Childhood Disintegrative Disorder

Every parent are sure proud when they see their baby learn to crawl, turn over, and walk, achieving developmental milestones. But what if a child suddenly regresses and become very delayed in development? This scenario exist, and this is called childhood disintegrative disorder.

What is childhood disintegrative disorder?

Childhood disintegrative disorder (CDD) also known as Heller’s syndrome and disintegrative psychosis, is a rare developmental disorder characterized by  late onset of developmental delays in language, social function, and motor skills.[1] CDD has some similarity to autism, and is sometimes considered a low-functioning form of it.[1][2][3] CDD is similar to dementia – skills already learned and acquired suddenly disappear and regress; the difference is CDD is occurring in toddlerhood, commonly in 3 years of age.

CDD  was originally described by Austrian educator Theodor Heller in 1908, 35 years before Leo Kanner and Hans Asperger described autism.[1] Heller had previously used the name dementia infantilis for the syndrome.[1][4]

Childhood disintegrative disorder is part of a larger category called autism spectrum disorder. However, unlike autism, someone with childhood disintegrative disorder shows severe regression after several years of normal development and a more dramatic loss of skills than a child with autism does. In addition, childhood disintegrative disorder can develop later than autism does.[5]

What causes CDD?

The cause of CDD is still unknown. There is not enough research on this rare disorder to determine a cause.[6]  It has been linked to brain and nervous system problems.[7]

How common is CDD?

More boys than girls appear to be affected. Childhood disintegrative disorder is perhaps 10 times less common than more strictly defined autism and is estimated to occur in between 1-2 children per 100,000.[8]

What are the symptoms of CDD?

CDD has the following characteristics taken from MedlinePlus[7]:

  • Delay or lack of spoken language
  • Impairment in nonverbal behaviors
  • Inability to start or maintain a conversation
  • Lack of play
  • Loss of bowel and bladder control
  • Loss of language or communication skills
  • Loss of motor skills
  • Loss of social skills
  • Problems forming relationships with other children and family members

Additional symptoms may include the onset of difficulty in the transition to waking from sleep. Social interactions become compromised (as manifested by aggressiveness, tantrums, or withdrawal from peers), as does motor function, resulting in poor coordination and possible awkwardness of gait.[9][10]

Image courtesy of Dreamstime. A toddler with childhood disintegrative disorder (CDD) may regress from walking to clumsy gait and needs support like walker.

Image courtesy of DSM-IV/rickpdx.files.wordpress.com. CDD is under the umbrella of autism spectrum disorders (ASD).

How is CDD diagnosed?

CDD is most commonly diagnosed when the parents of the affected child consult the pediatrician about the child’sloss of previously acquired skills. The doctor will first give the child a medical examination to rule out epilepsy or other medical conditions.[11] The child’shead may also be x rayed to rule out head trauma or a brain tumor. Following the medical examinations and tests, the child will be referred to a psychiatrist who specializes in treating children and adolescents. The psychiatrist will then make the differential diagnosis of CDD.[11]

To be diagnosed with CDD, a child must show loss or regression in at least two of the areas listed below. Usually regression occurs in more than two areas. These are[11]:

  • receptive language skills (language understanding)
  • expressive language skills (spoken language)
  • social or self-help skills
  • play with peers
  • motor skills
  • bowel or bladder control, if previously established

CDD must be differentiated from autism and such other specific pervasive developmental disorders as Rett’s disease. It also must be differentiated from schizophrenia . One of the differences between CDD and other PDDs is that to be diagnosed with CDD, a child must develop normally for at least two years before loss of skills occurs, and the loss must occur before age ten. Parents’ reports of the child’s development, records in baby books, medical records kept by the child’s pediatrician, and home movies are often used to document normal development through the first two years of life.[11]

How is CDD managed?

CDD’s management is similar to other autism spectrum disorder (ASD) management[1]:

  • Behavior therapy: The main aim of Applied Behavior Analysis (ABA) 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.[1]
  • Environmental Therapy: Sensory Enrichment Therapy uses enrichment of the sensory experience to improve symptoms in autism, many of which are common to CDD.[1]
  • 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.[1]

What is the prognosis of CDD?

The outlook for this disorder is poor. Most children with the condition have an impairment similar to that of children with severe autism by age 10.[7]

  • Effects on intellectual function, self-sufficiency and adaptive skills are profound, with most cases regressing to severe intellectual disability.[12]
  • Medical co-morbidities such as epilepsy commonly develop.[12]
  • Those with moderate-to-severe mental intellectual disability or with an inability to communicate tend to do worse than those left with a higher IQ and some verbal communication.[12]
  • Outlook is poor. Children will require lifelong support.[12]
  • Risk of seizures increases throughout childhood, peaking at adolescence, and seizure threshold may be lowered by SSRIs and neuroleptics.[12]
  • Life expectancy has previously been reported as normal. However, more recent studies suggest that mortality of people with autistic spectrum disorders is twice that of the general population, mainly due to complications of epilepsy.[12][13]

Because CDD is a rare condition, differential diagnoses are ruled out first before considering CDD as a disorder[12]:

The differential diagnosis incudes any of the other pervasive developmental disorders (autistic spectrum disorder, Rett’s Syndrome, PDD-NOS) or causes of general learning disability. Other specific conditions which need to be ruled out are[12]:

48421855

Image courtesy of thescrewjack.com. On the autism spectrum scale, CDD is considered the most severe form of ASD.

CDD is one of the most devastating developmental disorders. This really require awareness, acceptance, and support from all those involved – parents, family, medical personnel, and the community at large. Even if the child with CDD cannot speak, still show unconditional love for her and surely she will thank you for loving her no matter what.

Reference:

  1. https://en.wikipedia.org/wiki/Childhood_disintegrative_disorder
  2. McPartland J, Volkmar FR (2012). “Autism and related disorders”. Handb Clin Neurol 106: 407–18.doi:10.1016/B978-0-444-52002-9.00023-1.PMID 22608634.
  3. Venkat A, Jauch E, Russell WS, Crist CR, Farrell R (August 2012). “Care of the patient with an autism spectrum disorder by the general physician”. Postgrad Med J 88 (1042): 472–81. doi:10.1136/postgradmedj-2011-130727. PMID 22427366.
  4. Mouridsen SE (June 2003). “Childhood disintegrative disorder”. Brain Dev. 25 (4): 225–8.doi:10.1016/s0387-7604(02)00228-0. PMID 12767450.
  5. http://www.mayoclinic.org/diseases-conditions/childhood-disintegrative-disorder/basics/definition/con-20026858
  6. http://www.mayoclinic.org/diseases-conditions/childhood-disintegrative-disorder/basics/causes/con-20026858
  7. http://www.nlm.nih.gov/medlineplus/ency/article/001535.htm
  8. http://childstudycenter.yale.edu/autism/information/cdd.aspx
  9. http://emedicine.medscape.com/article/916515-overview
  10. Volkmar FR, State M, Klin A. Autism and autism spectrum disorders: diagnostic issues for the coming decade. J Child Psychol Psychiatry. 2009 Jan. 50(1-2):108-15.
  11. http://www.minddisorders.com/Br-Del/Childhood-disintegrative-disorder.html
  12. http://patient.info/doctor/childhood-disintegrative-disorder-hellers-syndrome
  13. Mouridsen SE, Bronnum-Hansen H, Rich B, et al; Mortality and causes of death in autism spectrum disorders: an update. Autism. 2008 Jul;12(4):403-14. doi: 10.1177/1362361308091653
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