eMedicine Specialties > Pediatrics: Developmental and Behavioral > Medical Topics

Childhood Disintegration Disorder

Author: Bettina E Bernstein, DO, Assistant Professor, Department of Psychiatry, Philadelphia College of Osteopathic Medicine; Private Practice at the Wynnewood House; Consultant, Child Guidance Resource Centers, Early Elementary Education Program, Clinical Affiliate, Department of Child and Adolescent Psychiatry, Children's Hospital of Philadelphia
Contributor Information and Disclosures

Updated: Nov 23, 2009

Introduction

Background

Childhood disintegrative disorder, also known as Heller syndrome, manifests as a loss of previously acquired language and social skills and results in a persistent delay in these areas. For example, a child previously able to speak in 2- or 3-word phrases gradually or abruptly loses the ability to communicate using words or uses only fragments. Social and emotional development also regress, resulting in an impaired ability to relate with others. For example, a child previously able to accept reassurance from his or her parent (eg, a hug) loses the ability to be consoled and even may withdraw from human (tactile) contact.

Childhood disintegrative disorder is very rare, occurring in about 2 per 100,000 children, much rarer than autistic disorder (which affects 20 per 10,000 children) or pervasive developmental disorder, not otherwise specified (PDD-NOS), which occurs in 30 per 10,000 children.1 Childhood disintegrative disorder generally manifests by the fourth year of life, after a period of at least 2 years of normal development.

Overall, the social, communicative, and behavioral features of childhood disintegrative disorder resemble those of autistic disorder. Affected children have distinct qualitative impairments in social interaction and communication. In addition, restricted, repetitive, or stereotyped patterns of behavior, interests, and activities occur. Motor loss of previously acquired skills (eg, child previously toilet trained soils during the day and night, child previously able to pedal a tricycle or draw shapes can no longer do so) occurs. Additional symptoms may include the onset of difficulty in the transition of waking from sleep. Social interactions become compromised (eg, aggressiveness, tantrums, withdrawal from peers), as does motor function, resulting in poor coordination and possible awkwardness of gait.2

Family home movies can be very helpful in early identification of autistic spectrum disorders, including childhood disintegrative disorder.3

Typical case study of childhood disintegrative disorder

A 3-year-old boy is referred because his behavior he recently gotten him "expelled" from daycare. He screams and tantrums and no longer accepts hugs from his daycare teacher, with whom he was previously familiar. He has lately become destructive to his toys and does not use them to interact with his peers. His parents report that he was doing "okay" up until the past 3 months. They do not report that he had had any language delays.

Pathophysiology

Historically, flawed theories have held that "refrigerator mothers" (a term coined based on the presumed emotional frigidity of such mothers toward their children) caused autistic spectrum disorder in their children; these theories have caused unnecessary psychological pain in countless numbers of families.

Current research has yet to reveal a clear-cut pathophysiology for childhood disintegrative disorder; debate within the developmental disabilities field regarding long-term outcome of children with this disorder is ongoing. Some researchers hypothesize that predisposing genetic factors combined with environmental stressors (eg, prenatal or postnatal virus exposure, birth trauma) result in brain deposition of amyloid and disruption of synaptic transmissions, possibly involving interleukin-1 or beta-endorphins. Brain plasticity may be restored if enriched environmental experiences are provided during critical periods of development, potentially mitigating genetic predispositions. However, earlier disruption of psychosocial development can result in even more severe developmental consequences.

Several researchers have proposed the theory that altered or inappropriate immune responses, potentially involving abnormal B-cell activation and neuronal function, may play a role in pathophysiology. This concept is supported by some studies that have found that lithium and hyperbaric oxygen4 reduce oxidative stress and may increase blood flow to the brain, lending some support to the idea that a possible endophenotype leads to oxidative stress and neuronal injury.5,6,7,1

Autoantibodies to myelin basic protein (MBP) in children with childhood disintegrative disorder appear to differ from those in children with nonregressive forms of autism; however, this finding does not yet seem to correlate with disease severity or prognosis.5

