RAPIDLY DESTRUCTIVE
Age: 1-4
Duration: Weeks to months
This stage has a rapid onset or may be more gradual, such as the beginning of meaningful hand movements and loss of language use.
Bliniquelized hand movements begin to appear, often initially manifesting as bringing the hand to the mouth. These movements most frequently involve bending or washing the hands in the midsection of the body, and are persistently performed while awake; these movements are absent when asleep. Other hand movements include clapping and lightly tapping the hand. The hands are sometimes tightly clasped behind the body or held in specific positions at the sides of the body, such as randomly touching, grasping, or releasing.
Respiratory irregularities may be observed in this stage, such as breath-holding and excessive breathing combined with empty coughing fits. Breathing is generally normal during sleep.
Some children exhibit autistic-like features while losing social communication and interaction.
General irritability and sleep disturbances may be observed.
They may exhibit noticeable trembling/rocking, especially when excited. Their gait is shaky and unsteady, and they may have difficulty with initiating motor movements.
When their head circumference shows a decline in the percentile chart (compared to children of the same age), a slowdown in head growth is usually observed between 3 months and 4 years of age.
PLATO
Age: Preschool to school years
Duration: Years
This stage occurs between the ages of 2 and 10, following the rapidly destructive stage.
Apraxia, motor problems, and seizures are very prominent.
Meanwhile, there is improvement in behavior, such as less irritability/irritability, less crying, and fewer autistic features. They show more interest in their surroundings, and their attention span, interest, and communication skills improve.
Many individuals with RS spend most of their lives in Stage III.
LATE MOTOR DETERIORATION
Age: When Stage III stops, ages 5-15-25-?
Duration: Over 10 years
This stage usually begins after age 10 and is characterized by decreased mobility. Some never walk, while others stop walking.
There is no decline in cognitive ability, communication, or fine motor skills. There may be a decrease in repetitive hand movements.
Scoliosis (S-shaped curvature of the spine) is the most noticeable feature.
Staring usually develops. Stiffness and dystonia (abnormally reduced muscle coordination or trunk positioning) are characteristic.
Puberty begins at the expected age in most girls.
Source: International Rett Syndrome Association – www.rettsyndrome.org