Assessment & Research

Williams syndrome and memory: a neuroanatomic and cognitive approach.

Sampaio et al. (2010) · Journal of autism and developmental disorders 2010
★ The Verdict

In Williams syndrome the hippocampus loses its normal right-side bulge, but this shape shift does not predict how well someone remembers.

✓ Read this if BCBAs who assess or teach learners with Williams syndrome.
✗ Skip if Clinicians only serving autism or ADHD where WS is not on the caseload.

01Research in Context

01

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.

02

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.

03

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.

04

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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Start your session with a short sequential list task; add spatial maps only if the learner stalls.

02At a glance

Intervention
not applicable
Design
case control
Population
other, neurotypical
Finding
not reported

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