Imitation in Angelman syndrome: the role of social engagement

Micheletti, Serena; Vivanti, Giacomo; Renzetti, Stefano; Martelli, Paola; Calza, Stefano; Fazzi, Elisa

doi:10.1038/s41598-020-72079-3

Cited by 7 publications

(7 citation statements)

References 55 publications

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“…Despite severe developmental delays in AS and Dup15q, all three groups exhibit the conventional behavioral traits associated with consciousness during wakefulness and diminished consciousness during NREM sleep. For instance, children with AS show the same level of social imitation of novel actions as NT children 14 , and children with AS who are nonverbal may communicate using gestures or alternative communication devices 15,16 . Nonetheless, the EEG phenotypes of these three groups are starkly different.…”

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confidence: 99%

Neural complexity is a common denominator of human consciousness across diverse regimes of cortical dynamics

et al. 2022

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What is the common denominator of consciousness across divergent regimes of cortical dynamics? Does consciousness show itself in decibels or in bits? To address these questions, we introduce a testbed for evaluating electroencephalogram (EEG) biomarkers of consciousness using dissociations between neural oscillations and consciousness caused by rare genetic disorders. Children with Angelman syndrome (AS) exhibit sleep-like neural dynamics during wakefulness. Conversely, children with duplication 15q11.2-13.1 syndrome (Dup15q) exhibit wake-like neural dynamics during non-rapid eye movement (NREM) sleep. To identify highly generalizable biomarkers of consciousness, we trained regularized logistic regression classifiers on EEG data from wakefulness and NREM sleep in children with AS using both entropy measures of neural complexity and spectral (i.e., neural oscillatory) EEG features. For each set of features, we then validated these classifiers using EEG from neurotypical (NT) children and abnormal EEGs from children with Dup15q. Our results show that the classification performance of entropy-based EEG biomarkers of conscious state is not upper-bounded by that of spectral EEG features, which are outperformed by entropy features. Entropy-based biomarkers of consciousness may thus be highly adaptable and should be investigated further in situations where spectral EEG features have shown limited success, such as detecting covert consciousness or anesthesia awareness.

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confidence: 99%

Neural complexity is a common denominator of human consciousness across diverse regimes of cortical dynamics

et al. 2022

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“…The eye-tracking technology was used to examine attentional patterns, in terms of fixations duration towards video-stimuli depicting a person performing an action displayed on a computer screen, in children with AS and age-matched typically-developing children, with the goal of addressing the following questions: (1)Do individuals with AS differ from mental age—matched children with typical development, in terms of proportion of time spent watching the two areas of interest (faces and actions) out of their total fixations duration to the screen? Based on the results of Micheletti et al 7 and previous literature on enhanced social drive in AS, we predicted that (a) both groups would display higher proportion of fixations duration (P-FD) towards the actor’s faces compared to her actions, and (b) the increased P-FD to the actor’s face versus her action would be significantly higher in participants with AS. (2)In participants with AS, is the P-FD towards faces and actions influenced by whether the actor in the video shows an emotionally engaging versus neutral affect?…”

Section: Introductionmentioning

confidence: 94%

“…(1)Do individuals with AS differ from mental age—matched children with typical development, in terms of proportion of time spent watching the two areas of interest (faces and actions) out of their total fixations duration to the screen? Based on the results of Micheletti et al 7 and previous literature on enhanced social drive in AS, we predicted that (a) both groups would display higher proportion of fixations duration (P-FD) towards the actor’s faces compared to her actions, and (b) the increased P-FD to the actor’s face versus her action would be significantly higher in participants with AS.…”

Section: Introductionmentioning

confidence: 94%

“…Recent research has shown a distinctive profile of imitation – an important dimension of social functioning – in children with AS, characterised by increased imitation in response to models showing an “emotionally playful” affect, compared to models displaying a neutral emotional expression 7 . Although in the same study typically developing children matched by developmental age were also showing enhanced imitation in response to emotional versus neutral models, the magnitude of the enhancement was higher in participants with AS.…”

Section: Introductionmentioning

confidence: 99%

“…The present study was designed to expand on the findings by Micheletti et al 7 , with the goal of examining social attention and the social-emotional modulation of attention in individuals with AS. The eye-tracking technology was used to examine attentional patterns, in terms of fixations duration towards video-stimuli depicting a person performing an action displayed on a computer screen, in children with AS and age-matched typically-developing children, with the goal of addressing the following questions: (1)Do individuals with AS differ from mental age—matched children with typical development, in terms of proportion of time spent watching the two areas of interest (faces and actions) out of their total fixations duration to the screen?…”

Section: Introductionmentioning

confidence: 99%

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Social attention and social-emotional modulation of attention in Angelman syndrome: an eye-tracking study

Micheletti

Vivanti²,

Renzetti³

et al. 2023

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Individuals with Angelman syndrome (AS) present with severe intellectual disability alongside a social phenotype characterised by social communication difficulties and an increased drive for social engagement. As the social phenotype in this condition is poorly understood, we examined patterns of social attention and social modulation of attention in AS. Twenty-four individuals with AS and twenty-one young children with similar mental age were shown videos featuring unfamiliar actors who performed simple actions across two conditions: a playful condition, in which the actor showed positive facial emotions, and a neutral condition, in which the actor showed a neutral facial expression. During the passive observation of the videos, participants’ proportion of time spent watching the two areas of interest (faces and actions) was examined using eye-tracking technology. We found that the playful condition elicited increased proportion of fixations duration to the actor’s face compared to the neutral condition similarly across groups. Additionally, the proportion of fixations duration to the action area was similar across groups in the two conditions. However, children with AS looked towards the actor’s face for a shorter duration compared to the comparison group across conditions. This pattern of similarities and differences provides novel insight on the complex social phenotype of children with AS.

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