No gene has yet been isolated as the cause of childhood disintegrative disorder; however, a gene that codes for elongator protein complex 4 (ELP4) has recently been identified on human chromosome 11 and appears to be associated with centrotemporal sharp waves (CTS), the electroencephalographic (EEG) feature typical of rolandic epilepsy (RE). ELP4 is one component of the elongator complex, a group of 6 genes that function in transcription and modification of transfer RNA (including regulating the actin cytoskeleton, cell motility, and migration) and that seem to play a critical role in influencing genes important to neuronal migration, axon growth, motility of growth cones, and related activities.8

ELP4 may also play a critical role in the transcription and modification of transfer RNA. Interference with necessary migration, synaptogenesis, and division in the CNS prior to adolescence may result from disruption of transcriptional regulatory and translational modification roles and may cause failure of dendrites to make the proper neuronal connections.9  

Autism spectrum disorders, including PDD, are generally associated with an increased incidence of seizures. Other idiopathic focal epilepsies occur during sleep. In addition, other epilepsy syndromes are associated with impaired frontal lobe and language function, potentially leading to continuous epileptiform discharges during sleep.10  

Frequency

United States

Childhood disintegrative disorder is very rare (2 per 100,000 children), much rarer than autistic disorder, which affects 20 per 10,000 children.1

International

No current studies are large enough to determine the international frequency of childhood disintegrative disorder.

Mortality/Morbidity

  • No mortality or morbidity is caused directly by childhood disintegrative disorder. Indirectly, a comorbid medical condition, such as a neurodegenerative disorder, may increase the risk of mortality and morbidity. The clinician should be alert to the possibility of Landau-Kleffner syndrome (LKS).11,12
  • LKS is a rare condition of unknown etiology that is more common in boys. LKS generally presents with more severe language impairment and later than childhood disintegrative disorder; LKS has a mean age of onset of 5.5 years. Excluding this syndrome is important because it is generally associated with seizure disorder and may respond to treatment with anticonvulsants such as valproic acid or steroids.11,12

Race

No studies have shown that childhood disintegrative disorder is any more or less common in any particular race or culture.

Sex

Childhood disintegrative disorder is slightly more common in males than in females.1

Age

Childhood disintegrative disorder, as defined by the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV), occurs only after a period of at least 2 years of normal development, when the child is younger than 10 years.13,1 Onset generally occurs in children aged 3-4 years and may be insidious or abrupt.

The average age at diagnosis is 3.9 years, similar to that of autistic disorder (average age of 3.1 years, with a range of 4.1-5.5 years in some studies).1

Clinical

History

  • Obtain a thorough history. If the family has home movies, this may help with early identification of a developmental departure from normalcy.3
  • Children with childhood disintegrative disorder are developmentally normal prior to the age of onset. This is similar to LKS; however, in LKS, the onset tends to be later (eg, age 5.5 y), whereas, in childhood disintegrative disorder, the onset is usually by age 3-4 years.13
  • Developmental delays in language, social, emotional, cognitive, or motor areas may not have been previously apparent to either the parent or pediatrician.
  • Impaired social sensitivity and affect regulation (inability to modulate anger and anxiety, resulting in rage and anxiety attacks) can occur with or without cognitive distortions (vivid grandiose fantasies, idiosyncratic logic) and can result in cascading effects that further decrease adequate socialization and diminish necessary preferential attention to the eyes of other human beings for adequate social interaction.10,2
  • Children diagnosed with childhood disintegrative disorder tend to have more long-lasting abnormalities of auditory responsiveness and verbal communication than children with PDD, but not as severe as in LKS. Although hyperlexia may be a feature of childhood disintegrative disorder, it is not as likely as in LKS.10,14

Physical

  • Perform a thorough physical examination.
  • Occasionally after diagnosis, mild neurologic abnormalities (eg, mild macrocephaly, microcephaly, motor incoordination, impaired sleep-wake cycles) are detected upon neurologic examination, necessitating a high index of suspicion for a seizure disorder.
  • Specific physical abnormalities are not diagnostic of this disorder; however, some affected children may have a history of increased ear infections reflective of possible decreased autoimmunity.15,16

Causes

No single causal factor for childhood disintegrative disorder is known. Current research emphasizes that a combination of genetic susceptibility, including possibly abnormal autoimmunity, and prenatal (or environmental) stress may explain the finding of higher-than-expected brain deposition of amyloid and disruption of synaptic transmission, possibly involving interleukin-1 or beta-endorphins.

