Tatton-Brown-Rahman syndrome: Cognitive and behavioural phenotypes

Lane, Chloe, Tatton-Brown, Katrina and Freeth, Megan (2020) Tatton-Brown-Rahman syndrome: Cognitive and behavioural phenotypes. Developmental Medicine & Child Neurology, 62 (8). pp. 993-998. ISSN 1469-8749

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The aim of this case series was to assess and characterize cognitive abilities, autistic traits, and adaptive behaviour in Tatton-Brown-Rahman syndrome (TBRS). The sample included 18 individuals with a clinical and genetic diagnosis of TBRS (11 males, seven females; mean age 17y 7mo, SD 9y 5mo, range 7y 2mo–33y 10mo). The British Ability Scales, Third Edition and the Autism Diagnostic Observation Schedule, Second Edition (ADOS-2) were administered to all participants. The Social Responsiveness Scale, Second Edition and the Vineland Adaptive Behaviour Scales, Third Edition were completed by a parent/caregiver. The majority of participants (n=15) had intellectual disability and General Conceptual Ability scores ranged from 39 to 76 (mean 53.17, SD 12.13). Participants displayed a profile of better verbal ability compared with non-verbal reasoning ability and spatial ability. Autistic traits were prevalent and eight participants scored above the cut-off on the ADOS-2, although symptoms were less pronounced in older individuals. Adaptive functioning was impaired but commensurate with intellectual ability. Overall, TBRS is associated with an uneven cognitive profile and a high prevalence of autistic traits. This has implications for identifying appropriate services and support that may be beneficial for individuals with TBRS.

Item Type: Article
Uncontrolled Keywords: Research & Development Unit
Subjects: Cognitive Processes, Theory of Mind > Cognitive Processes
Communication (incl. disorders of) > Communication
Department/People: Special Units
URI: https://repository.tavistockandportman.ac.uk/id/eprint/2642

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