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Williams syndrome and memory: a neuroanatomic and cognitive approach
Adriana Sampaio1, Nuno Sousa, Montse Férnandez
1School of Psychology, CIPsi, University of Minho, Braga, Portugal. adriana.sampaio@iep.uminho.pt
Journal of Autism and Developmental Disorders
|January 27, 2010
Summary
Williams Syndrome (WS) shows altered brain development, with a relative enlargement of the hippocampal formation (HF) and atypical asymmetry. Memory performance was not correlated with these brain structure differences.
Area of Science:
- Neuroscience
- Developmental Psychology
- Genetics
Background:
- Williams Syndrome (WS) is a genetic disorder characterized by cognitive and behavioral differences.
- Individuals with WS often exhibit a unique profile of memory system dissociation.
- Hippocampal formation (HF) integrity is crucial for memory function.
Purpose of the Study:
- To investigate hippocampal formation (HF) volumes and their association with memory performance in Williams Syndrome (WS).
- To compare HF volumes and asymmetry between individuals with WS and typically developing individuals.
Main Methods:
- Structural MRI scans were used to measure intracranial content and HF volumes.
- HF volumes were analyzed in absolute terms and after normalization.
- Neurocognitive assessments were conducted to evaluate memory measures.
Main Results:
- Individuals with WS had significantly reduced intracranial content but comparable absolute HF volumes to controls.
- Normalized analysis revealed a relative enlargement of the left HF in WS.
- Atypical HF asymmetry (absence of typical right > left pattern) was observed in WS.
- No significant correlations were found between HF volumetric data and memory measures in the WS group.
Conclusions:
- Relative HF enlargement and abnormal asymmetry in WS suggest atypical brain development.
- The dissociation in memory systems in WS may be linked to these observed structural brain differences.
- Further research is needed to elucidate the precise relationship between HF structure and memory function in WS.
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