title: The fusiform face area is enlarged in Williams syndrome.
abstract: 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.
authors.affiliation: Department of Psychology, Stanford University, Stanford, California 94305, USA.
authors.forename: Golijeh
authors.initials: G
authors.lastname: Golarai
authors.forename: Sungjin
authors.initials: S
authors.lastname: Hong
authors.forename: Brian W
authors.initials: BW
authors.lastname: Haas
authors.forename: Albert M
authors.initials: AM
authors.lastname: Galaburda
authors.forename: Debra L
authors.initials: DL
authors.lastname: Mills
authors.forename: Ursula
authors.initials: U
authors.lastname: Bellugi
authors.forename: Kalanit
authors.initials: K
authors.lastname: Grill-Spector
authors.forename: Allan L
authors.initials: AL
authors.lastname: Reiss
mesh_terms: 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
references.citation: Magn Reson Med. 2001 Sep;46(3):515-22
references.pmid: 11550244
references.citation: Trends Cogn Sci. 2000 Jul;4(7):267-278
references.pmid: 10859571
references.citation: Nat Genet. 1993 Sep;5(1):11-6
references.pmid: 7693128
references.citation: Brain Cogn. 2008 Apr;66(3):290-7
references.pmid: 17950967
references.citation: J Neurosci. 2004 May 26;24(21):5009-15
references.pmid: 15163693
references.citation: Nature. 2003 Jul 10;424(6945):157-64
references.pmid: 12853948
references.citation: J Intellect Disabil Res. 2009 Feb;53(2):169-81
references.pmid: 19192099
references.citation: J Neurosci. 1997 Jun 1;17(11):4302-11
references.pmid: 9151747
references.citation: Proc Natl Acad Sci U S A. 1994 Aug 30;91(18):8690-4
references.pmid: 8078943
references.citation: Genes Brain Behav. 2008 Mar;7(2):224-34
references.pmid: 17680805
references.citation: Vision Res. 2001;41(10-11):1409-22
references.pmid: 11322983
references.citation: J Cogn Neurosci. 2000;12 Suppl 1:7-29
references.pmid: 10953231
references.citation: Q J Exp Psychol (Hove). 2009 Jan;62(1):189-203
references.pmid: 18609381
references.citation: Arch Neurol. 2001 Feb;58(2):283-7
references.pmid: 11176967
references.citation: J Child Psychol Psychiatry. 2004 Oct;45(7):1258-74
references.pmid: 15335346
references.citation: Dev Med Child Neurol. 2007 Jun;49(6):433-8
references.pmid: 17518929
references.citation: Neuron. 2004 Sep 2;43(5):623-31
references.pmid: 15339645
references.citation: Neurology. 2004 Jun 8;62(11):2070-6
references.pmid: 15184616
references.citation: Dev Psychopathol. 2008 Winter;20(1):1-35
references.pmid: 18211726
references.citation: Hippocampus. 2006;16(7):604-16
references.pmid: 16770797
references.citation: Philos Trans R Soc Lond B Biol Sci. 2003 Mar 29;358(1431):435-45
references.pmid: 12689371
references.citation: J Neurosci. 2005 Apr 20;25(16):4146-58
references.pmid: 15843618
references.citation: J Neurosci. 2009 Jan 28;29(4):1132-9
references.pmid: 19176822
references.citation: Nat Neurosci. 2005 Aug;8(8):991-3
references.pmid: 16007084
references.citation: Arch Neurol. 1995 Feb;52(2):209-12
references.pmid: 7848137
references.citation: Brain Res Cogn Brain Res. 2004 Jun;20(1):37-45
references.pmid: 15130587
references.citation: Neuron. 1998 Oct;21(4):753-9
references.pmid: 9808462
references.citation: Cereb Cortex. 2008 Oct;18(10):2402-9
references.pmid: 18308711
references.citation: J Neurosci. 1998 Mar 15;18(6):2188-99
references.pmid: 9482803
references.citation: J Cogn Neurosci. 2000;12 Suppl 1:89-107
references.pmid: 10953236
references.citation: J Cogn Neurosci. 2000;12 Suppl 1:47-64
references.pmid: 10953233
references.citation: Rev Neurol. 2003 Feb;36 Suppl 1:S132-7
references.pmid: 12599114
references.citation: Neuroimage. 2007 Sep 1;37(3):966-73
references.pmid: 17616403
references.citation: J Cogn Neurosci. 2000;12 Suppl 1:30-46
references.pmid: 10953232
references.citation: Neuropsychologia. 2007 Sep 20;45(12):2839-49
references.pmid: 17597166
references.citation: Nat Neurosci. 2004 May;7(5):555-62
references.pmid: 15077112
references.citation: J Autism Dev Disord. 2009 Mar;39(3):421-31
references.pmid: 18787936
references.citation: J Cogn Neurosci. 2002 Nov 15;14(8):1264-74
references.pmid: 12495531
references.citation: Neuroimage. 2006 Sep;32(3):1001-7
references.pmid: 16806978
references.citation: Cold Spring Harb Symp Quant Biol. 1996;61:1-8
references.pmid: 9246429
references.citation: Cogn Neuropsychiatry. 2008 Jan;13(1):47-58
references.pmid: 18092225
references.citation: Nat Neurosci. 2001 Aug;4(8):845-50
references.pmid: 11477432
references.citation: Nat Neurosci. 2007 Apr;10(4):512-22
references.pmid: 17351637
references.citation: Trends Cogn Sci. 2008 Apr;12(4):152-62
references.pmid: 18359267
references.citation: Soc Cogn Affect Neurosci. 2006 Dec;1(3):175-82
references.pmid: 18985104
references.citation: Cereb Cortex. 2007 Mar;17(3):513-21
references.pmid: 16581980
references.citation: Neuropsychol Rev. 2006 Jun;16(2):87-94
references.pmid: 16967345
references.citation: Perspect Psychol Sci. 2009 May;4(3):274-90
references.pmid: 26158964
references.citation: Nat Neurosci. 2007 Jan;10(1):3-4
references.pmid: 17189940
references.citation: Brain Res Cogn Brain Res. 2001 Jan;10(3):355-64
references.pmid: 11167061
references.citation: Soc Cogn Affect Neurosci. 2009 Sep;4(3):278-85
references.pmid: 19633063
references.citation: Neurology. 2004 Apr 13;62(7):1219-20
references.pmid: 15079032