Background: Understanding the social meaning of the eyes is crucial to normal development. We studied this ability in a neuro-developmental genetic disorder, Williams syndrome (WS) that, among other characteristics, has a distinctive cognitive profile with reported proficiency in language, face processing and social skills, but seriously impaired visuo-spatial and number skills.

Methods: Based on our earlier challenges to claim about intact face processing and good social cognition skills in WS, as well as from our work on early social cognition in WS infants, we ran two simple experiments, both with control conditions, to test the hypothesis that the ability to process eye gaze direction and facial emotions would be impaired in WS adults, compared to control groups of typically developing 4- and 6-year-old children and normal adults.

Results: We found that adolescents and adults with WS were seriously delayed in the detection of eye gaze direction as well as being specifically impaired at interpreting "sadness" and "anger", even compared to 4-year-old controls.

Conclusions: We speculate that the WS problems lie not in their difficulty to process eyes per se, but in their problems with interpreting the social meaning of the eyes, implicating dysfunction of the amygdala circuit. Finally, our results lead us to question a prevailing view that WS and autism are situated at opposite ends of the continuum with respect to social cognition.

Key words: Williams syndrome; autism; face/eye-emotion processing