Heller's syndrome

Heller's syndrome

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  • 1 What is the 'Heller syndrome'?
  • 2 How does Heller syndrome begin to manifest?
  • 3 Clinical setting of 'Heller's syndrome' or 'childhood disintegrative disorder'
  • 4 'Heller's syndrome': diagnosis and treatment
  • 5 Severity levels in 'Heller's syndrome'

What is the 'Heller syndrome'?

The 'Heller's syndrome' or childhood disintegrative disorder, is a disorder that is manifested by the "disintegration" of multiple abilities and intelligences that the child had previously acquired, occurs after the first 2 years of life and always before the 10 years of age, after a period of apparent normalcy in its development.

The 'Heller syndrome' owes its name to Theodore Heller, researcher and educator who studied and described the condition in 1908. However, the disorder was recognized until a few years ago, it is also called disintegrative psychosis. It is not common among the population and the reference of epidemiological studies, it speaks of it being more frequent among boys than among girls.

How do you start manifesting Heller's syndrome?

"He didn't communicate, he didn't listen ... it seemed like he was in another world"... These are some of the statements made by parents of these patients. 'Childhood disintegrative disorder' is expressed when the child has a accentuated 'regression 'in their behavior, it is common for the child to experience changes in mood, be irritable, agitated and tantrums, may have hostile behaviors and even self-harm. He also suffers a lost clinically significant in previously acquired skills at least on two of the following areas:

  • Expressive or receptive language
  • Deterioration in social skills or adaptive behaviors

It can also be affected in other important areas of habitual functioning such as:

  • Bowel or bladder control
  • Leisure activities (game)
  • Motor skills

It also presents abnormalities in two of the following areas:

  1. Qualitative alteration of the social interaction:
  • Inability to develop relationships with peers
  • Absence of emotional or social reciprocity
  • Alteration of nonverbal behaviors
  1. Qualitative alterations of the communication:
  • Inability to start or hold a conversation
  • Delay or absence of spoken language
  • Repetitive and stereotyped language
  • Absence of varied realistic game
  1. Behavior patterns, restrictive, repetitive and stereotyped interests and activities, are included:
  • Mannerisms
  • Motor stereotypes, such as simple ones: idiosyncratic phrases, ecolalia, alignment of toys or change of place of certain objects.

For parents it is usually a great challenge, however, however difficult it is, it is recommended quickly transcend the stage of denial and move on to action and intervention, well In the face of obvious symptoms of a problem in the development of children, timely and adequate care is essential.

You may be interested: Disorder of stereotyped movements in childhood

Clinical setting of 'Heller's syndrome' or 'childhood disintegrative disorder'

In the DSM-IV-TR (Diagnostic and Statistical Manual of Mental Disorders), the following were included in the section of generalized developmental disorders (TGD): autistic disorder, Rett disorder, Asperger disorder, unspecified TGD and precisely at childhood disintegrative disorder. In the DSM-V, it is understood within autism spectrum disorder (ASD), in the neurodevelopmental disorders, once the clinical picture of 'Heller's syndrome' is fully established, it resembles autism precisely.

'Heller's syndrome' may be associated with another neurodevelopmental, cognitive or behavioral disorder. It is worth mentioning that it must be distinguished very well from other conditions for its better management and therapeutic intervention. In the DSM-V, it is noted that:

“For patients with a well-established diagnosis according to the DSM-IV of autistic disorder, Asperger's disease or generalized developmental disorder (TGD) not otherwise specified, the diagnosis of Autism Spectrum Disorder will be applied. Patients with notable social communication deficiencies, but whose symptoms do not meet the criteria for autism spectrum disorder, should be evaluated to diagnose social (pragmatic) communication disorder. ”

The social communication disorder does not usually show restrictive and repetitive patterns in their behavior, interests or activities. As can be distinguished with respect to disorders within the autistic spectrum (ASD).

'Heller's syndrome': diagnosis and treatment

A key difference to establish the diagnosis of 'Heller's syndrome' is often the age at which symptoms begin to manifest, within childhood disintegrative disorder, Syndromic symptoms occur most frequently between 3 and 4 years of age., being able to start from 2 years and until before 10 years after the birth of the child.

Currently, in the DSM-V, autism spectrum disorder (ASD) includes a wide range of phenotypes, considering that the fundamental characteristics of autism are:

  1. Development of social interaction and communication visibly abnormal or deficient.
  2. Very restricted repertoire of activities and interests.

To determine comorbidity with the autistic spectrum and intellectual disability: Social communication ability should be below expected for your overall level of development. The establishment of the diagnosis of 'Heller's syndrome' must remain in the hands of a psychiatrist, neurologist or psychologist with experience in the field: “in the case of any neurological disorder, early attention is essential for the child's health”.

The treatment of 'Heller's syndrome' must be based on an approach multi-disciplinary, which includes neurologist, psychiatrist, psychologist, can also benefit from the rehabilitation and some complementary therapies, for the improvement of some symptoms. It is recommended that the family initiate a psychotherapeutic treatment, behavioral therapy has shown good results in the follow-up of these cases and You can bring coping strategies to situations that occur in the daily live.

Severity levels in 'Heller's syndrome'

The most severe cases can reach mutism and dementia, so it was initially known as childhood dementia. The severity level of the condition must be specified based on:

  1. Deterioration of social communication
  2. Repetitive and restricted behavior patterns

You may be interested: Autism Spectrum Disorder, what it is and how it manifests

Bibliographic references

American Psychiatric Association (2014). DSM-5 Diagnostic Criteria Reference Guide. Washington, D.C; London, England: American Psychiatric Publishing.

Cruz-Hernández, Manuel et al. (2007). Treaty of Pediatrics. Volume II. Barcelona, ​​Spain: Ocean.


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