Williams Syndrome and memory: a neuroanatomic and cognitive approach

Williams Syndrome (WS) is described as displaying
a dissociation within memory systems. As the
integrity of hippocampal formation (HF) is determinant for
memory performance, we examined HF volumes and its
association with memory measures in a group of WS and in
a typically development group. A significantly reduced
intracranial content was found in WS, despite no differences
were observed for HF absolute volumes between
groups. When volumes were normalized, left HF was
increased in WS. Moreover, a lack of the normal
right[left HF asymmetry was observed in WS. No
positive correlations were found between volumetric and
neurocognitive data in WS. In sum, a relative enlargement
of HF and atypical patterns of asymmetry suggest abnormal
brain development in WS
This research was supported by the grants PIC/IC/83290/2007 from Fundação para a Ciência e Tecnologia (Portugal). This study was also supported, in part, by grants from the National Institutes of Health (K05 MH 01110).