QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

HOME  |   SEARCH  |   ARCHIVE  |   SUBSCRIBE  |   CONTACT  |   HELP

The Journal of Neuroscience, May 26, 2004, 24(21):5009-5015; doi:10.1523/JNEUROSCI.5272-03.2004

This Article Full Text Full Text (PDF) Supplementary Figure Submit an eLetter Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via ISI Web of Science (43) Citing Articles via Google Scholar Google Scholar Articles by Reiss, A. L. Articles by Galaburda, A. Search for Related Content PubMed PubMed Citation Articles by Reiss, A. L. Articles by Galaburda, A.

 Previous Article  |  Next Article 

Neurobiology of Disease
An Experiment of Nature: Brain Anatomy Parallels Cognition and Behavior in Williams Syndrome

Allan L. Reiss,¹ Mark A. Eckert,¹ Fredric E. Rose,² Asya Karchemskiy,¹ Shelli Kesler,¹ Melody Chang,¹ Margaret F. Reynolds,¹ Hower Kwon,¹ and Al Galaburda³

¹Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, Stanford, California 94305, ²Laboratory for Cognitive Neuroscience, Salk Institute, La Jolla, California 92037 and ³Harvard Medical School, Department of Neurology, Boston, Massachusetts 02215

Williams syndrome (WS) is a neurogenetic-neurodevelopmental disorder characterized by a highly variable and enigmatic profile of cognitive and behavioral features. Relative to overall intellect, affected individuals demonstrate disproportionately severe visual-spatial deficits and enhanced emotionality and face processing. In this study, high-resolution magnetic resonance imaging data were collected from 43 individuals with WS and 40 age- and gender-matched healthy controls. Given the distinct cognitive-behavioral dissociations associated with this disorder, we hypothesized that neuroanatomical integrity in WS would be diminished most in regions comprising the visual-spatial system and most "preserved" or even augmented in regions involved in emotion and face processing. Both volumetric analysis and voxel-based morphometry were used to provide convergent approaches for detecting the hypothesized WS neuroanatomical profile. After adjusting for overall brain volume, participants with WS showed reduced thalamic and occipital lobe gray matter volumes and reduced gray matter density in subcortical and cortical regions comprising the human visual-spatial system compared with controls. The WS group also showed disproportionate increases in volume and gray matter density in several areas known to participate in emotion and face processing, including the amygdala, orbital and medial prefrontal cortices, anterior cingulate, insular cortex, and superior temporal gyrus. These findings point to specific neuroanatomical correlates for the unique topography of cognitive and behavioral features associated with this disorder.

Key words: emotion; neuron; imaging; morphometry; visual; Williams syndrome; neuroanatomy

---

Received Nov 28, 2003; revised March 31, 2004; accepted April 8, 2004.

This article has been cited by other articles:

				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, February 27, 2008; (2008) bhn004v1. [Abstract] [Full Text] [PDF]

				M. Behrmann, G. Avidan, F. Gao, and S. Black Structural Imaging Reveals Anatomical Alterations in Inferotemporal Cortex in Congenital Prosopagnosia Cereb Cortex, October 1, 2007; 17(10): 2354 - 2363. [Abstract] [Full Text] [PDF]

				S. Marenco, M. A. Siuta, J. S. Kippenhan, S. Grodofsky, W.-l. Chang, P. Kohn, C. B. Mervis, C. A. Morris, D. R. Weinberger, A. Meyer-Lindenberg, et al. Genetic contributions to white matter architecture revealed by diffusion tensor imaging in Williams syndrome PNAS, September 18, 2007; 104(38): 15117 - 15122. [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]

				R. S. Greenwood, L. A. Tupler, J. K. Whitt, A. Buu, C. B. Dombeck, A. G. Harp, M. E. Payne, J. D. Eastwood, K. R. R. Krishnan, and J. R. MacFall Brain Morphometry, T2-Weighted Hyperintensities, and IQ in Children With Neurofibromatosis Type 1 Arch Neurol, December 1, 2005; 62(12): 1904 - 1908. [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]

				P. M. Thompson, A. D. Lee, R. A. Dutton, J. A. Geaga, K. M. Hayashi, M. A. Eckert, U. Bellugi, A. M. Galaburda, J. R. Korenberg, D. L. Mills, et al. Abnormal Cortical Complexity and Thickness Profiles Mapped in Williams Syndrome J. Neurosci., April 20, 2005; 25(16): 4146 - 4158. [Abstract] [Full Text] [PDF]

				G. M. McAlonan, V. Cheung, C. Cheung, J. Suckling, G. Y. Lam, K. S. Tai, L. Yip, D. G. M. Murphy, and S. E. Chua Mapping the brain in autism. A voxel-based MRI study of volumetric differences and intercorrelations in autism Brain, February 1, 2005; 128(2): 268 - 276. [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]

Home  |   Search  |   Archive  |   Subscribe  |   Contact  |   Help