Childhood disintegrative disorder

Childhood disintegrative disorder
Classification and external resources
ICD-10 F84.2-F84.3
ICD-9 299.10-299.11

Childhood disintegrative disorder (CDD), also known as Heller's syndrome and disintegrative psychosis, is a rare (1.7 cases per 100,000[1]) condition characterized by late onset (>3 years of age) 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, but an apparent period of fairly normal development is often noted before a regression in skills or a series of regressions in skills. Many children are already somewhat delayed when the disorder becomes apparent, but these delays are not always obvious in young children.

The age at which this regression can occur varies, and can be from age 2-10 with the definition of this onset depending largely on opinion.

Regression can be very sudden, and the child may even voice concern about what is happening, much to the parent's surprise.[dubious ] Some children describe or appear to be reacting to hallucinations, but the most obvious symptom is that skills apparently attained are lost. 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.

The syndrome was originally described by Austrian educator Theodor Heller in 1908,[2] 35 years before Leo Kanner and Hans Asperger described autism, but it has not been officially recognised until recently. Heller used the name dementia infantilis for the syndrome.[3]

Contents

Signs and symptoms

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 & 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 & interest patterns

Causes

The exact 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."[4] 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

There is no permanent cure for CDD - loss of language and skills related to social interaction and self-care are rather serious. The affected children face permanent disabilities in certain areas and require long term care. Treatment of CDD involves both behavior therapy and medications.

  • Behavior therapy: Its aim is to teach the child to relearn language, self-care and social skills. The programs designed in this respect "use a system of rewards to reinforce desirable behaviors and discourage problem behavior." The behavior therapy is used by a number of health care personnel from different fields like psychologists, speech therapists, physical therapists and occupational therapists. At the same time, parents, teachers and caregivers also use the behavior therapy. A consistent approach by all concerned results in better treatment.
  • Medications: There are no medications available to treat directly 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

Autism-stacking-cans 2nd edit.jpg Pervasive Developmental Disorders portal

External links


Wikimedia Foundation. 2010.

Look at other dictionaries:

  • childhood disintegrative disorder — noun A rare condition characterized by deterioration of language, social function, and motor skills in a child after normal development for the childs first three years. Syn: Hellers syndrome, disintegrative psychosis …   Wiktionary

  • childhood disintegrative disorder — [DSM IV] pervasive developmental disorder characterized by marked regression in a variety of skills, including language, social skills or adaptive behavior, play, bowel or bladder control, and motor skills, after at least two, but less than ten,… …   Medical dictionary

  • Childhood Autism Rating Scale — (CARS) is a behavior rating scale intended to help diagnose autism. CARS was developed by Eric Schopler, Robert J. Reichier, and Barbara Rochen Renner. CARS was designed to help differentiate children with autism from those with other… …   Wikipedia

  • disintegrative psychosis — 1. see Heller s syndrome. 2. a pervasive developmental disorder, often with features of autism, occurring as a result of a brain disease, such as encephalitis, in childhood …   Medical dictionary

  • Pervasive developmental disorder — [1] PDD redirects here. For other uses, see PDD (disambiguation). Not to be confused with PDD NOS. Pervasive developmental disorder Classification and external resources ICD 10 F …   Wikipedia

  • Nonverbal learning disorder — A nonverbal learning disorder or nonverbal learning disability (NLD or NVLD) is a condition characterized by a significant discrepancy between higher verbal and lower motor, visuo spatial, and social skills on an IQ test.[1] Some proponents of… …   Wikipedia

  • Multiple complex developmental disorder — Not to be confused with Multisystem developmental disorder. Multiple complex developmental disorder is a research category, proposed to involve several neurological and psychological symptoms where at least some symptoms are first noticed during… …   Wikipedia

  • Mental disorder — Classification and external resources Eight women representing prominent mental diagnoses in the 19th century. (Armand Gautier) ICD 10 F …   Wikipedia

  • Developmental disorder — Classification and external resources [[File:image|frameless|upright=1.06|alt=]] ICD 10 F80 F84 ICD 9 299 …   Wikipedia

  • Multisystem developmental disorder — Not to be confused with Multiple complex developmental disorder. Multisystem developmental disorder (MSDD) is a term used by Stanley Greenspan to describe children under age 3 who exhibit signs of impaired communication as in autism, but with… …   Wikipedia

Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”

We are using cookies for the best presentation of our site. Continuing to use this site, you agree with this.