Comparative study of cellular and synaptic abnormalities in brain tissue samples from pediatric tuberous sclerosis complex and cortical dysplasia type II
Open Access
- 1 July 2010
- Vol. 51 (s3), 160-165
- https://doi.org/10.1111/j.1528-1167.2010.02633.x
Abstract
Tuberous sclerosis complex (TSC) and severe cortical dysplasia (CD), or CD type II according to Palmini classification, share histopathologic similarities, specifically the presence of cytomegalic neurons and balloon cells. In this study we examined the morphologic and electrophysiologic properties of cells in cortical tissue samples from pediatric patients with TSC and CD type II who underwent surgery for pharmacoresistant epilepsy. Normal‐appearing pyramidal neurons from TSC and CD type II cases had similar passive membrane properties. However, the frequency of excitatory postsynaptic currents (EPSCs) was higher in neurons from TSC compared to severe CD cases, particularly the frequency of medium‐ and large‐amplitude synaptic events. In addition, EPSCs rise and decay times were slower in normal cells from TSC compared to severe CD cases. Balloon cells were found more frequently in TSC cases, whereas cytomegalic pyramidal neurons occurred more often in CD type II cases. Both cell types were similar morphologically and electrophysiologically in TSC and severe CD. These results suggest that even though the histopathology in TSC and severe CD is similar, there are subtle differences in spontaneous synaptic activity and topographic distribution of abnormal cells. These differences may contribute to variable mechanisms of epileptogenesis in patients with TSC compared with CD type II.This publication has 21 references indexed in Scilit:
- Noninvasive testing, early surgery, and seizure freedom in tuberous sclerosis complexNeurology, 2010
- Assessment and surgical outcomes for mild type I and severe type II cortical dysplasia: A critical review and the UCLA experienceEpilepsia, 2009
- Tuberous sclerosisThe Lancet, 2008
- Overlapping neurologic and cognitive phenotypes in patients with TSC1 or TSC2 mutationsNeurology, 2008
- Developmental lineage of cell types in cortical dysplasia with balloon cellsBrain, 2007
- A Hypothesis Regarding the Pathogenesis and Epileptogenesis of Pediatric Cortical Dysplasia and Hemimegalencephaly Based on MRI Cerebral Volumes and NeuN Cortical Cell DensitiesEpilepsia, 2007
- Cytomegalic InterneuronsJournal of Neuropathology and Experimental Neurology, 2007
- Neurons Recorded from Pediatric Epilepsy Surgery Patients with Cortical DysplasiaEpilepsia, 2000
- Tuberous sclerosis complexCurrent Opinion in Neurology, 2000
- Postoperative Seizure Control and Antiepileptic Drug Use in Pediatric Epilepsy Surgery Patients: The UCLA Experience, 1986–1997Epilepsia, 1999