Skip to main content

Clinical features and epileptogenesis of dysembryoplastic neuroepithelial tumor

Abstract

Introduction

Dysembryoplastic neuroepithelial tumor (DNT) frequently causes medically intractable epilepsy.

Objective

The aim of this study was to investigate the basic mechanism of epileptogenecity of the tumor.

Materials and methods

Clinicopathological data in 13 cases of DNT and immunohistochemical changes of ionotropic glutamate receptor subunits in the tumor and peritumoral epileptogenic cortex were studied.

Conclusions

Magnetic resonance imaging combined with electroencephalography (EEG), electrocorticography, and depth-electrode EEG was valuable to localize complicated epileptogenic zones of the patients with DNT. Neuropathological examinations of the peritumoral cerebral cortex presenting abnormal spikes showed different histopathological grades of neuronal migration disorder (NMD). The tumor cells in DNT disclosed increased immunopositivities of N-methyl-d-aspartate receptor 1 (NR1) and NR2A/B, and peritumoral epileptogenic NMD revealed increased immunopositivities of GluR2 and GluR3. The amplification of ionotropic glutamate receptor subunits in the tumor and peritumoral NMD may be the underlying cause of epileptic seizures in DNT patients.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3

References

  1. Aronica E, Leenstra S, Jansen GH, van Veelen CW, Yankaya B, Troost D (2001) Expression of brain-derived neurotrophic factor and tyrosine kinase B receptor proteins in glioneuronal tumors from patients with intractable epilepsy: colocalization with N-methyl-D-aspartic acid receptor. Acta Neuropathol (Berl) 101:383–392

    CAS  Google Scholar 

  2. Babb TL, Mathern GW, Leite JP, Pretorius JK, Yeoman KM, Kuhlman PA (1996) Glutamate AMPA receptors of the fascia dentata of human and kainate rat hippocampal epilepsy. Epilepsy Res 26:193–205

    PubMed  Article  CAS  Google Scholar 

  3. Daumas-Duport C, Varlet P, Bacha S, Beuvon F, Cervera-Pierot P, Chodkiewicz JP (1999) Dysembryoplastic neuroepithelial tumors: nonspecific histological forms - a study of 40 cases. J Neurooncol 41:267–280

    PubMed  Article  CAS  Google Scholar 

  4. Enger J Jr (1991) Outcome with respect to epileptic seizures. In: Engel J Jr (ed) Surgical treatment of epilepsy. Raven, New York 503–506

    Google Scholar 

  5. Fukao K, Watanabe Y, Shiraishi H, Yamada K, Matsuda K, Mihara T, Fujiwara T, Yagi K (2000) Magnetoencephalographic findings on patients having symptomatic localization-related epilepsy with dysembryoplastic neuroepithelial tumor as the epileptogenic lesion. Epilepsia 41:61–62

    Article  Google Scholar 

  6. Gorter JA, Petrozzino JJ, Aronica EM, Rosenbaum DM, Opitz T, Bennett MV, Connor JA, Zukin RS (1997) Global ischemia induces downregulation of GluR2 mRNA and increases AMPA receptor-mediated Ca2+ influx in hippocampal CA1 neurons of gerbil. J Neurosci 17:6179–6188

    PubMed  CAS  Google Scholar 

  7. Haglund MM, Berger MS, Kunkel DD, Franck JE, Ghatan S, Ojemann GA (1992) Changes in gamma-aminobutyric acid and somatostatin in epileptic cortex associated with low-grade gliomas. J Neurosurg 77:209–216

    PubMed  CAS  Article  Google Scholar 

  8. Lee MC, Kim KM, Woo YJ, Kim JH, Kim MK, Nam SC, Seo JJ, Jung WK, Lee JS, Kim SU (2001) Pathogenic significance of neuronal migration disorders in temporal lobe epilepsy. Hum Pathol 32:643–648

    PubMed  Article  CAS  Google Scholar 

  9. Lee DY, Chung CK, Hwang YS, Choe G, Chi JG, Kim HJ, Cho BK (2004) Dysembryoplastic neuroepithelial tumor: radiological findings (including PET, SPECT, and MRS) and surgical strategy. J Neurooncol 47:167–174

    Article  Google Scholar 

  10. Meldrum BS, Akbar MT, Chapman AG (1999) Glutamate receptors and transporters in genetic and acquired models of epilepsy. Epilepsy Res 36:189–204

    PubMed  Article  CAS  Google Scholar 

  11. Monyer H, Sprengel R, Schoepfer R, Herb A, Higuchi M, Lomeli H, Burnashev N, Sakmann B, Seeburg PH (1992) Heteromeric NMDA receptors: molecular and functional distinction of subtypes. Science 256:1217–1221

    PubMed  Article  ADS  CAS  Google Scholar 

  12. Nakanishi S (1992) Molecular diversity of glutamate receptors and implications for brain function. Science 258(5082):597–603

    PubMed  Article  ADS  CAS  Google Scholar 

  13. Nakanishi S, Masu M (1994) Molecular diversity and functions of glutamate receptors. Annu Rev Biophys Biomol Struct 23:319–348

    PubMed  Article  CAS  Google Scholar 

  14. Plate KH, Wieser HG, Yasargil MG, Wiestler OD (1993) Neuropathological findings in 224 patients with temporal lobe epilepsy. Acta Neuropathol (Berl) 86:433–438

    CAS  Google Scholar 

  15. Prayson PA, Morris HH, Estes ML, Comair YG (1996) Dysembryoplastic neuroepithelial tumor: a clinicopathologic and immunohistochemical study of 11 tumors including MIB1 immunoreactivity. Clin Neuropathol 15:47–53

    PubMed  CAS  Google Scholar 

  16. Sisodiya SM, Heffernan J, Harrison PJ, Squier MV, Thom M (2000) Sulcogyral variation in NMDA receptor 2A/B subunit immunoreactivity in human brain. Neuroreport 11:2601–2606

    PubMed  Article  CAS  Google Scholar 

  17. Wolf HK, Welimer J, Muller MB, Wiestler OD, Hufnagel A, Pietsch T (1995) Glioneuronal malformative lesions and dysembryoplastic neuroepithelial tumors in patients with chronic pharmacoresistant epilepsies. J Neuropathol Exp Neurol 54:245–254

    PubMed  CAS  Article  Google Scholar 

  18. Wong RK, Bianchi R, Taylor GW, Merlin LR (1999) Role of metabotropic glutamate receptors in epilepsy. Adv Neurol 79:685–698

    PubMed  CAS  Google Scholar 

  19. Ying Z, Babb TL, Comair YG, Bingaman W, Bushey M, Touhalisky K (1998) Induced expression of NMDAR2 proteins and differential expression of NMDAR1 splice variants in dysplastic neurons of human epileptic neocortex. J Neuropathol Exp Neurol 57:47–62

    PubMed  CAS  Google Scholar 

Download references

Acknowledgment

This work was supported by the Korea Science and Engineering Foundation through the Medical Research Center for Gene Regulation (R13-2002-013-01000-0) at Chonnam National University.

Author information

Affiliations

Authors

Corresponding author

Correspondence to Min-Cheol Lee.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Lee, MC., Kang, JY., Seol, MB. et al. Clinical features and epileptogenesis of dysembryoplastic neuroepithelial tumor. Childs Nerv Syst 22, 1611–1618 (2006). https://doi.org/10.1007/s00381-006-0162-z

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-006-0162-z

Keywords

  • Dysembryoplastic neuroepithelial tumor
  • Epilepsy
  • Glutamate receptor
  • Immunohistochemistry
  • Neuronal migration disorder