Intrinsic epileptogenicity of dysplastic cortex: Converging data from experimental models and human patients

Giorgio Battaglia, Francesca Colciaghi, Adele Finardi, Paola Nobili

Research output: Contribution to journalArticle

Abstract

Focal cortical dysplasia (FCD) is a brain malformation associated with particularly severe drugresistant epilepsy that often requires surgery for seizure control. The molecular basis for such enhanced propensity to seizure generation in FCD is not as yet elucidated. To investigate cellular and molecular bases of epileptogenic mechanisms and possible effect of severe epilepsy on the malformed cortex we have here performed a parallel analysis of a rat model of acquired cortical dysplasia previously established in our laboratory, i.e., the methylazoxymethanol/ pilocarpine (MAM-PILO) rats, and surgical samples from patients with type IIB FCD. Data from the MAM-PILO rat model and human FCD samples reveal in both conditions: (1) that status epilepticus (SE) and/or seizures can further modify the cellular and molecular settings of the malformed cortex; (2) excitation/inhibition imbalance, and dysregulation of the N-methyl-D-aspartate/ membrane-associated guanylate kinase (NMDA/MAGUK) expression; (3) activation of cell death in neurons and glia. The data therefore highlight the mechanistic relevance of glutamate/ NMDA hyperactivation in FCD epileptogenesis and suggest that epilepsy is a pathologic process capable of affecting structure and function of both neurons and glia.

Original languageEnglish
Pages (from-to)33-36
Number of pages4
JournalEpilepsia
Volume54
Issue numberSUPPL. 6
DOIs
Publication statusPublished - 2013

Fingerprint

Malformations of Cortical Development
Theoretical Models
Epilepsy
Seizures
Pilocarpine
N-Methylaspartate
Neuroglia
Guanylate Kinases
Neurons
Status Epilepticus
Pathologic Processes
Glutamic Acid
Cell Death
Brain

Keywords

  • Focal cortical dysplasia
  • Glutamatergic synapse
  • MAGUK proteins
  • NMDA receptor
  • Seizure-induced cell death

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology
  • Medicine(all)

Cite this

Intrinsic epileptogenicity of dysplastic cortex : Converging data from experimental models and human patients. / Battaglia, Giorgio; Colciaghi, Francesca; Finardi, Adele; Nobili, Paola.

In: Epilepsia, Vol. 54, No. SUPPL. 6, 2013, p. 33-36.

Research output: Contribution to journalArticle

Battaglia, Giorgio ; Colciaghi, Francesca ; Finardi, Adele ; Nobili, Paola. / Intrinsic epileptogenicity of dysplastic cortex : Converging data from experimental models and human patients. In: Epilepsia. 2013 ; Vol. 54, No. SUPPL. 6. pp. 33-36.
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