Focal Epilepsy Associated with Glioneuronal Tumors

Giulia Loiacono,Alberto Verrotti,Chiara Cirillo,Francesco Chiarelli

doi:10.5402/2011/867503

Giulia Loiacono, Alberto Verrotti + Show 2 more

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https://doi.org/10.5402/2011/867503

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Journal: ISRN Neurology	Publication Date: Jul 7, 2011
Citations: 69	License type: CC BY 3.0

Affiliation: University of Chieti-Pescara

Abstract

Glioneuronal tumors are an increasingly recognized cause of partial seizures that occur primarily in children and young adults. Focal epilepsy associated with glioneuronal tumors is often resistant to pharmacological treatment. The cellular mechanisms underlying the epileptogenicity of glioneuronal tumors remain largely unknown. The involved mechanisms are certain to be multifactorial and depend on specific tumor histology, integrity of the blood-brain barrier, characteristics of the peritumoral environment, circuit abnormalities, or cellular and molecular defects. Glioneuronal tumors presenting with epilepsy were observed to have relatively benign biological behavior. The completeness of the tumor resection is of paramount importance in avoiding tumor progression and malignant transformation, which are rare in cases of epileptogenic glioneuronal tumors. An evolving understanding of the various mechanisms of tumor-related epileptogenicity may also lead to a more defined surgical objective and effective therapeutic strategies, including antiepileptogenic treatments, to prevent epilepsy in at-risk patients.

Highlights

Glioneuronal tumors are an increasingly recognized cause of partial seizures that occur primarily in children and young adults [1, 2].These are tumors with an admixture of glial and neuronal components
Several mechanisms are possibly involved, including a direct regulation of neuronal excitability via NMDA receptors [48]. Both type plasminogen activator (tPA) and urokinasetype plasminog activator (uPA) may contribute to the disruption of bloodbrain barrier integrity and amplification of inflammatory infiltrates [52], which have been recently shown to play a critical role in chronic refractory epilepsy [17, 53]
Patients with drug-resistant epilepsy associated to glioneuronal tumors should undergo evaluation in dedicated epilepsy surgery programmes

Summary

Introduction

Glioneuronal tumors are an increasingly recognized cause of partial seizures that occur primarily in children and young adults [1, 2]. These are tumors with an admixture of glial and neuronal components. Entrapment of preexisting neurons by an infiltrating glioma has to be distinguished from glioneuronal tumors. The glial component in these tumors varies but often resembles either a pilocytic astrocytoma or an infiltrating glioma with astrocytic or oligodendroglial features [3]. Focal epilepsy that is often resistant to pharmacological treatment is a common presenting symptom of glioneuronal tumors [1, 2]. While a subset of epilepsy patients with glioneuronal tumors may be candidates for epilepsy surgery, a better understanding of underlying mechanisms of epileptogenesis in this group of developmental brain disorders could lead to more effective therapeutic strategies, including antiepileptogenic treatments to prevent epilepsy in at-risk patients

Epileptogenesis

Treatment

Findings

Conclusion