Childhood Disintegrative Disorder

Have you heard about childhood disintegrative disorder? Today's article will describe it and tell you its causes, as well as inform you about the current treatment options.

Childhood disintegrative disorder is rare; it causes significant loss of previously acquired skills between the ages of two and 10. It’s also known as Heller’s syndrome, dementia infantilis, symbiotic psychosis, and disintegrative psychosis.

It used to be a part of the pervasive developmental disorders, along with autism, Rett’s disorder, and Asperger’s disorder in the DSM-IV-TR. However, it disappeared from the DSM-5, and doctors only diagnosed it if the criteria for autism spectrum disorder are also there as “autism spectrum disorder associated with a known medical condition” (childhood disintegrative disorder).

This article will tell you the symptoms of childhood disintegrative disorder, how doctors diagnose it, what causes it, and the best treatments for these children.

A little bit of history

Around 1905, Sante de Sanctis (1862-1953), an Italian doctor, psychologist, and psychiatrist, described a picture similar to the disintegrative disorder of children. He created the category of early dementia, in which he included various disorders that had mental retardation as a symptom.

Later, in 1908, Theodor Heller, an Austrian educator, described several cases of disintegrative psychosis. This consisted of a condition that began around the age of four, after a period of normal development. Hence, child disintegrative disorder is also known as Heller’s syndrome.

The name “symbiotic psychosis” is due to Margaret Mahler, who emphasized the contribution of constitutional factors to a kind of child psychosis that appeared between the ages of three and six. She referred to it as symbiotic psychosis.

Childhood disintegrative disorder diagnosis

As you read above, the DSM-5 eliminated this disorder from its medical illness list. However, autism spectrum disorder has the specifier “associated with a known medical condition”. This allows the diagnosis of this condition (if a child meets the diagnostic criteria), along with childhood disintegrative disorder.

The DSM-IV-TR did include the diagnostic criteria for this disorder. There’s apparently normal development during the first two years of life. It manifests by the presence of communication, social relationships, play, and adaptive behavior typical of the age.

However, one can appreciate significant losses of skills already acquired from the age of two and before the age of 10 in at least two of the following areas:

• Firstly, expressive and receptive language.
• Social skills or adaptive behavior.
• Bowel or bladder control.
• Play.
• Motor skills.

In addition, there must be alterations in two of the following areas. These coincide with the alterations characteristic of autism: qualitative alteration of social interaction and communication or repetitive and stereotyped patterns of behavior, interests, and activities.

Finally, these symptoms can’t be better explained by the presence of other pervasive developmental disorders or by the presence of schizophrenia in order to make the diagnosis. This is a prerequisite.

Symptoms of childhood disintegrative disorder

• Loss of language-related skills. A child loses the acquired vocabulary and ability to communicate with others, including the receptive ability.
• Problems in social relationships and adaptive behavior. There’s a reduced interaction with peers and family that drives children to isolation. This is due to a complete lack of interest in the environment.
• Loss of motor skills. Children begin to experience difficulties in gross motor skills such as running (or walking, in more severe cases). This usually manifests along with clear difficulties in fine motor skills (grasping objects with the hand).
• An inability to control bowel and bladder. Children usually acquire bowel and bladder control between the ages of two and four. The ones with childhood disintegrative disorder usually lose this ability.
• Qualitative alteration of social interaction that can manifest itself with deficits in non-verbal communication, inability to establish social relationships, or lack of social or emotional reciprocity, etc.
• Stereotypical behavior and restricted interests, such as inflexible adherence to certain routines, intolerance to change, motor mannerisms and stereotypes, and foreign interests (such as a taste for the edges of objects, rather than an interest in the object itself).

Cause and treatment of childhood disintegrative disorder

The etiology, meaning the origin, of childhood disintegrative disorder isn’t completely clear. Concrete mechanisms are yet to be found. However, there are some possible causes for the appearance of this disorder, such as injuries to the central nervous system during development and the appearance of some neurological diseases, such as tuberous sclerosis. However, there’s no evidence of this at the moment.

As mentioned above, childhood disintegrative disorder is a rare disorder (more frequent in males) and there’s no cure for it. Therefore, what doctors can do is offer certain treatments that allow for the improvement of the health and quality of life of these children, and to promote the skills they retain.

Interventions

They’re usually multidisciplinary:

• Pharmacotherapy can help reduce stereotypical behaviors (self-inflicted in many cases) and the symptoms of other comorbid disorders.
• Nutritional therapy guarantees the supply of nutrients and this is important because these children often have problems chewing and swallowing food.
• Behavioral therapy helps reduce unwanted behaviors, such as stereotyping, and helps improve any retained skills. In fact, a child can redevelop a lost skill in some cases.
• Alternative therapies usually accompany medical and behavioral treatment. Music and equine therapy are often used and have proven benefits. Not only for children with childhood disintegrative disorder but also for those with other neurodevelopmental disorders.

By definition, these symptoms appear between the ages of two and 10. They can appear abruptly or insidiously and may be accompanied by prodromal symptoms such as irritability, hyperactivity, anxiety, or small losses in some skills.

Once there’s an established upheaval, they can make some small improvements, but the social, communicative, and behavioral deficiencies are constant and stable throughout life. Thus, any treatment that offers improvement, however small, in the symptoms and quality of life of these children is welcome.