III. Electrophysiological studies of face processing in Williams syndrome

Williams Syndrome (WMS) is a genetically based disorder characterized by pronounced variability in performance across different domains of cognitive functioning. This study examined brain activity linked to face-processing abilities, which are typically spared in individuals with WMS. Subjects watched photographic pairs of upright or inverted faces and indicated if the second face matched or did not match the first face. Results from a previous study with normal adults showed dramatic differences in the timing and distribution of ERP effects linked to recognition of upright and inverted faces. In normal adults, upright faces elicited ERP differences to matched vs. mismatched faces at approximately 320 msec (N320) after the onset of the second stimulus. This "N320" effect was largest over anterior regions of the right hemisphere. In contrast, the mismatch/match effect for inverted faces consisted of a large positive component between 400 and 1000 msec (P500) that was largest over parietal regions and was symmetrical. In contrast to normal adults, WMS subjects showed an N320-mismatch effect for both upright and inverted faces. Additionally, the WMS subjects did not display the N320 right-hemisphere asymmetry observed in the normal adults. WMS subjects also displayed an abnormally small negativity at 100 msec (N100) and an abnormally large negativity at 200 msec (N200) to both upright and inverted faces. This ERP pattern was observed in all subjects with WMS but was not observed in the normal controls. These results may be linked to increased attention to faces in subjects with WMS and might be specific to the disorder. These results were consistent with our ERP studies of language processing in WMS, which suggested abnormal cerebral specialization for spared cognitive functions in individuals with WMS.