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WIREs Cogn Sci
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Cognition in Down syndrome: a developmental cognitive neuroscience perspective

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Abstract Down syndrome (DS) is the most common genetic form of intellectual disability. DS results in a characteristic profile of cognitive and neurological dysfunction. The predominant theory of the pattern of neural deficits in this syndrome suggests that DS affects ‘late‐developing’ neural systems, including the function of the prefrontal cortex and hippocampus. In order to evaluate the validity of this theory, in this review, I highlight data addressing the neurological and cognitive phenotype in DS across development. In particular, I address the evidence suggesting that DS may impact late‐developing neural systems and end with the conclusion that some cognitive difficulties in DS must result from poor communication between late‐developing regions. Analogous to recent theories of cognitive processing in autism, cognitive deficits in DS may be substantially impacted by less efficient interregional communication. Finally, I discuss some ways in which understanding the impact of altered neurodevelopment in DS has the potential to inform our understanding of species‐typical trajectories of cognitive development. WIREs Cogn Sci 2013, 4:307–317. doi: 10.1002/wcs.1221 This article is categorized under: Psychology > Brain Function and Dysfunction

Patterns of search for a hidden object (located at ‘X’) in a rhombic environment in Down syndrome (DS). In the condition of a 10‐second delay with no disorientation, children with DS (n = 10) were able to find a hidden object with 92% accuracy. When disoriented, children with DS were unable to use geometric information (either alone or in conjunction with a landmark). These findings suggest specific difficulty in utilizing the spatial environment to guide search behavior in DS.

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