Cognition, communication and behaviour in individuals with Rett Syndrome

One of the main criteria for clinical diagnosis of Rett syndrome (RTT), as defined by Neul et al. (2010), is that of ‘partial or complete loss of acquired spoken language’ (p. 946). For many parents, the communication difficulties experienced by their child (at any age) are one of the greatest challenges – and one of the greatest frustrations – they face. Questions of how an individual understands and interacts with the world, how they can best be helped to express themselves, how they can be educated and enabled to participate in society in a meaningful way are of fundamental importance and are grounded in the phenomenologically overlapping areas of cognition, communication, and behavior. In this chapter, we will review the research to date and the current levels of knowledge and thinking with regard to cognition, communication, and behavior in individuals with RTT, including early development, later skills and deficits, and strategies for intervention and management