Social behaviour and Social Cognition in Rubinstein-Taybi Syndrome
Funded by Cerebra and supported by the Rubinstein-Taybi Syndrome Support Group from 2007 –2010
One of the most frequently documented characteristics in Rubinstein-Taybu syndrome (RTS) is the ability to form social contacts despite cognitive delay (Hennekam, 2006). Reports have repeatedly described those with RTS as happy, loving, friendly individuals who love adult attention (Baxter & Beer, 1992; Hennekam, 2006; Padfield, et al. 1968; Rubinstein & Taybi, 1963; Stevens et al. 1990a). However, parents and carers of individuals with RTS have frequently reported concerns that the people they care for may be 'overfriendly' and show no concept of stranger danger. A growing body of research has highlighted the possibility that Theory of Mind (ToM) is central to a range of social activities. Evidence for such an assertion has come from research that has demonstrated that ToM concepts are exceptionally difficult for individuals with autism - who characteristically show a number of social impairments. Such findings highlight the likelihood that ToM abilites/deficits might also underpin the social behaviours found in other neurodevelopmental disorders and rare genetic syndromes.
We aimed to (i) develop a Theory of Mind assessment battery suitable for individuals with varying levels of intellectual disability, (ii) gather normative data from typically developing children on the newly developed battery and (iii) investigate Theory of Mind development in individuals with Rubinstein Taybi syndrome and assess how it differs from typical development.
Thirty individuals with RTS of varying ages and abilities were recruited into the study. The individuals included needed to have a confirmed diagnosis of RTS and were also required to be mobile. One hundred and forty typically developing individuals aged between nine months and three years also participated.
Each individual completed the Theory of Mind battery in one of four counterbalanced orders.
Progress to date
Our findings have shown that early developing social cognitive/ToM skills, including behaviours such as 'helping' and 'cooperation', are preserved in people with RTS relative to their mental age. More specifically, these skills require a ‘motivation for social contact’ and this seems to be strong in people with RTS. However, later developing Theory of Mind skills that require more complex reasoning about people’s intentions and thoughts are impaired relative to mental age. We think that impaired ToM ability taken together with a strong motivation for social contact may leave individuals - particularly older individuals with greater independence - vulnerable to social exploitation. Anecdotal reports suggest this is the case.
Primary contact: Laurie Powis