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Epileptogenesis and Cortical Dysplasias

  • Carlos Cepeda
  • Véronique M. André
  • Harry V. Vinters
  • Robin S. Fisher
  • Michael S. Levine
  • Gary W. Mathern

Definition

Cortical dysplasia (CD) is a malformation of brain development characterized by aberrant lamination of the cerebral cortex, pyramidal cell misorientation, and in severe cases, the presence of dysplastic cytomegalic neurons and balloon cells (Taylor et al. 1971). In children undergoing epilepsy neurosurgery, CD is the most frequently identified etiology accounting for over 40% of cases (Harvey et al. 2008). In fact, CD is found in approximately 70% of epilepsy surgery patients operated in the first 2 years of life.

Classification

In a widely accepted classification, CD has been divided, based on histopathological criteria, into two main types; mild Type I CD, in which cortical dyslamination is the main feature, and severe Type II CD where, in addition to altered cortical architecture, dysplastic cells are observed (Mischel et al. 1995; Palmini et al. 2004). Dysplastic cells have been hypothesized as potential generators of epileptic discharges (Kerfoot et al. 1999;...

Keywords

Pyramidal Neuron Tuberous Sclerosis Complex Cortical Dysplasia Epileptic Activity Epileptic Discharge 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

List of Abbreviations

CD

Cortical dysplasia

NMDA

N-methyl-D-aspartate

TSC

Tuberous sclerosis complex

UCLA

University of California Los Angeles

Notes

Acknowledgment

The authors would like to thank the young patients and their parents for allowing use of resected tissue samples for research purposes. We also thank Raymond S. Hurst, Jorge Flores-Hernández, Elizabeth Hernández-Echeagaray, Nanping Wu, Irene Yamazaki, and Marea K. Boylan, for their assistance in electrophysiological data collection and morphological analyses. The expertise and dedication of the UCLA Hospital Pediatric Neurology Staff are also greatly appreciated. This work was supported by NIH grant NS 38992 from the NINDS.

References

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Copyright information

© Springer-Verlag London Limited 2010

Authors and Affiliations

  • Carlos Cepeda
    • 1
  • Véronique M. André
    • 1
  • Harry V. Vinters
    • 3
  • Robin S. Fisher
    • 1
  • Michael S. Levine
    • 1
  • Gary W. Mathern
    • 2
  1. 1.Mental Retardation Research Center, David Geffen School of MedicineUniversity of California, Los AngelesLos Angeles, CA 90024USA
  2. 2.Department of Neurosurgery, David Geffen School of MedicineUniversity of California, Los AngelesLos Angeles, CA 90024USA
  3. 3.Division of Neuropathology, David Geffen School of MedicineUniversity of California, Los AngelesLos Angeles, CA 90024USA

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