Childhood disintegrative disorder

Signs and symptoms

CDD is a very rare condition, with only 1.7 cases per 100,000.

A child affected with childhood disintegrative disorder shows normal development initially. Up until at least three years old, the child has developed normally in the areas of language skills, social skills, comprehension skills, and has maintained those skills for about two years. However, between the ages of three and ten, these skills acquired are lost almost completely in at least two of the following six functional areas:

• Expressive language skills (being able to produce speech and communicate a message)
• Receptive language skills (comprehension of language – listening and understanding what is communicated)
• Social skills and self care skills
• Control over bowel and bladder
• Play skills
• Motor skills

Lack of normal function or impairment also occurs in at least two of the following three areas:

• Social interaction
• Communication
• Repetitive behavior and interest patterns

Most children with CDD regress to severe intellectual disability.

Causes

All of the causes of childhood disintegrative disorder are still unknown. Sometimes CDD surfaces abruptly within days or weeks, while in other cases it develops over a longer period of time. A Mayo Clinic report indicates: "Comprehensive medical and neurological examinations in children diagnosed with childhood disintegrative disorder seldom uncover an underlying medical or neurological cause. Although the occurrence of epilepsy is higher in children with childhood disintegrative disorder, experts don't know whether epilepsy plays a role in causing the disorder."

CDD, especially in cases of later age of onset, has also been associated with certain other conditions, particularly the following:

• Lipid storage diseases: In this condition, a toxic buildup of excess fats (lipids) takes place in the brain and nervous system.
• Subacute sclerosing panencephalitis: Chronic infection of the brain by a form of the measles virus causes subacute sclerosing panencephalitis. This condition leads to brain inflammation and the death of nerve cells.
• Tuberous sclerosis (TSC): TSC is a genetic disorder. In this disorder, tumors may grow in the brain and other vital organs like kidneys, heart, eyes, lungs, and skin. In this condition, noncancerous (benign) tumors, hamartomas, grow in the brain.
• Leukodystrophy: In this condition, the myelin sheath does not develop in a normal way, causing white matter in the brain to eventually fail and disintegrate.
• Encephalitis: Encephalitis is inflammation of the brain sometimes caused by viral or bacterial infection, highlighting the importance of childhood vaccinations, especially in people with compromised immune systems.

Treatment

Loss of language and skills related to social interaction and self-care are serious. The affected children face ongoing disabilities in certain areas and require long-term care. Treatment of CDD involves both behavior therapy, environmental therapy and medications.

• Behavior therapy: Applied behavior analysis (ABA) is considered to be the most effective form of treatment for autism spectrum disorders by the American Academy of Pediatrics. The primary goal of ABA is to improve quality of life, and independence by teaching adaptive behaviors to children with autism, and to diminish problematic behaviors like running away from home, or self-injury by using positive or negative reinforcement to encourage or discourage behaviors over time.
• Environmental therapy: Sensory enrichment therapy uses enrichment of the sensory experience to improve symptoms in autism, many of which are common to CDD.
• Medications: There are no medications available to directly treat CDD. Antipsychotic medications are used to treat severe behavior problems like aggressive stance and repetitive behavior patterns. Anticonvulsant medications are used to control seizures.

References

References

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