What are the Stages of Rett Syndrome?

There are four stages of Rett syndrome:

Stage I

Stage I, called early onset stagnation period usually begins when the child is between six and 18 months old. This stage is often overlooked because, in this early part of the child’s life, symptoms of the disorder may not be very noticeable to the parents or the child’s doctor.

During this stage, the infant may begin to have less eye contact and not be as interested in her toys. Infants may demand less attention or be irritable and restless. There may be delays in skills like sitting and crawling. Hand wringing and decreasing head growth may occur, but not enough to draw attention. This stage usually lasts for a few months but can persist for more than a year.

Stage II

Stage II, or the rapid development regression period, usually begins between ages one and four and may last for weeks or months. Symptoms may appear slowly or rapidly. During this stage hand skills and expressive language can be lost.

Certain hand movements that are typical of Rett syndrome, such as wringing, washing, clapping, or tapping, as well as repeatedly moving the hands to the mouth may be seen. Hands are sometimes clasped behind the back or held at the sides, with random touching, grasping and releasing. The movements persist while the child is awake but disappear during sleep.

Breathing irregularities such as episodes of apnea (breathe holding) and hyperventilation (over breathing) may occur, although breathing is usually normal during sleep. Some girls also display symptoms that are like autism – such as loss of social interaction and communication. General irritability and sleep irregularities may be seen. The child may have an unsteady walking gait and trouble starting motor movements. Some children may develop seizures.

Decline of head growth is noted in most children at this point.

Stage III

Stage III, also called the pseudo stationary stage, usually begins between ages two and 10 and can last for years. Apraxia (being unable to perform complex movements) and seizures occur often during this stage. Impairments in hand function and hand stereopathies become more evident in this stage.

However, there may also be improvement in the child’s behavior, such as less irritability, crying and autistic-like features. The child may make better eye contact and use gazes to communicate (so called eye pointing behavior). A person in Stage III may show more interest in her surroundings, and her alertness, attention span and communication skills may improve. Many remain in this stage for most of their lives. Girls and women in this stage can learn about new people, things and situations.

Stage IV

Stage IV, the last stage, is called the late motor deterioration stage, and can last for years or decades. This stage begins when the ability to ambulate ceases. Muscle weakness, rigidity (stiffness), spasticity (uncontrolled muscle activity), dystonia (increased muscle tone with abnormal posturing of extremity or trunk) and scoliosis (curvature of the spine) are other major features of this stage. Hand stereopathies typically become simpler and less intense. However, general eye contact and communication remain intact in stage IV and are important to recognize and utilize for participation.

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Want to learn more about how Rett syndrome is diagnosed?

International Rett Syndrome Foundation