Childhood disintegrative disorder, also known as Heller's syndrome or disintegrative psychosis, is a rare disorder that’s part of a larger developmental disorder category on the autism spectrum. This condition tends to affect children over the age of three, or those with at least two years of normal development without concerns. This disorder is associated with seizure activity, and in at least half of cases, abnormal EEG results are present. EEG, or electroencephalography, is a method of recording electrical activity in the brain.
Childhood disintegrative disorder, or CDD, is listed under autism spectrum disorders in the Diagnostic and Statistical Manual of Mental Disorders (DSM-5) by the American Psychiatric Association. New descriptions due to a better understanding of autism have led to better diagnosing of spectrum disorders. These advances in the understanding of autism spectrum disorders have led to the following disorders falling under autism spectrum disorders in the DSM-V on the American Psychiatric Association website:
- Autistic spectrum disorder: ASD is a disorder that tends to cause problems with social interaction. It is characterized by repetitive behaviors and may cause problems with communication.
- Pervasive developmental disorder: This disorder is often characterized by avoidance of eye contact, problems controlling emotions, a high pitched or flat tone of voice, problems communicating thoughts through language, and repetitive behaviors.
- Childhood disintegrative disorder: Childhood disintegration disorder (CDD) is characterized by a loss of achieved developmental skills that usually occurs after the age of three.
The symptoms of CDD tend to be severe and obvious, and researchers are still searching for the cause of this disorder.
Children with this disorder tend to experience a sudden reversal in social skills, motor skills, and language skills.
Before modern research into CDD, it was thought that this condition was a medical disorder with identifiable medical causes. Eventually, researchers realized that the same medical or neurological factors do not always cause CDD. The symptoms of this disorder are well documented, but the cause is not.
The Symptoms Of Childhood Disintegrative Disorder
The symptoms of childhood disintegrative disorder revolve around a loss of achieved skill sets. The symptoms have a late onset and become obvious at or after the age of three after the child has learned to speak, walk, eat without help, and use the bathroom. The symptoms can become severe, and seizures and abnormal EEGs are common. Speech impediments are a common concern that should be treated as soon as possible. Although the disorder usually arises between the ages of three and four, it can occur up to the age of ten.
The following is a list of common symptoms of childhood disintegrative disorder:
- Loss of expressive language skills, or the ability to speak
- Loss of receptive language skills, or the ability to understand
- Loss of ability to communicate effectively and develop peer realtions
- Loss of social skills, inability to interact with others, and appearance of ignoring others
- Lack of communicating needs, such as hunger, fatigue, or thirst
- Lack of ability to control emotions
- Loss of the ability to walk
- Loss of the ability to understand language
- Loss of hand-eye coordination
The symptoms of this disorder can range in severity. A child who has lost their ability to wash themselves, brush their teeth, use the toilet, maintain bowel or bladder control, or feed themselves may have symptoms of CDD. This is a complex disorder and a rare condition characterized by a reversal in a child’s development after having apparently normal development before. These symptoms may seem to follow new seizure activity or a long period of illness. However, they may also appear out of the blue.
A specialist must diagnose this condition by careful analysis of the symptoms, the onset, and the severity. A primary care doctor, therapist, clinical assistant professor, or medical director should be able to guide you toward the correct specialist. They will likely use differential diagnosis to ensure accuracy, because, like schizophrenia spectrum disorders, CDD has many crossover symptoms. Although the symptoms of CDD progress over time, some individuals experience slower progression of developmental delays than others.
The rarity of this disorder can make it difficult to give an exact prognosis. Some children regress faster than others, and the extent of regression can vary from case to case. Research into this disorder shows that some children may stop regressing and re-learn skills, while others continue to regress. It can help to recognize the symptoms of CDD as early as possible so that a diagnosis can be made and therapy can begin.
