Social–emotional processing in nonverbal individuals with Angelman syndrome: evidence from brain responses to known and novel names

Abstract

Background The combination of intellectual, communicative and motor deficits limits the use of standardised behavioural assessments in individuals with Angelman syndrome (AS). The current study aimed to objectively evaluate the extent of social–emotional processing in AS using auditory event‐related potentials (ERPs) during passive exposure to spoken stimuli. Methods Auditory ERP responses were recorded in 13 nonverbal individuals with the deletion subtype of AS, age 4–45 years, during the name recognition paradigm, in which their own names and names of close others (relative or friend) were presented among novel names. No behavioural responses were required. Results Contrary to findings in typical children and adults, there was no significant evidence of differential neural response to known vs. novel names in participants with AS. Nevertheless, greater amplitude differences between known and unknown names demonstrated the predicted association with better interpersonal relationships and receptive communication abilities. Conclusions These findings indicate good tolerability of ERP procedures (85% success rate). The lack of own name differentiation is consistent with increased incidence of the autism‐related symptoms in AS. Strong associations between the caregiver reports of adaptive functioning and neural indices of known name recognition support the utility of brain‐based measures for objectively evaluating cognitive and affective processes in nonverbal persons with neurodevelopmental disorders.

Electrophysiological and Behavioral Evidence for Hyper- and Hyposensitivity in Rare Genetic Syndromes Associated with Autism

“…Taken together, the increased phasic inhibition might be related to attenuated early ERP components, while lower inhibition in the frontal cortex might cause the quicker and smaller attention-related ERPs. ↑ ∆ power [114][115][116][117] in patients with deletion: ↓ β power [114] ↑ θ power [114] ↑ amplitude 200-500 ms for repeated non-words was associated with better communication skills [118,119] n/a in patients with deletion: ↑ N1m amplitude [120] ↓ P1m amplitude [120] Prolonged N1m, P1m component [120] PMS 63%, between 2-6 y.o. [133,134] ↓ γ power [140] ↓ β power [140] ↓ α power [135,138,139] ↓ 40Hz ASSR [142] ↓ P50, P2 amplitude [148,149] ↑ P2 habituation [148] ↑ N250 latency [150] ↓ P60-N75, N75-P100 amplitude [140] n/a RS 60-80%, between 2-4 y.o.…”

“…ERPs findings in AS are rather sparse. Key and colleagues investigated ERPs in response to words in non-verbal AS patients [118,119]. The authors found that a larger ERP amplitude of 200-500 ms component for repeated nonwords compared to novel ones were associated with caregiver reports of more adaptive communication skills.…”

Searching for a “Brain Signature” of Neurodevelopmental Disorders: Event-Related Potentials and the Quest for Biomarkers of Cognition

“…The different patterns of ERP responses to own name in these clinical groups help elucidate the potential role of specific genetic mechanisms in cognitive performance. Similarly, persons with Angelman syndrome showed no significant evidence of preferential processing of their own name 54 but were able to form a memory trace for novel nonwords presented repeatedly among constantly changing distractors, 55 suggesting functional auditory short-term learning mechanisms but possibly altered long-term memory and/or affective processing. In both studies, more typical ERP responses to own name and repeated nonwords were associated with caregiver reports of more adaptive social and communicative functioning.…”