The fusiform face area is enlarged in Williams syndrome.
Williams syndrome (WS) is a genetic condition characterized by atypical brain structure, cognitive deficits, and a life-long fascination with faces. Face recognition is relatively spared in WS, despite abnormalities in aspects of face processing and structural alterations in the fusiform gyrus, part of the ventral visual stream. Thus, face recognition in WS may be subserved by abnormal neural substrates in the ventral stream. To test this hypothesis, we used functional magnetic resonance imaging and examined the fusiform face area (FFA), which is implicated in face recognition in typically developed (TD) individuals, but its role in WS is not well understood. We found that the FFA was approximately two times larger among WS than TD participants (both absolutely and relative to the fusiform gyrus), despite apparently normal levels of face recognition performance on a Benton face recognition test. Thus, a larger FFA may play a role in face recognition proficiency among WS.
Golarai
Golijeh
G
Department of Psychology, Stanford University, Stanford, California 94305, USA.
Hong
Sungjin
S
Haas
Brian W
BW
Galaburda
Albert M
AM
Mills
Debra L
DL
Bellugi
Ursula
U
Grill-Spector
Kalanit
K
Reiss
Allan L
AL
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D005145:Face; D005260:Female; D006801:Humans; D007360:Intelligence; D007361:Intelligence Tests; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D010364:Pattern Recognition, Visual / Q000502:physiology*; D010775:Photic Stimulation; D021641:Recognition, Psychology / Q000502:physiology; D012815:Signal Processing, Computer-Assisted; D013702:Temporal Lobe / Q000503:physiopathology*; D018980:Williams Syndrome / Q000503:physiopathology*; D055815:Young Adult
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