Williams syndrome (WS), a neurodevelopmental genetic disorder due to a microdeletionin chromosome 7, is described as displaying an intriguing socio-cognitive phenotype.Deficits in prosody production and comprehension have been consistently reported inbehavioral studies. It remains, however, to be clarified the neurobiological processesunderlying prosody processing in WS.This study aimed at characterizing the electrophysiological response to neutral, happy,and angry prosody in WS, and examining if this response was dependent on the semanticcontent of the utterance. A group of 12 participants (5 female and 7male), diagnosed withWS, with age range between 9 and 31 years, was compared with a group of typicallydeveloping participants, individually matched for chronological age, gender and laterality.After inspection of EEG artifacts, data from 9 participants with WS and 10 controls wereincluded in ERP analyses.Participants were presented with neutral, positive and negative sentences, in twoconditions: (1) with intelligible semantic and syntactic information; (2) with unintelligiblesemantic and syntactic information (‘pure prosody’ condition). They were askedto decide which emotion was underlying the auditory sentence.Atypical event-related potentials (ERP) components were related with prosodicprocessing (N100, P200, N300) in WS. In particular, reduced N100 was observed forprosody sentences with semantic content; more positive P200 for sentences withsemantic content, in particular for happy and angry intonations; and reduced N300 forboth types of sentence conditions.These findings suggest abnormalities in early auditory processing, indicating a bottomupcontribution to the impairment in emotional prosody processing and comprehension.Also, at least for N100 and P200, they suggest the top-down contributions of semanticprocesses in the sensory processing of speech. This study showed, for the first time, thatabnormalities in ERP measures of early auditory processing in WS are also present duringthe processing of emotional vocal information. This may represent a physiologicalsignature of underlying impaired on-line language and socio-emotional processing.
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