A Guide to Childhood Disintegrative Disorder

Elemy
Updated: January 4, 2022

Childhood disintegrative disorder (CDD) is a rare developmental condition that involves a regression of skills a child has already learned. It impacts only one or two children in every 100,000.

CDD can occur at any point from age 2 to 10. It causes children to lose social, language, and motor skills they had already acquired. Children develop typically up until an average age of 3 before they are diagnosed. CDD is classified as a part of autism spectrum disorder (ASD).

CDD has many differentiating factors from ASD, however. It is vital to recognize the signs to obtain a specific diagnosis for optimal treatment. CDD symptoms can be more significant and occur later in childhood than autism typically develops.
Treatment methods for CDD are particular to each individual and often include behavioral and environmental therapies and medications. Early intervention is key, and children often need long-term care and support.

Childhood Disintegrative Disorder Explained

Childhood disintegrative disorder is uncommon and falls under the same diagnostic category as autism spectrum disorder, although it once held its own diagnosis. CDD is also called Heller’s syndrome for the special educator who was the first to describe it in the early 1900s.

It is considered a late-onset form of autism that can come on very quickly in early childhood. The average age of onset is 3 years old; however, children as young as 2 and as old as 10 years of age have developed CDD. Children often develop typically up until this point and then begin to lose skills they previously learned.

Children often are aware that they are losing skills they have already learned and can become fearful and appear to be responding to hallucinations. They may also ask what is happening to them, hold their heads, and suffer from nightmares and bouts of terror. The regression of social, motor, and language skills is often rapid with CDD.

Cause & Symptoms of CDD

There is no known cause of CDD, but around half of the children with this diagnosis also show atypical brain waves indicating it could be related to the neurobiology of the brain. Seizures are also common with CDD, which can make it more likely that brain waves and electrical impulses are impacted.
Again, children with CDD reach typical developmental milestones all the way up until at least 2 years old. At this point, they start to lose skills and often do so rapidly.

Children with CDD can lose their acquired skills entirely in at least two of the following six areas of functioning:

Motor skills
Play skills
Self-care and social skills
Receptive language skills (comprehending, understanding, and listening to what is being communicated)
Bladder and bowel control
Expressive language skills (being able to produce speech and communicate effectively)

Repetitive behaviors, actions, and interests are also common indicators of childhood disintegrative disorder. Children can also become averse to physical touch, even if it was something they enjoyed previously. A child who has been speaking at least two- or three-word phrases can also stop communicating or attempting to talk altogether.

Childhood Disintegrative Disorder & Autism

Childhood disintegrative disorder is under the same umbrella as autism and is considered part of the autism spectrum. As with autism, children with CDD experience regression of skills, a loss of interest in social interactions, and engage in repetitive behaviors.

With autism, this regression usually begins around age 2. With CDD, the regression is later in childhood, with an average age of just over 3.

Symptoms are often more severe with CDD versus autism. Regression can be faster, later in childhood, and include more typical development prior to the regression. A child can have more affective symptoms, such as anxiety, depression, and behavior issues.

People with CDD can regularly have high intellectual capacities and an ability to regain social skills in comparison with autism. However, other facets of functioning and daily life can be more impaired.

Epilepsy and seizures are more common with CDD than autism, as is awareness of losing skills. Children with CDD are more likely to need lifelong support as well and can benefit from early diagnosis and intervention to help manage symptoms.

Diagnosing CDD

Diagnosis of CDD is often done in an exclusionary manner. This means ruling out other medical and neurological causes of sudden and abrupt skill regression before making a diagnosis. Parents should keep detailed records of their child’s behaviors and developmental milestones to help reach an accurate diagnosis of CDD.

A CDD diagnosis requires that your child has developed typically for at least the first 2 years, then experienced regression of skills they had already learned. While this regression is often rapid, it can also be more gradual.
Tests and exams are essential to rule out other potential causes of this regression. These can include the following:

CT or MRI scan
Liver function test
Thyroid function test
Testing for HIV
EEG (electroencephalogram)
Urine testing for aminoaciduria
Check for glucose and urea levels
Complete blood count (CBC)

An early diagnosis can help manage the symptoms and develop a treatment plan that can support the child and parents. Talk to your child’s doctor about any changes in their development as soon as you notice them for early intervention efforts.

Treating CDD in Children

Each treatment for CDD is specific to the child as symptoms can vary in range and severity. Children with CDD will often need long-term support and care as communication, social, and motor skills are often significantly impaired. With a structured care plan, certain skills and abilities can be successfully retained.

Children with CDD will often require a mixture of medications, behavioral, and environmental therapies. Medications are specific to symptoms that arise with CDD. These can include the following:

Anticonvulsants for seizures
Antipsychotics for repetitive patterns and aggressive behaviors
Selective serotonin reuptake inhibitors (SSRIs) to help manage problematic behaviors

If medical conditions co-occur with CDD, medications can be beneficial to treat these underlying conditions as well.

Behavioral therapies often center around applied behavior analysis (ABA), which helps to reinforce desired actions and behaviors. Maladaptive behaviors are discouraged through ABA using a reward system. With ABA and behavioral interventions, children can relearn skills and behaviors that were lost through regression.

The following additional services can be beneficial:

Speech therapy
Occupational therapy
Physical therapy
Social skills training
Sensory integration and enrichment therapies

Assistive technology can aid in enhancing communication and language skills, which can be improved with the use of specialized programs and devices. It is helpful for parents, caregivers, and families to receive education on how best to support a child with CDD so the entire care team can work together.

Supporting Adults With CDD

A structured treatment plan that begins early can help people with CDD learn how to manage symptoms and relearn skills that were lost during a period of regression.
Adults with CDD often have additional medical issues and require supportive care as the disorder can impact daily life. With a comprehensive care team, however, skills can be retained and tools learned to help with communication and self-care.

Treatment for CDD can often be similar to that of autism treatment modalities, including the following supportive services:

Occupational therapy
Social skills trainings
Physical therapy
Sensory integration therapies
Behavioral interventions
Speech and language therapies

Support groups can help adults with CDD learn effective coping mechanisms and healthy peer interactions.

Cognitive behavioral therapy (CBT) is a behavioral intervention that can help adults with autism and CDD learn how to regulate emotions, manage stress, and develop tools for coping with daily life. Individuals can learn how emotions and thoughts can impact actions and behaviors and work to maintain a healthy balance between them. CBT can help with communication skills, especially when modified to support visual language for ASD treatment.

The earlier interventions and treatment plans are formed, the more effective they can be. Family units should work together with complete medical teams to provide the highest quality of life possible for everyone. CDD is a rare subset of autism that can be successfully supported with a team effort.