Childhood Disintegrative Disorder
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.

Alternative Names:

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 a 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.

Treatment

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 medications.

•        
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.
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