CDD was originally described by Austrian educator Theodor Heller (1869–1938) in 1908, 35 years beforeLeo Kanner andHans Asperger described autism. Heller had previously used the namedementia infantilis for this disorder.[9]
An apparent period of fairly normal development is often noted beforeregressions in skills occurs.[10] The age at which this regression can occur varies,[11] but regression after three years of normal development is typical.[12] The regression, known as aprodrome, can be so dramatic that the child may be aware of it, and may in its beginning even ask, vocally, what is happening to them.[13] Some children describe or appear to be reacting tohallucinations, but the most obvious symptom is that skills apparently attained are lost. Many children are already somewhat delayed when the disorder becomes apparent, but these delays are not always obvious in young children. Many writers have described the condition's impact as devastating, affecting both the family and the individual's future. As is the case with all PDD categories, there is considerable controversy about the right treatment for CDD.
CDD is a very rare condition, with only 1.7 cases per 100,000.[4][5]
A child affected with childhood disintegrative disorder shows normal development initially. Up until at least three years old,[1] the child has developed normally in the areas of language skills, social skills, comprehension skills, and has maintained those skills for about two years.[14][15] 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:[16]
Expressive language skills (being able toproduce speech and communicate a message)
All of the causes of childhood disintegrative disorder are still unknown.[1] Sometimes CDD surfaces abruptly within days or weeks, while in other cases it develops over a longer period of time. AMayo 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 ofepilepsy is higher in children with childhood disintegrative disorder, experts don't know whether epilepsy plays a role in causing the disorder."[19]
CDD, especially in cases of later age of onset, has also been associated with certain other conditions, particularly the following:[12]
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 themeasles 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.
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.[21]
Behavior therapy:Applied behavior analysis (ABA) is considered to be the most effective form of treatment forautism spectrum disorders by theAmerican Academy of Pediatrics.[22] The primary goal of ABA is to improve quality of life, and independence by teaching adaptive behaviors to children with autism,[23] and to diminish problematic behaviors like running away from home, or self-injury[24] by using positive or negative reinforcement to encourage or discourage behaviors over time.[25]
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 controlseizures.
^abcdefCharan, Sri Hari (January–April 2012)."Childhood disintegrative disorder".Journal of Pediatric Neurosciences.7 (1):55–57.doi:10.4103/1817-1745.97627.PMC3401658.PMID22837782.Childhood disintegrative disorder (CDD), also known as Heller's syndrome and disintegrative psychosis, is a rare condition characterized by late onset (>3 years of age) of developmental delays in language, social function, and motor skills. [...] The cause of childhood disintegrative disorder is unknown.
^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.PMID10660829.
^Dobbs, David (6 July 2016)."The most terrifying childhood condition you've never heard of".Spectrum. Simons Foundation Autism Research Initiative.Archived from the original on 25 September 2022. Retrieved25 September 2022.During this anxiety-ridden prologue, known as a 'prodrome,' a child will often seem keenly aware that something is wrong.
^Mughal, Saba; Faizy, Rubina M.; Saadabadi, Abdolreza (January 2022).Autism Spectrum Disorder. Treasure Island, Florida: StatPearls Publishing.PMID30247851.Archived from the original on 25 September 2022 – via National Center for Biotechnology Information Bookshelf.Treatment of childhood disintegrative disorder requires behavior therapy, environmental therapy, and medications.
^Matson, Johnny; Hattier, Megan; Belva, Brian (January–March 2012). "Treating adaptive living skills of persons with autism using applied behavior analysis: A review".Research in Autism Spectrum Disorders.6 (1):271–276.doi:10.1016/j.rasd.2011.05.008.
Westphal A, Schelinski S, Volkmar F, Pelphrey K (February 2013). "Revisiting regression in autism: Heller's dementia infantilis. Includes a translation of Über Dementia Infantilis".J Autism Dev Disord.43 (2):265–71.doi:10.1007/s10803-012-1559-z.PMID22677931.S2CID3924122.
