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Rett Disorder & Childhood Disintegrative Disorder

© Sharon Gillson

Pervasive Developmental Disorders (PDD) is a term used for a catagory of specific disorders. These disorders are Autistic Disorder (or Autism), Rett Disorder, Childhood Disintegrative Disorder, Asperger Disorder, and Pervasive Developmental Disorder Not Otherwise Specified (PDDNOS).

Articles concerning Autism can be found here, and Asperger Disorder has been discussed here. This article will present information on Rett Syndrome and Childhood Disintegrative Disorder.

RETT SYNDROME

In 1966, Rett Syndrome was first recognized by Andreas Rett, and is a neurological disorder that affects primarily females. The prevalence of Rett Syndrome is approximately 1 in 10,000 to 15,000 births.

Children with Rett Syndrome will often display autistic-like behaviors. These behaviors include repetitive hand movements, body rocking, prolonged toe walking, and sleep problems.

In most cases of those with Rett Syndrome, there is a deterioration in cognition, social, behavioral and motor skills throughout their lifetime.

Typical characteristics include:

• Normal development until 1/2 to 1 1/2 years
• Shakiness of the torso, and possibly the limbs
• Unsteady, stiff-legged gait
• Seizures (approximately 80% have epilepsy)
• Breathing difficulties (hyperventilation, apnea, air swallowing)
• Retarded growth and small head
• Functioning level is usually between severely and profoundly mentally retarded
• Hypoactivity

Diagnostic Criteria from DSM-IV --- American Psychiatric Association: 299.80 Rett's Disorder

(A) All of the following:
1. apparently normal prenatal and perinatal development
2. apparently normal psychomotor development through the first 5 months after birth
3. normal head circumference at birth
(B)Onset of all of the following after the period of normal development:
1. deceleration of head growth between ages 5 and 48 months
2. loss of previously acquired purposeful hand skills between ages 5 and 30 months with the subsequent development of stereotyped hand movements (e.g., hand-wringing or hand washing)
3. loss of social engagement early in the course (although often social interaction develops later)
4. appearance of poorly coordinated gait or trunk movements
5. severely impaired expressive and receptive language development with severe psychomotor retardation

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CHILDHOOD DISINTEGRATIVE DISORDER

Childhood Disintegrative Disorder is a condition that occurs in 3 and 4 year old children. After having reached a normal range of functioning, the disorder results in a deterioration of intellectual, social, and language functioning in these children over a period of several months.

The cause of this disorder has been linked to neurological conditions. A couple of possibilities listed are seizure disorder and tuberous sclerosis. Children with this disorder have a loss of communication skills, regression in nonverbal behaviors, and significant loss of previously acquired skills. This condition is very similar to autistic disorder (autism).

Symptoms:

• loss of social skills
• loss of bowel and bladder control