns of behavior, interests, and activities, including motor stereotypies and mannerisms
(D)
The disturbance is not better accounted for by another specific Pervasive Developmental Disorder or by Schizophrenia.
Introduction
Background
Childhood disintegrative disorder is a rare disorder, occurring in fewer than 5 in 10,000 children. It generally manifests by the fourth year of life, after a period of at least 2 years of normal development. Childhood disintegrative disorder manifests with a loss of previously acquired language and social skills and results in persistent delay in these areas. For example, a child previously able to speak in 2- or 3-word phrases gradually or abruptly loses the ability to communicate using words or uses only fragments. Social and emotional development also regress, resulting in impaired ability to relate with others. For example, a child previously able to accept reassurance from his or her parent (eg, a hug) loses the ability to be consoled and even may withdraw from human (tactile) contact.
Overall, the social, communicative, and behavioral features of childhood disintegrative disorder resemble those of autistic disorder. Distinct qualitative impairments in social interaction and communication are present. In addition, restricted, repetitive, or stereotyped patterns of behavior, interests, and activities occur. Motor loss of previously acquired skills (eg, child previously toilet trained soils during the day and night, child previously able to pedal a tricycle or draw shapes can no longer do so) is present. Additional symptoms may include the onset of difficulty in the transition of waking from sleep. Social interactions become compromised (eg, aggressiveness, tantrums, withdrawal from peers), as does motor function, resulting in poor coordination and possible awkwardness of gait.
Pathophysiology
No clear-cut pathophysiology is proven to cause this disorder; debate within the developmental disabilities field regarding long-term outcome of children with this disorder is noted. Some researchers hypothesize that predisposing genetic factors combined with environmental stressors (eg, prenatal or postnatal virus exposure, birth trauma) result in brain deposition of amyloid and disruption of synaptic transmissions, possibly involving interleukin-1 or beta-endorphins.
Frequency United States
Frequency is very rare (<5 in 10,000 children). Childhood disintegrative disorder is much less common than autistic disorder.
International
No current studies are large enough to determine international frequency.
Mortality/Morbidity
* No mortality or morbidity is caused directly by childhood disintegrative disorder. Indirectly, an increased risk of mortality and morbidity may be present because of a comorbid medical condition, such as a neurodegenerative disorder. The clinician should be alert to the possibility of Landau-Kleffner syndrome (LKS).
* LKS is a rare condition of unknown etiology that is more common in boys who generally present with more severe language impairment and later than those with childhood disintegrative disorder; LKS has a mean onset of 5.5 years. Determining the presence of this syndrome is important because it is generally associated with seizure disorder and may respond to treatment with anticonvulsants such as valproic acid or steroids, in some cases.
Sex
This disorder is slightly more common in males than in females.
Age
Childhood disintegrative disorder, as defined by the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV), occurs only after a period of at least 2 years of normal development, when the child is younger than 10 years.1 Onset generally occurs in children aged 3-4 years and may be insidious or abrupt.
Clinical History
* Obtain a thorough history.
* The child is developmentally normal prior to the age of onset. This is similar to Landau-Kleffner syndrome (LKS); however, in LKS, the onset tends to be later (eg, age 5.5 y), whereas, in childhood disintegrative disorder, the onset is usually by age 3-4 years.
* Developmental delays in language, social, emotional, cognitive, or motor areas are not apparent to either the parent or pediatrician prior to the onset of the disorder.
* Children diagnosed with childhood disintegrative disorder tend to have more long lasting abnormalities of auditory responsiveness and verbal communication than children with pervasive developmental disorder but not as severe as in LKS.
Physical
* Perform a thorough physical examination.
* Occasionally after diagnosis, mild neurologic abnormalities (eg, mild macrocephaly, microcephaly, motor incoordination) are detected upon neurologic examination.
* Specific physical abnormalities are not diagnostic of this disorder.
Causes
No single causal factor for childhood disintegrative disorder is known. Current research emphasizes that a combination of genetic susceptibility and prenatal (or environmental) stress may explain the finding of higher-than-expected brain deposition of amyloid and disruption of synaptic transmission, possibly involving interleukin-1 or beta-endorphins.
* Environmental risk factors
o Viral exposure (usually intrauterine transmission) - Toxoplasmosis, other infections, rubella, cytomegalovirus infection, and herpes simplex (TORCH)
o Birth trauma
o Toxin exposure
o Prematurity
* Genetic factors
o Possible susceptibility to chromosomal breakage or disruption
o Family history of autism or Asperger disorder
* Associated disorders
o Autoimmune disorders
o Allergy or asthma