Treatment Of CDD
There is currently no cure for CDD, but early intervention and therapy may help to lessen the severity of the symptoms children develop. Treatment for CDD tends to focus on slowing the loss of acquired skills and using therapy to help children re-learn what has been lost. As with most pervasive developmental disorders, some children respond better than others, but every bit of progress can help.
The therapies used for CDD tend to be similar to those used for the treatment of autism. Also, medications such as anti-convulsants may be used if seizures are diagnosed, and antipsychotics and selective serotonin reuptake inhibitors may be prescribed to control other severe behavioral problems that may develop.
Therapy for CDD often includes:
- Medication therapy
- Behavioral therapy
- Sensory Enrichment Therapy
Behavioral therapy may be used to slow down the loss of skills and help a child re-learn skills and adaptive behaviors when possible. Qualified, certified professionals develop plans for slowing loss and re-teaching skills using a system of rewards for desired behavior. Speech therapists, occupational therapists, and physical therapists have different types of expertise to deliver customized therapy. A behavior analyst may first study a child's behaviors and then use that analysis to develop a care plan.
Sensory Enrichment Therapy
This type of therapy is designed to provide sensory enrichment that can ease the symptoms of CDD. Sensory enrichment therapy is often used to improve the quality of life of those with autism spectrum disorders or developmental disorders. The exact type of sensory enrichment is usually developed by a specialist who provides a customized experience. This therapy is not typically designed to slow down the loss of skills or help a child re-learn skills that are lost; it is usually meant to provide a means of interaction and communication that improves quality of life.
Family And In-Home Caregivers
Children with this disorder can respond well to therapy, but it is not yet known why some respond better than others. This condition is rare, and research is based on the limited number of cases available to study. Support for parents of children with CDD is often essential. Support groups for families can be an invaluable source of information and connection.
Family therapists can also be a useful resource for families of children diagnosed with CDD or another intellectual disability. The diagnosis can be overwhelming for loved ones, but talking with a qualified family therapist can help everyone through the challenges associated with CDD. Family therapy or group talk therapy can provide an outlet for families to discuss all the changes they are facing after receiving diagnosis. Siblings often have to adjust to new routines as parents work to accommodate the changes to their daily routines. All families must learn about the disorder and learn how to manage life with the changes taking place.
At some point, the regression experienced by those with CDD usually begins to affect bodily function, peers, motor skills, and bowel and bladder control. An individual with this condition may need extra support from a professional caregiver as they grow older. An in-home caregiver can make it easier as both parents and the child age.
An in-home caregiver is someone who tends to know all about the disorder, and they are trained to handle any situation that arises. This extra help can give parents and other family members time to relax and take care of the other demands of life. An in-home caregiver also gives the child interaction with someone other than family; this can be therapeutic as well.
Another option for care is a personal aide. A child with CDD may require around-the-clock supervision and care. A personal caregiver or personal aide is someone who provides that supervision and care when the primary caregiver cannot. Children with autism spectrum disorders often attend school with aides who help them in class. Depending on the regression, a child with CDD may still attend school for a period, and if the disorder stops progressing, they may remain in school.
In-home or personal aides tend to deliver the same quality of care and supervision the primary caregiver does. They usually provide this care in the absence of the primary caregiver or alongside the primary caregiver. This type of care can help individuals with this disorder handle life situations, providing quality of life and exposure to new situations.
If you have a child with childhood disintegrative disorder (CDD), you don’t have to face this challenge alone. It may help to talk to a therapist with knowledge and experience helping families with the challenges associated with CDD. If leaving the house is difficult due to childcare, you may opt for online therapy, which has been proven to be just as effective as in-person therapy. With BetterHelp, you and your family can talk to a therapist from the comfort of your home via audio or video chat. Also, if questions arise in between sessions, you can contact your therapist via in-app messaging, and they’ll get back to you as soon as they can.
If you know you’d like to talk to a therapist about CDD, other mental disorders, or if you simply have questions about the therapy process, you can contact BetterHelp to get answers to your questions. With BetterHelp, you can get matched with a therapist with experience helping families facing the challenges of CDD and autism spectrum disorders. Take the first step and reach out.
