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Cognitive functioning in bilateral perisylvian polymicrogyria (BPP): clinical and radiological correlations
An C Jansen1, Gabriel Leonard, Alexandre C Bastos
1Department of Neurology and Neurosurgery, McGill University, Montreal, Quebec, Canada.
Epilepsy & Behavior : E&B
|April 12, 2005
Summary
Bilateral perisylvian polymicrogyria (BPP) is a brain development disorder. Neuropsychological testing revealed varied intelligence levels, with some cognitive functions linked to cortical disorganization severity in BPP patients.
Area of Science:
- Neuroscience
- Developmental Biology
- Cognitive Psychology
Background:
- Bilateral perisylvian polymicrogyria (BPP) is a rare cortical malformation.
- BPP is often linked to severe speech impairments (dysarthria/anarthria).
- Previous studies have not detailed the neuropsychological profiles of BPP patients.
Purpose of the Study:
- To investigate the detailed neuropsychological profile of patients with Bilateral Perisylvian Polymicrogyria (BPP).
- To assess cognitive functions and their correlation with the extent of cortical malformation in BPP.
- To challenge the assumption of global intellectual disability in BPP.
Main Methods:
- Neuropsychological assessment of 14 patients diagnosed with BPP.
- Correlation analysis between cognitive scores and the extent of cortical disorganization.
- Analysis of factors like age at seizure onset and their impact on cognitive outcomes.
Main Results:
- Only a minority of BPP patients exhibited extremely low intelligence.
- Cognitive function correlated with the degree of cortical disorganization.
- Early seizure onset was linked to higher Performance IQ and less severe lesions.
- Receptive and expressive language skills were consistently poor.
- Frontal lobe functions and memory were relatively preserved.
Conclusions:
- BPP does not invariably lead to severe intellectual disability.
- Cognitive deficits in BPP are partly related to specific cortical dysfunction areas, not just global impairment.
- The extent of cortical malformation and seizure onset influence cognitive development in BPP.