Cortical dysplasia: electroclinical, imaging, and neuropathologic study of 13 patients

Epilepsia. 2001 Sep;42(9):1112-23. doi: 10.1046/j.1528-1157.2001.00501.x.

Abstract

Purpose: The aim of this study was to correlate the electroclinical and radiologic data with the neuropathologic findings and surgical outcome in epileptic patients with epilepsy and Taylor's focal cortical dysplasia (TFCD) and to characterize further the abnormal intermediate filaments expression in the balloon cell present in the peculiar dysplasia.

Methods: We retrospectively selected 13 TFCD patients who underwent surgery for intractable epilepsy with the aim of removing the magnetic resonance (MR)-detectable lesion and/or the epileptogenic zone defined by stereoelectroencephalographic recordings. The surgical specimens were analyzed by means of routine neuropathologic and immunocytochemical studies. Antisera against different intermediate filaments also were used in serial adjacent sections to evaluate their coexpression in balloon cells.

Results: Histopathologic abnormalities typical of TFCD were found not only within the MR-visible lesions but also in most of the epileptogenic zones with no MR signal alterations. Furthermore, the MR-visible lesions contained a high proportion of cells with an abnormal expression of intermediate filament proteins. After a long follow-up, 10 of the patients are now seizure free.

Conclusions: Our findings indicate that highly epileptogenic zones may correspond to tissue alterations not revealed by neuroimaging. Furthermore, the immunocytochemical data show that the dysplastic tissue detected by MR contained high concentrations of cells filled with abnormal intermediate filaments. The detected colocalization of neuronal and glial markers in balloon cells indicates a failure of cellular commitment during development.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Brain Diseases / diagnosis
  • Brain Diseases / metabolism
  • Brain Diseases / pathology
  • Cerebral Cortex / abnormalities*
  • Cerebral Cortex / metabolism
  • Cerebral Cortex / pathology
  • Child
  • Electrodes, Implanted
  • Electroencephalography / methods
  • Electroencephalography / statistics & numerical data
  • Epilepsy / diagnosis*
  • Epilepsy / metabolism
  • Epilepsy / surgery
  • Female
  • Follow-Up Studies
  • Gene Expression
  • Gliosis / diagnosis
  • Gliosis / pathology
  • Humans
  • Immunohistochemistry
  • Intermediate Filament Proteins / metabolism
  • Magnetic Resonance Imaging / statistics & numerical data
  • Male
  • Nervous System Malformations / diagnosis
  • Nervous System Malformations / metabolism
  • Nervous System Malformations / pathology

Substances

  • Intermediate Filament Proteins