Anomalous sylvian fissure morphology in Williams syndrome

Bibliographic Collection: 
CARTA-Inspired Publication
Publication Type: Journal Article
Authors: Eckert, M. A.; Galaburda, A. M.; Karchemskiy, A.; Liang, A.; Thompson, P.; Dutton, R. A.; Lee, A. D.; Bellugi, U.; Korenberg, J. R.; Mills, D.; Rose, F. E.; Reiss, A. L.
Year of Publication: 2006
Journal: Neuroimage
Volume: 33
Edition: 2006/08/01
Number: 1
Pagination: 39-45
Date Published: Oct 15
Type of Article: Research Support, N.I.H., Extramural
Publication Language: eng
ISBN Number: 1053-8119 (Print)1053-81
Accession Number: 16876437
Keywords: Adult, Cerebral Cortex/*pathology, Computer-Assisted, Female, Fluorescence, Fractals, Functional Laterality/physiology, Humans, Image Processing, In Situ Hybridization, Magnetic Resonance Imaging, Male, Occipital Lobe/pathology, Temporal Lobe/pathology, W

The unusual sensitivity and attraction to auditory stimuli in people with Williams syndrome (WS) has been hypothesized to be the consequence of atypical development of brain regions surrounding the Sylvian fissure. Planum temporale surface area, which is determined in part by Sylvian fissure patterning, was examined in 42 WS and 40 control participants to determine if anomalous Sylvian fissure morphology is present in WS. WS participants had significantly reduced leftward asymmetry of the planum temporale compared to control participants, due to a significant expansion in the size of the right planum temporale. The increased right planum temporale size was largely due to WS participants (24%) who had a right hemisphere Sylvian fissure that coursed horizontally and failed to ascend into the parietal lobe. This sulcal pattern is unusual in the right hemisphere and is more commonly found in the left hemisphere of typically developing individuals. There were no control participants with this type of right hemisphere Sylvian fissure pattern. The right hemisphere Sylvian fissure sulcal patterns were also related to a measure of cortical complexity and the amount of right hemisphere occipital lobe volume, suggesting that intrinsic genetic influences leading to anomalous visual system development in WS have widespread influences on cortical morphology that are similar in manner to extrinsic embryonic visual system lesions.


Neuroimage. 2006 Oct 15;33(1):39-45. Epub 2006 Jul 28.

Alternate Journal: NeuroImage
Author Address:

Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, Charleston, 29425, USA.