What are the symptoms of childhood disintegrative disorder?
The current (5th) edition of the Diagnostic and Statistical Manual of Mental Disorders, also known as the DSM-V, does not classify CDD separately from autism spectrum disorder (ASD). The previous edition, or DSM-IV, described two categories of symptoms for childhood disintegrative disorder. Diagnosis required an individual to display multiple symptoms from both categories.
Category #1: Regression symptoms (loss of developmental skills). This could include impairments and diminishments in abilities like:
- Previously acquired language skills, including both the ability to understand and use words
- Social skills and ability to relate to peers
- Motor skills
- Bowel and bladder control
- Adaptive behaviors (life skills and self care)
- Imagination and play
Category #2: Abnormal functioning. Diagnosis of CDD under previous DSM criteria required at least two of the following types of behavioral difficulties:
- Diminished social function, such as an inability to develop peer relations or reciprocate emotions and social cues
- Challenges with communication, such as an inability to start or continue conversations or a repetitive, restricted set of verbal expressions
- Limited, repetitive, and often non-functional behavior, including a narrow range of interests and patterns of movements or gestures without an obvious purpose
- Lack of interest in the surrounding environment, including objects and other people
Another important diagnostic feature was the lack of apparent symptoms before at least age 2, followed by a rapid, severe regression. The resulting loss of abilities may be more severe than in other autism-related conditions, and more frequently accompanied by seizures. During the onset of regression, children often display fear, pain, and confusion. Some may show signs of hallucinations and delusions.
What is the difference between CDD and autism?
Many psychological researchers and mental health practitioners do not consider CDD a separate disorder from autism. However, others have argued that its developmental course and characteristic symptoms are distinct enough that they should be diagnosed, studied, and treated as different illnesses.
Research suggests that, compared with more typical examples of ASD, many children with CDD exhibit:
- Faster regression
- More typical-seeming development before onset
- Greater loss of function
- A higher prevalence and intensity of emotional disturbance
- More widespread loss of developmental gains like toilet training
A 2017 analysis of the brain structure and genetic makeup of patients with childhood disintegrative disorder noted characteristic patterns of gene expression and neurological function that appeared different from more common cases of ASD. Several of the genes apparently connected to CDD were widely expressed outside the neocortex, the part of the brain most active in higher-order functions like abstract thought, language, and spatial reasoning. In contrast, many of the genes known to be linked to other forms of autism appear to be most concentrated in the neocortex.
These characteristics may be related to differences in eye-tracking behavior. People with CDD seem to be more likely to focus on faces than those with other subtypes of ASD. Some of the genes identified in the 2017 study appear to be highly expressed in brain areas that are active when patients are paying attention to faces.
Is there a cure for childhood disintegrative disorder?
Currently, there is no known cure for childhood disintegrative disorder. Though some forms of treatment may help manage symptoms or assist individuals in regaining some limited function, no reliable way to halt or reverse the effects of CDD has been found.
Research into treatment is difficult due to the lack of a single clearly understood cause. Despite its often dramatic onset, no genes have been definitively identified as responsible. It’s possible that CDD represents a pattern of disrupted development that can result from multiple causes.
For example, a case report published in the Journal of Pediatric Neurosciences describes an apparent incidence of childhood disintegrative disorder following a severe upper respiratory tract infection with prolonged fevers. It could be that certain kinds of infection-related brain damage can trigger the developmental regression seen in CDD.
How do you treat childhood disintegrative disorder?
Treatment for childhood disintegrative disorder often involves a combination of the following:
- Specialized education and counseling. Family members caring for a child with CDD may benefit from learning more about the condition, as well as coaching on how to better help the affected individual. People with CDD may also be able to regain some functioning with the help of special education programs.
- Environmental therapy. Childhood disintegrative disorder may also be treated with sensory-based therapies to reduce both cognitive and behavioral symptoms.
