Cerebral Cortex Advance Access originally published online on March 28, 2004
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Cerebral Cortex August 2004; 14:840-850
© Oxford University Press 2004
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Brain Anatomy in Turner Syndrome: Evidence for Impaired Social and SpatialNumerical Networks
1 INSERM U 562, Service Hospitalier Frédéric Joliot, CEA/DSV, IFR 49, Orsay, France, 2 Unité de Neuro-Activation Fonctionnelle, Service Hospitalier Frédéric Joliot, CEA/DSV, IFR 49, Orsay, France, 3 INSERM ERM 0205, IFR 49, Service Hospitalier Frédéric Joliot, CEA/DSV, IFR 49, Orsay, France, 4 Service de Neurologie 1, Hôpital Pitié-Salpétrière, Paris, France
Analysis of brain structure in Turner syndrome (TS) provides the opportunity to identify the consequences of the loss of one X chromosome on brain anatomy and to characterize the neural bases underlying the specific cognitive profile of TS subjects which includes deficits in spatialnumerical processing and social cognition. Fourteen subjects with TS and fourteen controls were investigated using voxel-based analysis of high resolution anatomical and diffusion tensor images and using sulcal morphometry. The analysis of anatomical images provided evidence for macroscopical changes in cortical regions involved in social cognition such as the left superior temporal sulcus and orbito-frontal cortex and in a region involved in spatial and numerical cognition such as the right intraparietal sulcus. Diffusion tensor images showed a displacement of the greywhite matter interface of the left and right superior temporal sulcus and revealed bilateral microstuctural anomalies in the temporal white matter. The analysis of fiber orientation suggests specific alterations of fiber tracts connecting posterior to anterior temporal regions. Last, sulcal morphometry confirmed the anomalies of the left and right superior temporal sulci and of the right intraparietal sulcus. Our results thus provide converging evidence of regionally specific structural changes in TS that are highly consistent with the hallmark symptoms associated with TS.
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