The development of gesture and prelinguistic communication in Angelman syndrome

Effie Pearson, Lucy Wilde, Sotaro Kita, Emily Goodman, Joanna Moss, Olivia Massey, Mary Heald, Chris Oliver

Research output: Contribution to journalConference abstractpeer-review

Abstract

Background: Angelman syndrome (AS) is caused by deletion or inactivation of UBE3A. Minimal or absent speech is a core characteristic, and there is a suggested dissociation between speech and non‐verbal communication abilities, proposing an isolated speech production impairment in AS. Given that for both typical and clinical populations the emergence of prelinguistic communication, including gestures, can provide insight into concurrent and future language abilities, exploration of the development of prelinguistic communication in AS can ascertain to what extent developmental mechanisms underlie the absence of speech or whether it is related to the inactivation of UBE3A. Methods: Questionnaire data were collected on comprehension, speech production and gesture use for children with AS (N = 40, Mage = 9.58, SD = 3.98). Gesture use, criteria of intentionality, communicative complexity, verbal and non‐verbal communication were also assessed using behavioural coding (N = 47, Mage = 9.26, SD = 3.54). Results: A strong, positive correlation was found between the number of communicative gestures and comprehension scores in AS (rs = .725, p < .001), which was comparable to typically developing children of similar comprehension abilities (rs = .721, p < .001). Guttman scaling analysis showed that gestures and prelinguistic communication emerged in a reliable, ordered sequence (CR = .959), that converged with the sequence seen in typical development, except for the presence of verbalisations. Conclusions: Developmental mechanisms appear to underlie gesture use and prelinguistic communication in AS, suggestive of delay rather than impairment. In contrast, these mechanisms cannot account for the absence of speech, providing further evidence that this is an isolated impairment that could be related to the inactivation of UBE3A. These results have further implications for the use of standardised developmental assessments for individuals with AS, due to the reliance on spoken language, which may not accurately capture the developmental ability and communicative variability seen in AS.
Original languageEnglish
Pages (from-to)1084-1084
JournalJournal of Intellectual Disability Research
Volume63
Issue number9
DOIs
Publication statusPublished - 1 Sep 2019

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