Heller’s syndrome

Alternative names
Disintegrative psychosis; Childhood disintegrative disorder

Definition
Childhood disintegrative disorder is a condition occurring in 3- and 4-year-olds who have developed normally to age 2. Over several months, a child with this disorder will deteriorate in intellectual, social, and language functioning from previously normal behavior.

Causes, incidence, and risk factors

The cause of childhood disintegrative disorder is unknown, but it has been linked to neurological problems. An affected child shows a loss of communication skills, has regression in nonverbal behaviors, and significant loss of previously-acquired skills. The condition is very similar to autistic disorder (autism).

Symptoms

     
  • Loss of social skills  
  • Loss of bowel and bladder control  
  • Loss of expressive or receptive language  
  • Loss of motor skills  
  • Lack of play  
  • Failure to develop peer relationships  
  • Impairment in nonverbal behaviors  
  • Delay or lack of spoken language  
  • Inability to start or sustain a conversation

Signs and tests

This disorder must be differentiated from both childhood schizophrenia and pervasive developmental disorder (autism).

The most important signs of childhood disintegrative disorder are loss of developmental milestones. The child tends to have normal development through age 3 to 4 and then over a few months undergoes a gradual loss of previously established abilities (e.g., language, motor, or social skills). Generally, the diagnosis is made with a loss of functioning in at least two areas described above.

Treatment
Treatment is the same for autistic disorder (autism) because of the similarity in the two disorders.

Expectations (prognosis)

Unfortunately, the prognosis for this disorder is limited. The loss of functioning will likely be permanent. However, to some degree, behaviors can be modified.

Calling your health care provider

Call your provider if your child has any delays in development or starts to lose developmental abilities.

Johns Hopkins patient information

Last revised: December 2, 2012
by Arthur A. Poghosian, M.D.

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