Childhood disintegrative disorder is a condition which displays itself at 3 and 4 years of age on children who have typical
development until age 2. Over several months, a child with this childhood disintegrative disorder will regress in academic,
social, and language functioning from previously typical behavior. Childhood disintegrative disorder is associated with severe
cognitive impairment. Behavioral features of autistic disorder must be present in at least two of the following areas: inability to
interact socially, incapable of communicating needs, prefers solitary play; restricted repetitive and stereotyped features in
behavior. A loss of skills may occur in two of the following areas: expressive or receptive language, social skills and/or
flexible behavior, bowel or bladder control, play, or motor skills.
Disintegrative psychosis; Heller syndrome
Signs of Childhood Disintegrative Disorder
Childhood disintegrative disorder indicators may include two or more of the following signs:
• Significant loss of previously achieved age appropriate developmental skills
• Dramatic reverse in expressive and/or receptive language and communication
• Decline of social skills and/or proper behavior ex. Frequently shows distress
• Change in manner of play no longer natural it now becomes a ritual
• Delays in developmental motor skills ex. (gross, fine and cognitive skills)
• Failure to control bowel and/or bladder movements
Characteristics of childhood disintegrative disorder are as follows:
Limited social interactions for instance lack of mutual emotion, avoids eye contact, unable to develop social relationships with
peers the same age, or inappropriate disturbances.
Poor communication and/or language skills such as fails to use acquired language, failure to initiate or maintain conversation,
involuntary repetition of words or phrases just spoken by others ex. Echolalia.
Changes in pattern of active play likes lining up toys and has no imaginative play, interests include insignificant objects and
predictable schedule when in action.
Social and gross motor traits or mannerisms such as spinning, looking in short glances, looking through fingers, looking
sideways, hand flapping, and tip toe walking.
Causes of Childhood Disintegrative Disorder:
The cause of childhood disintegrative disorder is unknown, but it has been linked to neurological irregularities. Children with
childhood disintegrative disorder show a loss of communication skills, have a relapse in nonverbal behaviors, and significant
loss of formerly acquired skills. The condition is very similar to autism spectrum disorders. Childhood disintegrative disorder
has some similarity to autism, but a visible period of fairly normal development is often observed before a loss in skills or a
sequence of regressions in all developmental skills. Many children are already noticeably delayed when the disorder becomes
apparent, but these delays are not always obvious in very young children.
The age at which this regression can occur varies, and can be from age 2-10 with the description of the onset depending
largely on a diagnosis. Deterioration can be very sudden, and the child may even voice concern about what is occurring,
much to everyone’s amazement. Some children explain or seem to be portraying a daydream, but the most obvious symptom
is that skills that were once gained are then lost. This has been described by clinicians as a devastating condition, affecting
both the family and the child's future.
The exact causes of Childhood disintegrative disorder are still unknown and further research; investigation and study are
required to understand the causes which result in this disorder. Occasionally childhood disintegrative disorder emerges
suddenly within days or weeks while sometimes it develops over a longer period of time. Extensive medical and neurological
investigation in children diagnosed with childhood disintegrative disorder rarely uncovers an underlying medical or neurological
cause. Although the incident of epilepsy is higher in children with childhood disintegrative disorder, experts don't know
whether epilepsy plays a role in causing the disorder.
Childhood disintegrative disorder has also been associated with other conditions, particularly the following:
Lipid storage diseases: Lipid storage diseases, or the lipidoses, are a group of inherited metabolic disorders in which harmful
amounts of fatty materials called lipids accumulate in some of the body’s cells and tissues. People with these disorders either
do not produce enough of one of the enzymes needed to metabolize lipids or they produce enzymes that do not work properly.
Over time, this excessive storage of fats can cause permanent cellular and tissue damage, particularly in the brain, peripheral
nervous system, liver, spleen, and bone marrow.
Subacute sclerosing panencephalitis: is rare chronic, progressive encephalitis that affects primarily children and young
adults, caused by a persistent infection of immune resistant measles virus (which can be a result of a mutation of the virus
itself). 1 in 100,000 people infected with measles are at risk. SSPE is 'incurable' but the condition can be managed by
medication if treatment is started at an early stage.
Tuberous sclerosis or tuberous sclerosis complex (TSC): is a rare, multi-system genetic disease that causes benign tumors
to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin. A combination of symptoms
may include seizures, developmental delay, behavioral problems, skin abnormalities, and lung and kidney disease.
Experts also suspect there may be a genetic basis or that an autoimmune response (the immune response of the body against
substance normally present in the body) may play a role in the development of childhood disintegrative disorder. In an
autoimmune response, your body's immune system attacks itself.
There is no established cure for childhood disintegrative disorder, loss of language; social interaction and self-care are
significantly serious. Treatment for the disorder is basically the same as for autism it involves both behavior therapy and
• Behavior therapy: Its aim is to teach the child to improve language, self-care and social skills. Both behavioral training and
behavioral management use positive reinforcement to improve behavior. They also use social skills training to improve
communication. The specific program should be chosen according to the child's needs. Consistent use of these behavioral
interventions produces the best results. The child's functional abilities, behavior, and daily environment should be thoroughly
assessed before behavioral training and management begins. Parents, other family members, teachers, and caregivers of the
child should all be trained in these techniques.
• Medications: There are no medications available to treat directly the childhood disintegrative disorder. Antipsychotic
medications are used to treat severe behavior problems like aggressive demeanor and repetitive behavior patterns.
Anticonvulsant medications are used to control epileptic seizures.
The outcome for children with childhood disintegrative disorder is usually severe, and more intense than for children with
autism. The loss of language, cognitive, social and self-care skills tends to be significant and permanently disabling. As a
result, children with the disorder often need residential care in a group home or long term care facility during adulthood.
|Childhood Disintegrative Disorder
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