The Rett Syndrome Association of Ireland

Rett Syndrome is often described as having four main stages or phases. When considering these stages it’s very important to remember some things:

  • these stages are only very broad categorisations
  • transition from one stage to another is subtle and often virtually invisible
  • there can be enormous individual variation

Stage One is sometimes called Early Onset. This is described as been between six and 18 months and is most often only recognised in hindsight. The signs may be very subtle – a Rett person might be slower reaching developmental milestones, maybe have a tendency to low muscle tone, perhaps little interest in engaging with toys – and hard to identify.

Stage Two is described as beginning anywhere between 12 months and 4 years. It can last weeks or months. It’s characterised by the loss of skills that had previously been acquired. Finger and hand skills like pointing and gripping can be damaged, mobility can be affected and language skills lost. This is when the stereotypical hand gestures – wringing, hand-mouthing, tapping – begin to appear. Breathing irregularities can be observed and there can be feeding issues too. This phase is sometimes called the Regression phase.

Stage Three is often referred to as the Plateau. This is usually a stable period. Regression is over and learning can take place – though slowly. Medical problems like Scoliosis and Epilepsy may develop, Apraxia and motor problems continue. There maybe improvement in interaction with the world, attention span and communication skills. Many people remain in the Plateau period for their lifetimes.

Stage Four may occur with some but for many it never happens. It can present as increased motor problems – spasticity, rigidity, reduced movement – but there is no loss at a cognitive level.

Again, it has to be stressed that there is enormous individual variation from person to person. It some, these stages can be seen quite clearly, in others they are not obvious at all. They are just a general guide to the progress of the condition.