Relative sparing of primary auditory cortex in Williams Syndrome

Bibliographic Collection: 
CARTA-Inspired Publication
Publication Type: Journal Article
Authors: Holinger, D. P.; Bellugi, U.; Mills, D. L.; Korenberg, J. R.; Reiss, A. L.; Sherman, G. F.; Galaburda, A. M.
Year of Publication: 2005
Journal: Brain Res
Volume: 1037
Edition: 2005/03/22
Number: 1-2
Pagination: 35-42
Date Published: Mar 10
Type of Article: Research Support, N.I.H., ExtramuralResearch Support, Non-U.S. Gov'tResearch Support, U.S. Gov't, P.H.S.
Publication Language: eng
ISBN Number: 0006-8993 (Print)0006-89
Accession Number: 15777750
Keywords: Adult, Auditory Cortex/*pathology, Cell Count, Cell Size, Computer-Assisted, Female, Functional Laterality/physiology, Humans, Image Processing, Male, Middle Aged, Neurons/pathology/ultrastructure, Williams Syndrome/*pathology

Williams Syndrome (WS) is a neurodevelopment disorder associated with a hemizygous deletion on chromosome 7. WS is characterized with mental retardation, severe visual-spatial deficits, relative language preservation, and excellent facial recognition. Distinctive auditory features include musical ability, heightened sound sensitivity, and specific patterns of auditory evoked potentials. These features have led to the hypothesis that the dorsal forebrain is more affected than the ventral. Previously, we reported primary visual area 17 abnormalities in rostral striate cortex, a region contributing to the dorsal visual pathway. Based on the dorsal-ventral hypothesis, and language and auditory findings, we predicted a more normal histometric picture in auditory area 41. We used an optical dissector method to measure neurons in layers II-VI of area 41 in right and left hemispheres of the same 3 WS and 3 control brains used in the area 17 study. There was a hemisphere by diagnosis interaction in cell packing density (CPD) in layer IV and in cell size in layer III between WS and control brains. Post hoc analysis disclosed in control brains, but not WS, a layer IV left > right asymmetry in CPD, and a layer III left < right asymmetry in cell size. WS brains showed more large neurons bilaterally in layer II and in left layer VI. Histometric alterations in area 41 were less widespread than rostral visual cortex. Also, there was less asymmetry in the WS brain. We interpret layers II and VI differences as reflecting increased limbic connectivity in primary auditory cortex of WS.


Brain Res. 2005 Mar 10;1037(1-2):35-42.

Alternate Journal: Brain research
Author Address:

Division of Behavioral Neurology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02215, USA.