- Behavior therapy. Other therapies for CDD include a variety of methods aimed at building particular types of skills and teaching appropriate behaviors, such as communicating emotions or fostering peer relations. The American Academy of Pediatrics considers Applied Behavior Analysis, a type of behavior therapy, to be the most effective treatment for autism disorders such as CDD.
- Medication. There are currently no known pharmacological treatments that directly relieve the developmental deficits of childhood disintegrative disorders. However, medications such as atypical antipsychotics may be used to reduce behavior problems such as aggression and repetitive behavior. Patients with seizures may also receive anticonvulsant medications.
What are the characteristics of CDD?
According to a systematic review of the literature on CDD, its notable characteristics include many features often found in autism spectrum disorder, including:
- Limited language skills
- Intellectual impairment
- Social deficits
- Narrow, stereotyped interests
- Repetitive behaviors
- Diminished imaginative play
- Regression after apparently typical childhood development
- Disruptive or difficult-to-manage behavior
However, the researchers noted that the level of cognitive and developmental regression in CDD was often much greater than that observed in patients with ASD. Loss of social and intellectual skills seemed to be both more severe and more rapid. It was also more likely to occur without warning signs.
Widespread loss of basic adaptive skills such as bladder control or the ability to feed oneself seems more common in CDD. So does the incidence of epileptic seizures, loss of speech, and severe intellectual disability. There also seems to be a greater likelihood that children with CDD will exhibit emotional disturbance such as fear and anxiety, or psychotic symptoms such as hallucinations, during regression.
What type of autism is high-functioning autism?
“High-functioning autism” is not a formal subtype or category of autism. Instead, it’s a description used for people with ASD who are able to achieve some level of independence, social functioning, and autonomy despite the difficulties that come with their condition.
In the past, the term was often used specifically for ASD without accompanying intellectual and cognitive impairment. Many experts argue that this is an imprecise and misleading usage since there’s not a clear correlation between cognitive ability and successful day-to-day functioning in individuals with autism.
Why is CDD so important?
Researchers who study CDD believe that it may offer important clues to the causes and mitigating factors involved in various types of autism. It may also shed light on both healthy and unhealthy childhood development.
Since childhood disintegrative disorder seems to involve more severe symptoms, as well as a more sudden onset, it may help scientists understand the mechanisms behind these symptoms. If certain kinds of genetic differences, environmental influences, brain pathways, or other factors are more prevalent in CDD, they may be important targets for the diagnosis and treatment of various kinds of neurodevelopmental disorders in the future.
What is childhood disintegrative disorder also known as?
Childhood disintegrative disorder is also sometimes called “Heller’s disorder”, after the special educator (Theodor Heller) who first described it in 1908. Heller called the disease “dementia infantilis”. It’s also sometimes called “disintegrative psychosis”, in reference to the hallucinations and other apparent psychotic symptoms sometimes seen during the period of regression.
What are some facts about childhood disintegrative disorder?
According to the National Institutes of Health, some key facts about childhood disintegrative disorder include:
- This disease is rare, estimated to occur in about 1.7 out of 100,000 children
- The developmental regression seen in CDD most commonly occurs at age four, though there is considerable variation
- The near-total loss of function in certain aspects of development is usually complete by age ten
- Late-onset CDD may be linked to certain specific diseases, including:
- Subacute sclerosing panencephalitis, a measles-related infection that causes brain inflammation and death of nerve tissue
- Tuberous sclerosis (TSC), a genetic disorder leading to benign tumors in the brain and other organ systems
- Leukodystrophy, a developmental disorder causing malformation of the myelin sheath (part of the neuron important for clear nerve transmission)
- Lipid storage diseases, conditions in which unhealthy deposits of fatty tissue accumulate in the brain and nerves
- The prognosis for CDD is poor compared to other forms of autism, but if diagnosed early and managed with help from a trained care team, some limited improvement in life skills and outcomes may be possible
- Previous Article
- Next Article