Childhood disintegrative disorder
|Childhood disintegrative disorder|
|Classification and external resources|
Childhood disintegrative disorder (CDD), also known as Heller's syndrome and disintegrative psychosis, is a rare (1.7 cases per 100,000) condition characterized by late onset (>3 years of age) of developmental delays in language, social function, and motor skills. Researchers have not been successful in finding a cause for the disorder.
CDD has some similarity to autism, and is sometimes considered a low-functioning form of it, but an apparent period of fairly normal development is often noted before a regression in skills or a series of regressions in skills. Many children are already somewhat delayed when the disorder becomes apparent, but these delays are not always obvious in young children.
The age at which this regression can occur varies, and can be from age 2-10 with the definition of this onset depending largely on opinion.
The regression can be so dramatic, that the child may be aware of it, and in its beginning he may even ask, vocally, what is happening to him. Some children describe or appear to be reacting to hallucinations, but the most obvious symptom is that skills apparently attained are lost. This has been described by many writers as a devastating condition, affecting both the family and the individual's future. As is the case with all pervasive developmental disorder categories, there is considerable controversy about the right treatment for CDD.
The syndrome was originally described by Austrian educator Theodor Heller in 1908, 35 years before Leo Kanner and Hans Asperger described autism, but it has not been officially recognised until recently. Heller had previously used the name dementia infantilis for the syndrome.
Signs and symptoms 
A child affected with childhood disintegrative disorder shows normal development and he/she acquires "normal development of age-appropriate verbal and nonverbal communication, social relationships, motor, play and self-care skills" comparable to other children of the same age. However, from between the age of 2 through the age of 10, 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 & 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
- Repetitive behavior & interest patterns
In her book, Thinking in Pictures, Temple Grandin claims that comparing to "Kanner's classic autism" and to Asperger syndrome, CDD is characterized with more severe sensory integration dysfunction but less severe cognitive problems. She also claims that comparing to most persons with autism, persons with CDD have more severe speech pathology and they usually do not respond well to stimulants.
The exact 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 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.
|This section does not cite any references or sources. (February 2013)|
There is no permanent cure for CDD - loss of language and skills related to social interaction and self-care are serious. The affected children face permanent disabilities in certain areas and require long term care. Treatment of CDD involves both behavior therapy and medications.
- Behavior therapy - Applied Behavior Analysis (ABA): Its aim is to systematically teach the child to relearn language, self-care and social skills. The treatment programs designed in this respect "use a system of rewards to reinforce desirable behaviors and discourage problem behavior." ABA programs may be designed by a board-certified specialist in behavior analysis called a "BCBA" (Board Certified Behavior Analyst), but ABA is also widely used by a number of other health care personnel from different fields like psychologists, speech therapists, physical therapists and occupational therapists with differing levels of expertise. Parents, teachers and caregivers are instructed to use these behavior therapy methods at all times.
- 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.
- Hendry CN (January 2000). "Childhood disintegrative disorder: should it be considered a distinct diagnosis?". Clin Psychol Rev 20 (1): 77–90. doi:10.1016/S0272-7358(98)00094-4. PMID 10660829.
- "Yale Developmental Disabilities Clinic: Childhood Disintegrative Disorder". Retrieved 2006-12-04.
- Childhood Disintegrative Disorder - Causes
Further reading 
- Let Me Hear Your Voice: A Family's Triumph over Autism / Catherine Maurice
- Westphal A, Schelinski S, Volkmar F, Pelphrey K. Revisiting Regression in Autism: Heller's Dementia Infantilis : Includes A Translation of Über Dementia Infantilis. J Autism Dev Disord. 2012 Jun 8. [Epub ahead of print] PubMed PMID: 22677931.