Anomalous brain activation during face and gaze processing in Williams syndrome.
OBJECTIVE
To investigate the discrete neural systems that underlie relatively preserved face processing skills in Williams syndrome (WS).


METHODS
The authors compared face and eye-gaze direction processing abilities in 11 clinically and genetically diagnosed WS subjects with 11 healthy age- and sex-matched controls, using functional MRI (fMRI).


RESULTS
Compared to controls, WS subjects showed a strong trend toward being less accurate in determining the direction of gaze and had significantly longer response latencies. Significant increases in activation were observed in the right fusiform gyrus (FuG) and several frontal and temporal regions for the WS group. By comparison, controls showed activation in the bilateral FuG, occipital, and temporal lobes. Between-group analysis showed WS subjects to have more extensive activation in the right inferior, superior, and medial frontal gyri, anterior cingulate, and several subcortical regions encompassing the anterior thalamus and caudate. Conversely, controls had greater activation in the primary and secondary visual cortices.


CONCLUSIONS
The observed patterns of activation in WS subjects suggest a preservation of neural functioning within frontal and temporal regions, presumably resulting from task difficulty or compensatory mechanisms. Persons with WS may possess impairments in visual cortical regions, possibly disrupting global-coherence and visuospatial aspects of face and gaze processing.
Mobbs
D
D
Department of Psychiatry & Behavioral Sciences, Stanford University School of Medicine, CA 94305-5719, USA.
Garrett
A S
AS
Menon
V
V
Rose
F E
FE
Bellugi
U
U
Reiss
A L
AL
D000328:Adult; D000679:Amygdala / Q000503:physiopathology; D001288:Attention; D001921:Brain / Q000503:physiopathology*; D016022:Case-Control Studies; D005123:Eye; D005145:Face; D005260:Female; D005625:Frontal Lobe / Q000503:physiopathology; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D011930:Reaction Time; D013702:Temporal Lobe / Q000503:physiopathology; D014793:Visual Cortex / Q000503:physiopathology; D014796:Visual Perception / Q000502:physiology*; D018980:Williams Syndrome / Q000503:physiopathology* / Q000523:psychology