NEUROLOGY 2004;62:2070-2076
© 2004 American Academy of Neurology
Anomalous brain activation during face and gaze processing in Williams syndrome
D. Mobbs, BSc,
A. S. Garrett, PhD,
V. Menon, PhD,
F. E. Rose, PhD,
U. Bellugi, EdD and
A. L. Reiss, MD
From the Department of Psychiatry & Behavioral Sciences (D. Mobbs and Drs. Garrett, Menon, and Reiss), Program in Neuroscience (Drs. Menon and Reiss), Stanford Brain Research Institute (Drs. Menon and Reiss), Stanford University School of Medicine; and the Laboratory for Cognitive Neuroscience (Drs. Rose and Bellugi), The Salk Institute for Biological Studies, La Jolla, CA.
Address correspondence and reprint requests to Dr. Allan L. Reiss, Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, 401 Quarry Road, Stanford, CA 94305–5719; e-mail: reiss{at}stanford.edu
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.
Conclusion: 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.
Received July 17, 2003.
Accepted in final form February 2, 2004.
This article has been cited by other articles:
|
|
|
|
 
B. M. Paul, A. Z. Snyder, F. Haist, M. E. Raichle, U. Bellugi, and J. Stiles
Amygdala response to faces parallels social behavior in Williams syndrome
Soc Cogn Affect Neurosci,
September 1, 2009;
4(3):
278 - 285.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. K. Olsen, J. S. Kippenhan, S. Japee, P. Kohn, C. B. Mervis, Z. S. Saad, C. A. Morris, A. Meyer-Lindenberg, and K. F. Berman
Retinotopically defined primary visual cortex in Williams syndrome
Brain,
March 2, 2009;
(2009)
awn362v1.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. W. Haas, D. Mills, A. Yam, F. Hoeft, U. Bellugi, and A. Reiss
Genetic Influences on Sociability: Heightened Amygdala Reactivity and Event-Related Responses to Positive Social Stimuli in Williams Syndrome
J. Neurosci.,
January 28, 2009;
29(4):
1132 - 1139.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. Sarpal, B. R. Buchsbaum, P. D. Kohn, J. S. Kippenhan, C. B. Mervis, C. A. Morris, A. Meyer-Lindenberg, and K. F. Berman
A Genetic Model for Understanding Higher Order Visual Processing: Functional Interactions of the Ventral Visual Stream in Williams Syndrome
Cereb Cortex,
October 1, 2008;
18(10):
2402 - 2409.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
F. Hoeft, N. Barnea-Goraly, B. W. Haas, G. Golarai, D. Ng, D. Mills, J. Korenberg, U. Bellugi, A. Galaburda, and A. L. Reiss
More Is Not Always Better: Increased Fractional Anisotropy of Superior Longitudinal Fasciculus Associated with Poor Visuospatial Abilities in Williams Syndrome
J. Neurosci.,
October 31, 2007;
27(44):
11960 - 11965.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
H. Tager-Flusberg, D. P. Skwerer, and R. M. Joseph
Model syndromes for investigating social cognitive and affective neuroscience: a comparison of autism and Williams syndrome
Soc Cogn Affect Neurosci,
December 1, 2006;
1(3):
175 - 182.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. C. Van Essen, D. Dierker, A. Z. Snyder, M. E. Raichle, A. L. Reiss, and J. Korenberg
Symmetry of cortical folding abnormalities in Williams syndrome revealed by surface-based analyses.
J. Neurosci.,
May 17, 2006;
26(20):
5470 - 5483.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. Gothelf, N. Farber, E. Raveh, A. Apter, and J. Attias
Hyperacusis in Williams syndrome: Characteristics and associated neuroaudiologic abnormalities
Neurology,
February 14, 2006;
66(3):
390 - 395.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. S. Kippenhan, R. K. Olsen, C. B. Mervis, C. A. Morris, P. Kohn, A. Meyer-Lindenberg, and K. F. Berman
Genetic Contributions to Human Gyrification: Sulcal Morphometry in Williams Syndrome
J. Neurosci.,
August 24, 2005;
25(34):
7840 - 7846.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. A. Eckert, D. Hu, S. Eliez, U. Bellugi, A. Galaburda, J. Korenberg, D. Mills, and A. L. Reiss
Evidence for superior parietal impairment in Williams syndrome
Neurology,
January 11, 2005;
64(1):
152 - 153.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. L. Reiss, M. A. Eckert, F. E. Rose, A. Karchemskiy, S. Kesler, M. Chang, M. F. Reynolds, H. Kwon, and A. Galaburda
An Experiment of Nature: Brain Anatomy Parallels Cognition and Behavior in Williams Syndrome
J. Neurosci.,
May 26, 2004;
24(21):
5009 - 5015.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
