Williams syndrome and memory: a neuroanatomic and cognitive approach.
In Williams syndrome the hippocampus loses its normal right-side bulge, but this shape shift does not predict how well someone remembers.
01Research in Context
What this study did
The team scanned the brains of people with Williams syndrome. They measured the size of the hippocampus, a part we link to memory.
They compared these sizes to scans from people without the syndrome. Then they checked if bigger or smaller sizes matched memory test scores.
What they found
The hippocampus was not larger or smaller overall. Yet the left side was bigger than usual and the normal right-side lead was gone.
Surprise: these size changes did not track with how well anyone did on memory tasks. Structure and everyday memory did not line up.
How this fits with other research
Capitão et al. (2011) used the same MRI method one year later. They saw no absolute size gap in the amygdala, but once overall brain size was counted the amygdala looked relatively large. Together the papers show Williams brains shift several regions only when you adjust for their smaller total brain volume.
Lanfranchi et al. (2015) tested memory with puzzles instead of scanners. Their group struggled with spatial-simultaneous working memory but not with simple sequential lists. The 2010 scan study helps explain why: the hardware looks odd, yet the real-world gap shows up only on certain memory games.
Morris (2008) also found odd spatial bias in location memory two years earlier. That behavioral quirk now pairs with the odd hippocampal shape found here, giving a fuller picture of why directions and layouts feel harder for these learners.
Why it matters
For your next Williams syndrome learner, do not assume poor memory means the hippocampus is broken. Brain pictures may look different, yet daily performance can still be strong, especially on simple sequential tasks. Use short, step-by-step cues first, then add spatial-simultaneous supports like visual maps only if needed.
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02At a glance
03Original abstract
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.
Journal of autism and developmental disorders, 2010 · doi:10.1007/s10803-010-0940-z