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  • Updated 06.24.2022
  • Released 06.24.2022
  • Expires For CME 06.24.2025

Brain tumor-related epilepsy

Introduction

Overview

Seizures are commonly associated with brain tumors, regardless of the grade of brain tumor. The impact of seizures on patients’ quality of life cannot be understated. This article provides a summary of the current literature on the epidemiology, pathophysiology, medical and surgical management, and prognosis in this patient population.

Historical note and terminology

Essentially any structural lesion carries a risk of causing seizures. The likelihood of seizure varies depending on the type of lesion, location, and molecular genetics. The first known report of tumor-related epilepsy was documented by Hughlings Jackson in 1882 (42). The case report described a man with seizure onset at the age of 28, with variable semiology, including focal dystonic seizures and aphasic seizures that intermittently progressed to convulsions. A glial tumor in the left hemisphere was discovered at autopsy.

In time, the brain tumors most commonly associated with epilepsy were reported with ganglioglioma (1926) and with dysembryoplastic neuroepithelial tumors (1988) (37). The World Health Organization’s definition of glioneuronal tumors includes both gangliogliomas and dysembryoplastic neuroepithelial tumors. These glioneuronal tumors are highly epileptogenic, with seizures often presenting early in life, similar to pleomorphic xanthoastrocytomas and pilocytic astrocytomas.

As a group, these low-grade tumors were the first tumors to be included in the category of “long-term epilepsy-associated tumors” (LEATs) when the term was first defined by Luyken in 2003 (26). Since then, the term LEAT has also evolved to encompass papillary glioneuronal tumors, angiocentric gliomas, isomorphic diffuse gliomas, multinodular and vacuolating neuronal tumors, and polymorphous low-grade neuroepithelial tumors (37). Although the tumors included in the category have been established, the current understanding of LEATs as an entity is evolving with access to molecular genetic testing and DNA methylation profiling. The World Health Organization’s classification does not account for the variability of histopathology in LEATs. One proposed neuropathological classification system is A-B-C terminology, which is guided by immunohistochemical markers to allow for a more precise classification using more modern diagnostic testing results (01).

The terms “tumor-related epilepsy,” “brain tumor–related epilepsy,” and “tumoral epilepsy” are often used interchangeably to describe any patient at risk of seizures if they are without antiseizure medications in the context of a brain tumor. Seizures may occur at any point during the course of a patient’s disease, including prior to resection, in the context of ongoing radiation or chemotherapeutic treatment, or after completion of standard-of-care management. As such, epilepsy management in patients with tumor-related epilepsy must be re-evaluated on an ongoing basis to assess whether adjustments in their medication are warranted.

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