Epilepsy Related to Developmental Tumors and Malformations of Cortical Development

Abstract

Structural abnormalities of the brain are increasing-

Immunity and Inflammation in Epilepsy

“…Moreover, in a cohort FCD II case, the density of activated microglial cells significantly correlates with the duration of epilepsy, as well as with the frequency of seizures before surgical resection . Moreover, the number of activated microglial cells and CD3/CD8 positive T cells, as well as the expression of the major histocompatibility complex class I (MHC-I) in neuronal cells and the expression of complement components, IL1b and chemokines, was significantly higher in FCD type II than in FCD type I cortical specimens (Ravizza et al 2006;Iyer et al 2010a;Prabowo et al 2013a), supporting the notion that these two types of FCD are pathologically distinct (Aronica and Crino 2014). In FCD II, there is also evidence of activation of the plasminogen system (Iyer et al 2010b), overexpression of HMGB1 and its receptors (TLR2, TLR4, and RAGE) (Zurolo et al 2011), and focal BBB disruption (Prabowo et al 2013a).…”

“…Similarly to FCD II, a complex activation of proinflammatory signaling pathways has been reported in cortical tubers in TSC patients (Boer et al , 2010Iyer et al 2010b;Zurolo et al 2011;Aronica and Crino 2014). Interestingly, evaluation of fetal TSC cases showed a prenatal activation of the innate immune response with premature activation of key inflammatory pathways, such as TLR signaling .…”

“…These forms, which include FCD and cortical tubers of patients with TSC, as well as glioneuronal tumors (GNTs), are among the most common causes of pharmacologically intractable epilepsy (Blumcke et al 2009;Sisodiya et al 2009;Aronica et al 2012a;Barkovich et al 2012;Thom et al 2012;Aronica and Crino 2014).…”

Surgical strategies for pediatric epilepsy

“…Neoplastic causes of epilepsy comprise up to 20% of surgical cases in the pediatric population (59). Most are low-grade lesions, with the high rates of epileptogenesis possibly related to the nature of some of these tumors (i.e., gangliogliomas, dysembryoplastic neuroepithelial tumors) as developmental lesions on the spectrum of focal cortical dysplasias (60). The unique challenge of temporal lesionectomy is to determine the appropriate extent of resection.…”

Novel Concepts in Epileptogenesis and its Prevention

“…This section on "mechanisms" is designed to take the "epileptogenesis" discussion to a deeper and more specific level, focusing it on the most frequent and/or challenging epilepsy syndromes, including hippocampal sclerosis, tackled by Patterson et al [6]; the development of epilepsy after head trauma, addressed by Pitkänen and Immonem [7]; and the defiant issue of epileptogenesis in "non-lesional" epilepsies, undertaken by Guerrini and Marini [8]. A separate article by Aronica and Crino [9] brings to the forefront the state-of-the-art understanding of epileptogenesis in developmental brain tumors and malformations of cortical development. Special pediatric considerations related to pediatric epilepsy syndromes are highlighted in a dedicated paper by Pardo et al [10] This section is concluded by reviewing mechanisms of epileptogenesis in autoimmune epilepsies, as reviewed by Bien and Bauer [11].…”