Childhood disintegrative disorder
Childhood disintegrative disorder
Childhood disintegrative disorder, also known as Heller's syndrome, is a condition in which children develop normally until ages 2 to 4, but then demonstrate a severe loss of social, communication and other skills.
Childhood disintegrative disorder is very much like autism. Both are among the group of disorders known as pervasive developmental disorders, or autism spectrum disorders. And both involve normal development followed by significant loss of language, social, play and motor skills. However, childhood disintegrative disorder typically occurs later than autism and involves a more dramatic loss of skills. In addition, childhood disintegrative disorder is far less common than autism.
Treatment for childhood disintegrative disorder involves a combination of medications, behavior therapy and other approaches.
Children with childhood disintegrative disorder typically show the following signs and symptoms:
Dramatic loss of previously acquired skills in two or more of the following areas:
Loss of developmental milestones may occur abruptly over the course of days to weeks or gradually over an extended period of time.
When to see a doctor
There's no known cause of childhood disintegrative disorder. There's likely a genetic basis for autism spectrum disorders. The theory is that an abnormal gene is switched on in the early stages of development, before birth, and that this gene affects other genes that coordinate a child's brain development. Environmental exposures, such as to a toxin or infection, may contribute to these effects.
It's also possible that an autoimmune response may play a role in the development of childhood disintegrative disorder. In an autoimmune response, your body's immune system perceives normal body components as foreign and attacks them.
Childhood disintegrative disorder often occurs along with other conditions, including:
It's unknown whether these conditions play a part in triggering childhood disintegrative disorder or share genetic or environmental risk factors.
Preparing for your appointment
Developmental screenings are usually performed at regular well-child visits. But even if you specifically want your child's social and cognitive development evaluated, you're likely to start by seeing your child's regular doctor. If after preliminary testing your child's doctor suspects a developmental disorder, you'll probably be referred to a team of childhood development specialists for more detailed developmental testing.
Here's some information to help you get ready for your initial appointment, and what to expect from your doctor.
What you can do
List your questions from most important to least important in case your time with your doctor runs out. For childhood disintegrative disorder, some basic questions to ask your doctor include:
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions anytime during your appointment.
What to expect from your doctor
What you can do in the meantime
Tests and diagnosis
Diagnostic criteria for childhood disintegrative disorder include:
Normal development for at least the first two years of life.
Significant loss of previously acquired or learned skills
Lack or loss of normal function
If your doctor sees signs or symptoms of a developmental disorder or delay, your child will be referred to one or more specialists for a formal evaluation and diagnosis. These may include a child psychologist, a child psychiatrist, a doctor who specializes in conditions of the brain and nervous system (neurologist), a pediatrician specializing in behavioral and developmental problems, a hearing specialist (audiologist), a speech therapist, a physical therapist and an occupational therapist.
These professionals may perform some or all of the following tests:
The results of your child's tests will allow your health care team to look for any underlying medical or neurological conditions that may be causing your child's signs and symptoms, rule out other conditions or diseases that may share the same features as childhood disintegrative disorder, and make an accurate diagnosis. An accurate diagnosis helps the team of health professionals develop the best treatment plan for your child.
Treatments and drugs
There's no cure for childhood disintegrative disorder. Treatment for the disorder is basically the same as for autism. Treatment options may include:
The outcome for children with childhood disintegrative disorder is usually worse than for children with autism. The loss of language, cognitive, social and self-care skills tends to be severe and unlikely to improve. Children with the disorder generally need lifelong support with the activities of daily living, and may eventually need residential care in a group home or long term care facility.
Some parents choose to supplement traditional medical treatments and behavior therapy with alternative therapies. The safety and effectiveness of alternative therapies for autism spectrum disorders have not been proven. However, some anecdotal reports indicate that some therapies may be helpful on a case-by-case basis. Scientific studies are ongoing.
Complementary therapies for disorders on the autism spectrum may include special diets, vitamin and mineral supplements, art therapy, music therapy, and sensory integration, a therapy technique used by occupational therapists to help children adapt to normal sensory experiences in the environment.
Always talk with your doctor before trying a new therapy. Your doctor can help you understand the risks and benefits, and alert you to any possible side effects or interactions with existing medications.
Coping and support
Child disintegrative disorder is a rare, serious condition. As a parent or family member affected by this disorder, you'll find it important to have support to cope with the condition. Here are some suggestions:
Last Updated: 2010-09-16
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