Childhood disintegrative disorder
Childhood disintegrative disorder (CDD), also known as Heller's syndrome and disintegrative psychosis, is a rare (1.7 cases per 100,000) condition characterized by late onset (>3 years of age) of development
Child development
Child development stages describe theoretical milestones of child development. Many stage models of development have been proposed, used as working concepts and in some cases asserted as nativist theories....

al delays in language
Language may refer either to the specifically human capacity for acquiring and using complex systems of communication, or to a specific instance of such a system of complex communication...

, social function, and motor skills. Researchers have not been successful in finding a cause for the disorder
Mental illness
A mental disorder or mental illness is a psychological or behavioral pattern generally associated with subjective distress or disability that occurs in an individual, and which is not a part of normal development or culture. Such a disorder may consist of a combination of affective, behavioural,...


CDD has some similarity to autism
Autism is a disorder of neural development characterized by impaired social interaction and communication, and by restricted and repetitive behavior. These signs all begin before a child is three years old. Autism affects information processing in the brain by altering how nerve cells and their...

, 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 child
Biologically, a child is generally a human between the stages of birth and puberty. Some vernacular definitions of a child include the fetus, as being an unborn child. The legal definition of "child" generally refers to a minor, otherwise known as a person younger than the age of majority...

ren 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. Some children describe or appear to be reacting to hallucination
A hallucination, in the broadest sense of the word, is a perception in the absence of a stimulus. In a stricter sense, hallucinations are defined as perceptions in a conscious and awake state in the absence of external stimuli which have qualities of real perception, in that they are vivid,...

s, 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
Pervasive developmental disorder
Pervasive developmental disorders is a diagnostic category refers to a group of disorders characterized by delays or impairments in communication, social behaviors, and cognitive development.Pervasive developmental disorders include Autism, Asperger's syndrome, Rett's syndrome, Childhood...

 categories, there is considerable controversy about the right treatment for CDD.

The syndrome was originally described by Austrian educator Theodor Heller in 1908, 35 years before Leo Kanner
Leo Kanner
Leo Kanner was a Jewish American psychiatrist and physician known for his work related to autism. Kanner's work formed the foundation of child and adolescent psychiatry in the U.S. and worldwide....

 and Hans Asperger
Hans Asperger
Hans Asperger was an Austrian pediatrician, after whom Asperger syndrome was named. He wrote over 300 publications, mostly concerning autism in children.-Biography:...

 described autism
Autism is a disorder of neural development characterized by impaired social interaction and communication, and by restricted and repetitive behavior. These signs all begin before a child is three years old. Autism affects information processing in the brain by altering how nerve cells and their...

, but it has not been officially recognised until recently. Heller used the name dementia infantilis for the syndrome.

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


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." CDD has also been associated with certain other conditions, particularly the following:
  • Lipid storage diseases
    Lipid storage disorder
    Lipid storage disorders are a group of inherited metabolic disorders in which harmful amounts of 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...

    : In this condition, a toxic buildup of excess fats (lipids) takes place in the brain and nervous system.
  • Subacute sclerosing panencephalitis
    Subacute sclerosing panencephalitis
    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 . No cure for SSPE exists, but the condition can be managed by medication if treatment is started at...

    : Chronic infection of the brain by a form of the measles
    Measles, also known as rubeola or morbilli, is an infection of the respiratory system caused by a virus, specifically a paramyxovirus of the genus Morbillivirus. Morbilliviruses, like other paramyxoviruses, are enveloped, single-stranded, negative-sense RNA viruses...

     virus causes subacute sclerosing panencephalitis. This condition leads to brain inflammation and the death of nerve cells.
  • Tuberous sclerosis
    Tuberous sclerosis
    Tuberous sclerosis or tuberous sclerosis complex is a rare multi-system genetic disease that causes non-malignant 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...

    (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.


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
    An antipsychotic is a tranquilizing psychiatric medication primarily used to manage psychosis , particularly in schizophrenia and bipolar disorder. A first generation of antipsychotics, known as typical antipsychotics, was discovered in the 1950s...

     medications are used to treat severe behavior problems like aggressive stance and repetitive behavior patterns. Anticonvulsant
    The anticonvulsants are a diverse group of pharmaceuticals used in the treatment of epileptic seizures. Anticonvulsants are also increasingly being used in the treatment of bipolar disorder, since many seem to act as mood stabilizers, and in the treatment of neuropathic pain. The goal of an...

     medications are used to control seizure
    An epileptic seizure, occasionally referred to as a fit, is defined as a transient symptom of "abnormal excessive or synchronous neuronal activity in the brain". The outward effect can be as dramatic as a wild thrashing movement or as mild as a brief loss of awareness...


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