Imitation in Angelman syndrome: the role of social engagement

Serena Micheletti,Valentina Foresti,Laura Ferrari,Nicole D’Adda,Jessica Galli,Caterina Strobio,Paola Martelli,Elisa Fazzi,Elisa Scarano,Patrizia Accorsi,Stefano Renzetti,“Imitation In Angelman” Study Group ,Micaela De Simone,Lucio Giordano,Giacomo Vivanti,Anna Alessandrini,Stefano Calza

doi:10.1038/s41598-020-72079-3

Serena Micheletti, Valentina Foresti + Show 15 more

Open Access

https://doi.org/10.1038/s41598-020-72079-3

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Journal: Scientific Reports	Publication Date: Oct 2, 2020
Citations: 8	License type: open-access

Affiliation: University of Brescia

Abstract

Individuals with Angelman syndrome (AS) are characterized by severe cognitive impairments alongside an enhanced drive for social engagement. As knowledge on imitation skills in this population is limited, we conducted the first controlled study of imitation in AS. We examined how 23 individuals with AS and 21 typically developing young children with similar mental age imitated novel actions in response to socially or non-socially engaging models, and in response to video-recorded versus live demonstrations of novel actions. Individuals with AS imitated as frequently and as accurately as typical young children in response to live demonstrations; but they imitated less frequently and less accurately in response to video-recorded demonstrations. Further, imitation was modulated by whether the demonstrator was socially engaging or emotionally neutral in the AS group, while this modulation was not present in the comparison group. Individuals with higher mental age imitated more frequently and more accurately across groups. Imitation performance in AS appears to be more modulated by the social context compared to typical infants and young children with similar mental age, possibly reflecting an enhanced drive for social engagement. A socially engaging instructional style might facilitate imitative learning in this population.

Highlights

Individuals with Angelman syndrome (AS) are characterized by severe cognitive impairments alongside an enhanced drive for social engagement
Actions were imitated by the Angelman syndrome group (ASG) in 60.65% of cases (95% IC 48.94– 72.36), with an accuracy level of 37.7% (95% IC 27.61–47.87)
When the two conditions were considered separately, the ASG imitated 73.9% of the actions in the playful condition, while the frequency of imitation in response to the neutral condition was 45.65%

Summary

Introduction

Individuals with Angelman syndrome (AS) are characterized by severe cognitive impairments alongside an enhanced drive for social engagement. Penner and colleagues[13] found that only one of seven adults with AS was able to jointly attend and act on objects with another person and to participate in a turn taking exchange Despite these challenges, children with AS manifest a high desire to communicate with others[14,15], and show an atypically elevated frequency of laughing and smiling behaviors, during early childhood and in the context of highly engaging social interactions[16]. The co-existence of social communication difficulties with a distinctive drive for social engagement in children with AS provides a puzzling phenotypic expression, with important implication for treatment and research on typical and atypical social communication development Research in this area remains limited, partly due to methodological challenges[18], including difficulties with designing evaluation measures and conducting objective assessments that are adapted to the presence of severe cognitive impairment, tremors and ataxia, short attention span, motivational limitations and lack of verbal skills. In both typical and atypical development, imitation is longitudinally associated with cognitive and language s kills[25,26,27,28,29], and concurrently associated with joint a ttention[30,31], affect sharing32–34, empathy[35,36], and the ability to c ooperate[37]

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