Cognitive functioning in bilateral perisylvian polymicrogyria (BPP): clinical and radiological correlations
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Published on: August 12, 2019
An C Jansen1, Gabriel Leonard, Alexandre C Bastos
1Department of Neurology and Neurosurgery, McGill University, Montreal, Quebec, Canada.
Insights
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.
Abstract:
Bilateral perisylvian polymicrogyria (BPP) is a malformation of cortical development, frequently associated with severe dysarthria or anarthria. BPP patients are therefore often labeled as severely retarded, but a detailed neuropsychological profile has not been reported to date. In a series of 14 patients, we demonstrated that only a minority had extremely low intelligence, and that some aspects of cognitive function correlated with the extent of the cortical disorganization. Early age at seizure onset correlated positively with Performance IQ scores (P<0.05) and negatively with the extent of the lesion (P<0.01), reflecting that patients with more severe BPP are more likely to have early seizure onset, resulting in greater interference with ongoing cognitive development. Receptive and expressive language skills were found to be equally poor. Frontal lobe function and memory abilities were relatively well preserved, suggesting that the observed cognitive profiles were related, at least in part, to specific areas of cortical dysfunction and not only to global dysfunction.
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