Autism Spectrum Disorders

Childhood Disintegrative Disorder

Childhood disintegrative disorder, also termed Heller’s syndrome and disintegrative psychosis, is classified as both a pervasive developmental disorder and an autism spectrum disorder, due to its similarities with autism. A very rare disorder (fewer than 2 cases per 100,000), it is characterized by a period of at least two years of normal development followed by a loss of previously attained skills. Childhood disintegrative disorder generally occurs between 2 and 10 years of age, and results in a regression of skills and further developmental delays in language, social and motor skills. Previously acquired milestones, such as potty training and the ability to ride a bike, can be lost. Regression is generally rapid, but can be slow, such that children may be aware of the changes occurring. There is no known cause of childhood disintegrative disorder.

Symptoms

Childhood disintegrative disorder is described as a condition in which normal, age-appropriate development occurs for at least two years, followed by a loss of acquired skills. This includes loss of verbal and non-verbal communication skills, motor skills, social and play skills, as well as self-help skills, such as the ability to feed and wash. Diagnosis of childhood disintegrative disorder is based on the almost complete loss of at least two of the following skills:

• expressive communication, including loss in the ability to speak;

• receptive communication, including loss in the ability to understand others;

• motor, including loss in the ability to walk, climb and grasp objects;

• social, including loss in the ability to relate and interact with others;

• play, including loss of interest in games, activities and imaginary play;

• self-help, including urinary or fecal incontinence.

Generally, symptoms appear rapidly over a period of 6 to 9 months however, they can also appear gradually. Regression of skills is often accompanied by anxiety, agitation and anger. While regression will eventually stop, the skills lost are usually never to be gained back.

Causes

While there is no known cause of childhood disintegration disorder, researchers hypothesize that it arises in the neurobiology of the brain, since approximately half of the children diagnosed with this disorder have abnormal electrical activity in the brain and there is a strong association with seizures and epilepsy. Factors suggested to play a role in the development of this disorder include a combination of abnormal immune responses, genetic susceptibility and environmental stressors, such as viral exposure or trauma prior to or at birth. However, further research to determine a cause is impeded by the rare occurrence of the disorder.

Management

There is no cure for childhood disintegration disorder, so treatment is based on early and aggressive educational intervention. Similar to treatment for autism, treatment is based on relieving symptoms.

Behavioral treatments include speech and language therapy, social skill development, as well as occupational therapy to improve sensory and motor skills. The use of rewards to reinforce desired behaviors and discourage inappropriate behaviors is often helpful. Medications, such as anti-anxiety, anti-depressants and anti-psychotics, can be used in cases where severe behavior concerns can threaten safety of patients and others. Parental education is important to support the child’s treatment at home. Support groups for families can help reduce stress and the emotional toll that living with childhood disintegration disorder may bring.

Prognosis

The prognosis for childhood disintegration disorder is rather guarded, due to the fact that it is very rare and more information is needed. However, based on current knowledge, the prognosis for children suffering from this disorder is poor. While no mortality or morbidity is associated with this disorder, difficulties associated with behavioral, social and communication skills tend to impair function throughout life. Once skills are lost, they are usually not regained. Only approximately 20% of children will reacquire the ability to speak in sentences. The risk of seizure increases with age, however this peaks at adolescence. Adults suffering from this disorder remain dependent on full-time caregivers.