What are the Effects of Rett Syndrome? Insights into the Four Stages

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Who Suffers from Rett Syndrome?

Rett syndrome is a rare disorder that affects approximately one in 15,000 girls. When a diagnosis is presented, the first question parents usually ask is what are the effects of Rett syndrome?

The condition commonly follows a period of normal development and the symptoms appear over a period of time. The problems may become apparent from about five months of age but are often noticed between the ages of one and three. Perceptive doctors and parents may notice early signs in a baby but they are often not picked up until later.

How Does Rett Syndrome Affect a Person?

Rett syndrome is almost exclusive to girls and normally follows a progression of symptoms that worsen over a period of time. The symptoms and deterioration can be divided into four stages that affect the child in different ways.

Stage one symptoms appear between the age of six and eighteen months. They are often subtle and easily overlooked. The child may lose interest in toys and begin to make less eye contact with parents. Gross motor skills may be delayed and head growth decreases.

Stage two affects the child in more noticeable ways and the onset may be gradual or rapid. It usually begins between the ages of one and four and the child will lose spoken language and purposeful control of hands. The effects on the hands are characteristic of the syndrome and include movements such as wringing, clapping, tapping and touching the mouth as well as clasping the hands together. Autistic-like symptoms may appear during this stage with the loss of communication and social skills. Walking and motor control are impaired and head growth slows.

Stage three is called the plateau stage and usually begins between two and ten years of age. It affects the child’s motor skills, and seizures are common. Conversely, some sufferers may show improvement in communication skills, attention span and interest in surroundings. Many girls remain in this stage for the duration of their lives.

Stage four is the late motor deterioration stage and can last for years. The effects include decreased mobility due to scoliosis, muscle problems, rigidity, spasticity and abnormal posturing of limbs. The girl may lose her ability to walk, but hand control and communication skills generally remain unchanged or may even improve.

What are the Long-term Effects of Rett Syndrome

What are the effects of Rett syndrome is a question with varied answers. The condition progresses at different rates with different effects in each case. In the early stages, the child may display autistic-like behavior but the general long-term result will be reduced capacity of the body to perform motor movements.

Understanding the progression of the condition can help prepare parents in part for the future of their child. Girls with Rett syndrome often stabilize in stage three of the condition and may even improve in some ways. They generally live to their forties or fifties.


National Institute of Neurological Disorders and Stroke, https://www.ninds.nih.gov/disorders/rett/detail_rett.htm

National Institute of Child Health and Human Development, https://www.nichd.nih.gov/health/topics/rett_syndrome.cfm

Children with Autism, Michael D Powers, woodbine House Inc, 2000