The Rett Syndrome Complex

Abstract

Communicative functions and their relationship with overall developmental level and autistic features were studied in eight young women with disorders in the Rett syndrome complex, three with the classical variant and five with variants with partially preserved/regained speech. The Vineland Adaptive Behaviour Scales, individual structured observation, and a specially designed structured interview covering early pre-linguistic and linguistic development, current receptive and expressive language and non-verbal communication skills, were used. Autistic features were evaluated in accordance with the DSM-IV. Low levels of communicative abilities and overall functioning were demonstrated. In most cases, the communicative abilities did not reach the level expected on the basis of overall development. Joint attention behaviours and expressions of communicative intent were rare. However, six of the eight subjects showed clear examples of social interaction abilities.‘Eye pointing’, as distinct from ‘eye communication’, was demonstrated only in a minority of the cases. The level of communicative function at developmental arrest did not predict later language ability. It is suggested that intervention should focus on developing further the joint attention behaviours, intentional communications and communicative functions spontaneously used by individuals with disorders in the Rett syndrome complex.