Brain morphology in children with 47, XYY syndrome: a voxel- and surface-based morphometric study.

The neurocognitive and behavioral profile of individuals with 47,XYY is increasingly documented; however, very little is known about the effect of a supernumerary Y-chromosome on brain development. Establishing the neural phenotype associated with 47,XYY may prove valuable in clarifying the role of Y-chromosome gene dosage effects, a potential factor in several neuropsychiatric disorders that show a prevalence bias toward males, including autism spectrum disorders. Here, we investigated brain structure in 10 young boys with 47,XYY and 10 age-matched healthy controls by combining voxel-based morphometry (VBM) and surface-based morphometry (SBM). The VBM results show the existence of altered gray matter volume (GMV) in the insular and parietal regions of 47,XYY relative to controls, changes that were paralleled by extensive modifications in white matter (WM) bilaterally in the frontal and superior parietal lobes. The SBM analyses corroborated these findings and revealed the presence of abnormal surface area and cortical thinning in regions with abnormal GMV and WMV. Overall, these preliminary results demonstrate a significant impact of a supernumerary Y-chromosome on brain development, provide a neural basis for the motor, speech and behavior regulation difficulties associated with 47,XYY and may relate to sexual dimorphism in these areas

Written language production disorders: historical and recent perspectives

Written language production is often the least examined neuropsychological function, yet it provides a sensitive and subtle sign to a variety of different behavioral disorders. The dissociation between written and spoken language and reading and writing first came to clinical prominence in the nineteenth century, with respect to ideas about localization of function. Twentieth century aphasiology research focused primarily on patients with unifocal lesions from cerebrovascular accidents, which have provided insight into the various levels of processing involved in the cognitively complex task of producing written language. Recent investigations have provided a broader perspective on writing impairments in a variety of disorders, including progressive and diffuse brain disorders, and functional brain imaging techniques have been used to study the underlying processes in healthy individuals