Childhood disintegrative disorder
| Childhood disintegrative disorder | |
|---|---|
| Synonyms | Heller's syndrome, disintegrative psychosis |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Loss of previously acquired skills, including language, social, and motor skills |
| Complications | N/A |
| Onset | Typically after age 3, but before age 10 |
| Duration | Chronic |
| Types | N/A |
| Causes | Unknown, possibly genetic and environmental factors |
| Risks | Family history of autism spectrum disorder |
| Diagnosis | Based on clinical observation and developmental history |
| Differential diagnosis | Autism spectrum disorder, Rett syndrome, Childhood schizophrenia |
| Prevention | N/A |
| Treatment | Behavioral therapy, speech therapy, occupational therapy |
| Medication | N/A |
| Prognosis | Variable, often poor |
| Frequency | Rare, less than 2 per 100,000 |
| Deaths | N/A |
Childhood Disintegrative Disorder
Childhood Disintegrative Disorder (CDD), also known as Heller's syndrome, is a rare condition characterized by late onset of developmental delays—or severe and sudden reversals—in language, social function, and motor skills. It is classified under the umbrella of autism spectrum disorders (ASDs) and is considered one of the most severe forms.
History
The disorder was first described by Austrian educator Theodor Heller in 1908, who observed a pattern of normal development followed by a significant regression in children. Initially, it was considered distinct from autism, but later research has placed it within the autism spectrum due to overlapping symptoms.
Symptoms
Children with CDD typically develop normally for at least the first two years of life. They acquire skills in language, social interaction, and motor abilities. However, between the ages of 3 and 10, they experience a dramatic loss of these skills. The symptoms include:
- Loss of previously acquired language skills
- Loss of social skills and adaptive behaviors
- Loss of bowel or bladder control
- Loss of motor skills
- Lack of play
- Impaired nonverbal communication
Diagnosis
Diagnosis of CDD is challenging due to its rarity and the overlap of symptoms with other developmental disorders. A comprehensive evaluation by a team of specialists, including a pediatrician, neurologist, and psychologist, is often required. The diagnostic criteria include:
- Normal development for at least the first two years
- Significant loss of previously acquired skills before age 10
- Symptoms not better explained by another condition
Causes
The exact cause of CDD is unknown. However, it is believed to involve a combination of genetic, neurological, and environmental factors. Some studies suggest abnormalities in the brain's structure or function, but more research is needed to understand the underlying mechanisms.
Treatment
There is no cure for CDD, and treatment focuses on managing symptoms and improving quality of life. Interventions may include:
- Behavioral therapy
- Speech therapy
- Occupational therapy
- Medications to manage specific symptoms, such as anxiety or seizures
Early intervention is crucial to help children regain some of their lost skills and to improve their ability to function.
Prognosis
The prognosis for children with CDD varies. Some children may regain some skills with intensive therapy, while others may continue to experience significant challenges. The disorder often results in lifelong disability, requiring ongoing support and care.
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Contributors: Prab R. Tumpati, MD