What is Childhood Disintegrative Disorder (Heller’s Syndrome)?

What is Childhood Disintegrative Disorder (Hellers Syndrome)With the release of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders Edition V (DSM-V) in 2013, many developmental and behavioral disorders were reclassified or included in the new diagnosis of autism spectrum disorders (ASD). Childhood disintegrative disorder (CDD), though it was discovered and diagnosed long before autism, is now classified as one of the low-functioning subsets of ASD. Though it is on the ASD spectrum, CDD has its own specific nuances, characterizations and ramifications that make it important to discuss and understand. 

What Is Childhood Disintegrative Disorder?

Childhood disintegrative disorder, also known as Heller’s syndrome or disintegrative psychosis, follows a different pattern of onset from ASD.  It is no less detrimental to the psychological and social well-being of the children who suffer from it. Many would claim that it is more detrimental. In the article below, we’ll discuss the finer points of CDD disorder, including its history, current understandings, and related therapies, to provide a deeper understanding of what it is and how it is treated.

See Also: Who Was the First Person to be Diagnosed With Autism?

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An Early Discovery

Doctor Theodore Heller first discovered CDD in 1908, many years before the first diagnosis of autism. This is why it may also be referred to as Heller syndrome. It is a rare condition, characterized by its late onset (typically > 3 years old), that causes developmental delays in:

  • motor skills
  • play skills
  • social function
  • language
  • social skills
  • bowel and bladder control

Currently, its cause (etiology) is also unknown. 

Childhood disintegrative disorder is so named because those afflicted develop normally for the first several years of their life. However, around age three or four, they suddenly lose their acquired skills – essentially, their development ‘disintegrates.’ While the speed with which this disintegration can occur varies, it often occurs quickly (and for many parents, horrifyingly). Most often, the loss of skills takes place over a six-to-nine-month span. Dr. Heller codified this regression disorder after treating six children.  When he originally noticed the extreme developmental regression, he noted ‘dementia infantilis’ to describe the loss of basic skills that was observed.

A Diagnostic Evolution

In 1994, CDD (which is what dementia infantilis has come to be known as) was officially recognized and added to the DSM-IV. It is categorized as a part of the current autism spectrum because it shares such a similar profile of debilitating developmental effects. In an unofficial capacity, physicians and psychiatrists both have recognized the symptom profile for some time. Over the last several decades, it was called both disintegrative psychosis of childhood (DPC) or childhood-onset pervasive developmental disorder (COPDD). Before the clarification of CDD in the DSM-IV diagnosticians and care providers simply labeled it as late childhood onset autism.

As a rule, many ASDs present with delayed or absent developmental features (particularly at the age they should commonly be developed). This includes:

  • social skills
  • receptive language skills
  • play with peers
  • motor skills
  • bowel or bladder control issues

CDD and its symptoms fit within these classifications, so it remains a subset of ASD.

Signs and Symptoms of CDD

Because children with this disorder tend to develop at a normal rate for the first three to four years of life, physicians (and parents) must know the signs of its onset. The first indication many doctors have is when a parent brings their child to them for observation, specifically related to new onset of behavior or communication issues.

In about 75% of CDD cases, the loss of skills or regressive behaviors is preceded by intense anxiety and/or terror in the child. This may present as nightmares or waking nightmares, or confusion/patterns of jumpy disturbance. This type of preceding behavior or occurrences are known as a prodrome (this is not required for CDD diagnosis). 

Diagnostic Criteria

True diagnostic criteria for CDD require that a child exhibit abnormal or regressive behavior in at least two major areas (with normal development for at least the first two years after birth), such as:

  • Ability to maintain or initiate conversations with others (receptive and expressive language skills)
  • Ability to develop peer relations and demonstrate social and emotional reciprocity 
  • Natural motor skills and behaviors consistent with healthy physical/spatial awareness
  • Bowel or bladder control (if already present)
  • Restricted, repetitive or stereotyped behaviors (like head bobbing or hand flapping) unrelated to other conditions

Any regression in these areas should be followed up with a physician.  A doctor can perform tests to rule out other possible conditions like a head injury or brain tumor.

What Does Childhood Integrative Disorder Look Like?

How might this look to a parent or care provider? Children with CDD will often be incapable of extended dialogue with others and may exhibit a marked reticence to initiate communication, even in comfortable surroundings. They may also:

  • exhibit regression in social skills already acquired
  • no longer able to make or keep friends
  • respond in appropriate ways to the emotional needs of those friends

They may also respond inappropriately (or not respond) in social situations.  They may be lacking the ability to do things like say hello or goodbye or answer questions directed at them. Lastly, a parent may notice the onset of new repetitive movements or urinary or stooling accidents when there were none.

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Related Resource: Top 20 Best Applied Behavior Analysis Programs

Therapy for Childhood Disintegrative Disorder

Treatment for CDD is going to be similar to treatment for autism. Like ASD treatments, the emphasis is usually on early intervention, largely behavioral-based, and based on the child’s specific needs. Possible therapies for CDD include:

  • speech and language therapy
  • occupational therapy
  • sensory integration therapy
  • social skills development

Much like ASD, there are no pharmacological therapies specific for CDD.  Depending on a child’s symptoms, some antipsychotics or selective serotonin reuptake inhibitors (SSRIs) may help treat some of the behavioral symptoms. 

Parent involvement and education can prove integral to any form of treatment success. Though therapies with licensed care providers are necessary, the child will spend much more time with their primary caregivers. To best enhance what is being taught and learned in therapy, a child must also be encouraged and instructed in the home.

Applied Behavior Analysis

Applied Behavior Analysis may be an effective treatment option for individuals with CDD.  This type of research-based treatment is effective in increasing positive or desirable behaviors while decreasing behaviors that are counterproductive.  A Board Certified Behavior Analyst can work with the child to develop an individualized plan that addresses their unique needs.

What is the Prognosis for Children with CDD?

Unfortunately, CDD has no known cure.  Children with CDD typically require lifelong support.  Children have severe impairments related to intellectual functioning and adaptive skills.  Some children lose their ability to communicate.

It is interesting to note that epilepsy occurs at a much higher rate in children with CDD compared to in those with autism.  Epilepsy is a neurological condition with a variety of causes.  The risk of seizures increases throughout childhood but stabilizes during adolescence.  SSRIs and neuroleptics can help reduce the frequency and severity of seizure activity.

The life expectancy of individuals with CDD is reported as normal.  Epilepsy can cause a heightened mortality risk.  It is important that individuals with CDD and epilepsy receive appropriate medical care to monitor their condition.

Building a Support System

Families with a loved one diagnosed with CDD have supports available to help them navigate the challenges they face.  Support groups and organizations like Autism Speaks can provide valuable information and advice for parents and support persons.  It is important to have support since caring for an individual with CDD is a lifelong commitment.

Autism Speaks is a great advocacy organization founded by the grandparents of a child with autism.  They conduct research into areas like the causes and prevention of autism.  Their website features advocacy toolkit with information about ways to advocate at school and in the community.

The medical community continues to work to broaden its understanding of autistic disorders to render appropriate and helpful therapies and aids to those who suffer from them. Greater understanding of the physiological underpinnings and genetic components of such developmental disorders continue to help establish effective treatments or preventative therapy(ies). While CDD is a recognizably different type of autism spectrum disorder, it continues to impact the lives of children, parents, and caregivers in similar ways and continues to be treated comparably.

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Updated October 2020

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