HELLER Syndrome: Symptoms, Characteristics, Causes and Treatment

Heller syndrome, also known as childhood disintegrative disorder, is a rare generalized developmental disorder that involves an alteration of the ability to develop language, social skills, and motor skills. Unfortunately, to this day the causes that cause it are unknown.

In this Psychology-Online article we explain what is Heller syndrome, what are its symptoms and its main characteristics, the possible causes that cause it, as well as the treatment indicated for this serious disorder.

Index

1. Heller syndrome symptoms
2. Heller syndrome characteristics
3. Heller syndrome causes
4. Treatment for Heller syndrome

Children affected by Heller syndrome or infantile disintegrative disorder show a clinically significant loss of previously acquired skills in at least two of the following fields:

• Expressive language skills
• Receptive language skills
• Social and self-care skills
• Bowel and bladder control
• Game skills
• Motor skills

This disorder can also affect other relevant areas in the child's behavior:

• Social interaction
• Communication
• Restricted interests
• Repetitive behaviors

The child presents these difficulties after at least two years of apparently normal development. This usually occurs when they are between 3 and 4 years old, but usually before their 10th birthday.

The onset of Heller syndrome can be abrupt or gradual. This disorder is usually serious enough that children be aware of the regression in his capacities, which leads them to ask what is happening to them. In general, parents and professionals do not previously notice abnormalities in aspects such as language or non-communication. verbal, social relationships, play or abnormal development, hence the diagnosis many times results traumatic.

A typical presentation of the Heller syndrome would be that of a child who is able to communicate in sentences of two or three words and lose this ability. Eventually he would either stop speaking entirely or retain only fragments of his previous speech. There may be social and emotional problems, such as a child who previously showed joy from being hugged and suddenly becomes averse to physical contact. In addition, some children also describe what could be hallucinations.

Difference between Heller syndrome and ASD

Heller syndrome has been compared, on occasion, with some variants of the autism spectrum disorder. In both disorders the child shows social and communication deficits Similar; however, Heller syndrome is distinguished from autism in that in the latter there is no rapid regression after a period of apparent normality in psychosocial development. It should also be noted that children with Heller syndrome are more likely to show fear and stereotypical behaviors early.

Children suffering from this serious syndrome suffer from epilepsy more often than children with autism spectrum disorder. In the following article you will find more information about the epilepsy.

the degree of intellectual disability appears to be more "uniform" compared to autism, even though the overall degree of disability and its outcome appear to be similar in both groups.

Initially, the Heller syndrome it was strictly considered a medical disorder and was believed to have identifiable medical causes. However, after the scientific community reviewed various reported cases of the syndrome, no specific medical or neurological cause was found that could explain the syndrome in full.

What causes Heller syndrome is currently unknown. Research results suggest that it could be the result of neurobiological alterations in the child's brain. About half of the children diagnosed with this syndrome have a abnormal electroencephalogram (EEG) (EEGs measure the electrical activity of the brain generated by nerve transmission).

Heller syndrome has also been occasionally associated with seizure occurrence, another indicator that there could be a brain neurobiological cause involved in the onset of this disorder. On the other hand, some researchers consider that the syndrome is actually a type of childhood dementia, suggesting that brain amyloid deposits could be the cause of the disorder, as occurs in the Alzheimer disease. However, this last hypothesis has not been proven and the investigations are ongoing.

The current treatment for Heller syndrome is very similar to the treatment indicated for children with autism spectrum disorder. The key is to address this disorder through early and intensive educational interventions, since although the disorder as such has no cure, it is possible stimulate skills and the child's own capacities, taking into account the limitations and specific needs of each one.

Most of the treatment is based on behavior modification techniques that are carried out in highly structured environments. One of the best known and most widely used therapies is applied behavior analysis or ABA. This methodology includes learning principles to modify children's behavior. Along with behavioral therapy, behavioral therapy is also usually applied. sensory enrichment and drug therapy (mainly antipsychotics and serotonin reuptake inhibitors).

Finally, it should be noted the importance of involve parents throughout the therapeutic process, so that they can also consult any doubts and problems that are encountered. Sometimes families can feel overwhelmed without knowing how to react to certain situations or to some disruptive behaviors of the child, hence it is It is essential to take into account the indications of health professionals, so that what the child learns in therapy can later be transferred to other areas (school or home).

This article is merely informative, in Psychology-Online we do not have the power to make a diagnosis or recommend a treatment. We invite you to go to a psychologist to treat your particular case.

If you want to read more articles similar to Heller syndrome: symptoms, characteristics, causes and treatment, we recommend that you enter our category of Neurological disorders.

• Bray, M. A., Kehle, T. J., Theodore, L. A., & Broudy, M. S. (2002). Case study of childhood disintegrative disorder — Heller's syndrome. Psychology in the Schools, 39(1), 101-109.
• Hendry, C. N. (2000). Childhood disintegrative disorder: should it be considered a distinct diagnosis?. Clinical Psychology Review, 20(1), 77-90.