As the child experiences developmental departure from normalcy, the diminished preferential attention to the eyes of others has cascading detrimental effects, decreasing further socialization. The child's attention focuses to aspects (point sounds, lights, lip motions), further impairing appropriate social interaction (eye contact, facial expression).17

  • Environmental risk factors
    • Viral exposure (usually intrauterine transmission) -Toxoplasmosis, other infections, rubella, cytomegalovirus infection, and herpes simplex (TORCH)
    • Birth trauma
    • Toxin exposure
    • Prematurity
    • Teratogenicity: Recent research directions include the possible association of ophthalmologic malformations with autistic spectrum disorder resulting from the teratogenicity of thalidomide and misoprostol.18,19
  • Genetic factors
    • Possible susceptibility to chromosomal breakage or disruption
    • Family history of autism or Asperger disorder
    • Family history of rolandic epilepsy (RE), with or without centrotemporal spikes (CTS): RE commonly affects prepubescent children aged 3-12 years, occurring in about 1 per 500 children. Most cases (60%) are diagnosed in boys. Seizures in RE typically begin as groaning noises (in the larynx) that interfere with speech and then may present as sensorimotor activity. Seizures in benign RE (BRE) occur at night and during sleep, possibly because sleep is associated with a lowered seizure threshold.20,21
    • CTS is also associated with attention deficit/hyperactivity disorder (ADHD), speech disorders, and developmental coordination disorder. In many cases, CTS due to RE is missed, as the child’s history of falls can be mistakenly attributed to poor coordination.21
  • Associated disorders
    • Autoimmune disorders16
    • Allergies (eg, food sensitivity reactions) and gastrointestinal disorders, which can include asthma22
    • Insomnia23

More on Childhood Disintegration Disorder

Overview: Childhood Disintegration Disorder
Differential Diagnoses & Workup: Childhood Disintegration Disorder
Treatment & Medication: Childhood Disintegration Disorder
Follow-up: Childhood Disintegration Disorder
References
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Further Reading

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Keywords

childhood disintegrative disorder, childhood disintegration disorder, Heller syndrome, dementia infantilis, disintegrative psychosis, language loss, social development regression, emotional development regression, neuroleptic malignant syndrome, NMS, autistic disorder, autism, tantrums, pervasive developmental disorder, Asperger disorder

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Contributor Information and Disclosures

Author

Bettina E Bernstein, DO, Assistant Professor, Department of Psychiatry, Philadelphia College of Osteopathic Medicine; Private Practice at the Wynnewood House; Consultant, Child Guidance Resource Centers, Early Elementary Education Program, Clinical Affiliate, Department of Child and Adolescent Psychiatry, Children's Hospital of Philadelphia
Bettina E Bernstein, DO is a member of the following medical societies: American Academy of Child and Adolescent Psychiatry and American Psychiatric Association
Disclosure: Nothing to disclose.

Medical Editor

Carol Diane Berkowitz, MD, Executive Vice Chair, Department of Pediatrics, Professor, Harbor-University of California at Los Angeles Medical Center
Carol Diane Berkowitz, MD is a member of the following medical societies: Alpha Omega Alpha, Ambulatory Pediatric Association, American Academy of Pediatrics, American College of Emergency Physicians, American Medical Association, American Pediatric Society, and North American Society for Pediatric and Adolescent Gynecology
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Caroly Pataki, MD, Professor of Clinical Psychiatry and Behavioral Sciences, Department of Psychiatry, Division Chair, Child and Adolescent Psychiatry, Director of Training, Child and Adolescent Psychiatry Residency Program, University of Southern California Keck School of Medicine
Caroly Pataki, MD is a member of the following medical societies: American Academy of Child and Adolescent Psychiatry, New York Academy of Sciences, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

Chief Editor

Caroly Pataki, MD, Professor of Clinical Psychiatry and Behavioral Sciences, Department of Psychiatry, Division Chair, Child and Adolescent Psychiatry, Director of Training, Child and Adolescent Psychiatry Residency Program, University of Southern California Keck School of Medicine
Caroly Pataki, MD is a member of the following medical societies: American Academy of Child and Adolescent Psychiatry, New York Academy of Sciences, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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