Search

Williams syndrome (WS), a neurodevelopmental genetic disorder due to a microdeletion in chromosome 7, is described as displaying an intriguing socio-cognitive phenotype. Deficits in prosody production and comprehension have been consistently reported in behavioral studies. It remains, however, to be clarified the neurobiological processes underlying 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 semantic content of the utterance. A group of 12 participants (5 female and 7male), diagnosed with WS, with age range between 9 and 31 years, was compared with a group of typically developing participants, individually matched for chronological age, gender and laterality. After inspection of EEG artifacts, data from 9 participants with WS and 10 controls were included in ERP analyses. Participants were presented with neutral, positive and negative sentences, in two conditions: (1) with intelligible semantic and syntactic information; (2) with unintelligible semantic and syntactic information (‘pure prosody’ condition). They were asked to decide which emotion was underlying the auditory sentence. Atypical event-related potentials (ERP) components were related with prosodic processing (N100, P200, N300) in WS. In particular, reduced N100 was observed for prosody sentences with semantic content; more positive P200 for sentences with semantic content, in particular for happy and angry intonations; and reduced N300 for both types of sentence conditions. These findings suggest abnormalities in early auditory processing, indicating a bottomup contribution to the impairment in emotional prosody processing and comprehension. Also, at least for N100 and P200, they suggest the top-down contributions of semantic processes in the sensory processing of speech. This study showed, for the first time, that abnormalities in ERP measures of early auditory processing in WS are also present during the processing of emotional vocal information. This may represent a physiological signature of underlying impaired on-line language and socio-emotional processing., This work was supported by a Doctoral Grant (SFRH/BD/35882/2007) awarded to APP, as well as by the grant PIC/IC/83290/2007, Fundação para a Ciência e a Tecnologia (FCT)

Williams syndrome (WS), a genetic neurodevelopmental disorder due to microdeletion in chromosome 7, has been described as a syndrome with an intriguing socio-cognitive phenotype. Cognitively, the relative preservation of language and face processing abilities coexists with severe deficits in visual-spatial tasks, as well as in tasks involving abstract reasoning. However, in spite of early claims of the independence of language from general cognition in WS, a detailed investigation of language subcomponents has demonstrated several abnormalities in lexical-semantic processing. Nonetheless, the neurobiological processes underlying language processing in Williams syndrome remain to be clarified. The aim of this study was to examine the electrophysiological correlates of semantic processing in WS, taking typical development as a reference. A group of 12 individuals diagnosed with Williams syndrome, with age range between 9 and 31 years, was compared with a group of typically developing participants, individually matched in chronological age, gender and handedness. Participants were presented with sentences that ended with words incongruent (50%) with the previous sentence context or with words judged to be its best completion (50%), and they were asked to decide if the sentence made sense or not. Results in WS suggest atypical sensory ERP components (N100 and P200), preserved N400 amplitude, and abnormal P600 in WS, with the latter being related to late integration and re-analysis processes. These results may represent a physiological signature of underlying impaired on-line language processing in this disorder, This research was supported by a Doctoral Grant (SFRH/BD/35882/2007) awarded to the first author, as well as by the grant PIC/IC/83290/2007, both from FCT (Fundação para a Cieˆncia e a Tecnologia), in Portugal.