Aronica Eleonora, Mühlebner Angelika
Department of Neuropathology, Academic Medical Center and Swammerdam Institute for Life Sciences, Center for Neuroscience, University of Amsterdam, Amsterdam, the Netherlands; Stichting Epilepsie Instellingen Nederland, the Netherlands.
Department of Neuropathology, Academic Medical Center and Swammerdam Institute for Life Sciences, Center for Neuroscience, University of Amsterdam, Amsterdam, the Netherlands.
Handb Clin Neurol. 2017;145:193-216. doi: 10.1016/B978-0-12-802395-2.00015-8.
Epilepsy is one of the most common neurologic disorders, affecting about 50 million people worldwide. The disease is characterized by recurrent seizures, which are due to aberrant neuronal networks resulting in synchronous discharges. The term epilepsy encompasses a large spectrum of syndromes and diseases with different etiopathogenesis. The recent development of imaging and epilepsy surgery techniques is now enabling the identification of structural abnormalities that are part of the epileptic network, and the removal of these lesions may result in control of seizures. Access of this clinically well-characterized neurosurgical material has provided neuropathologists with the opportunity to study a variety of structural brain abnormalities associated with epilepsy, by combining traditional routine histopathologic methods with molecular genetics and functional analysis of the resected tissue. This approach has contributed greatly to a better diagnosis and classification of these structural lesions, and has provided important new insights into their pathogenesis and epileptogenesis. The present chapter provides a detailed description of the large spectrum of histopathologic findings encountered in epilepsy surgery patients, addressing in particular the nonneoplastic pathologies, including hippocampal sclerosis, malformations of cortical development, Sturge-Weber syndrome, and Rasmussen encephalitis, and reviews current knowledge regarding the underlying molecular pathomechanisms and cellular mechanisms mediating hyperexcitability.
癫痫是最常见的神经系统疾病之一,全球约有5000万人受其影响。该疾病的特征是反复发作的癫痫发作,这是由异常的神经元网络导致同步放电引起的。癫痫这一术语涵盖了具有不同病因发病机制的广泛综合征和疾病。成像和癫痫手术技术的最新发展现在能够识别癫痫网络的一部分结构异常,切除这些病变可能会导致癫痫发作得到控制。获得这种临床特征明确的神经外科材料,为神经病理学家提供了机会,通过将传统的常规组织病理学方法与分子遗传学以及切除组织的功能分析相结合,来研究与癫痫相关的各种脑结构异常。这种方法对这些结构病变的更好诊断和分类做出了巨大贡献,并为其发病机制和癫痫发生机制提供了重要的新见解。本章详细描述了癫痫手术患者中遇到的广泛组织病理学发现,特别讨论了非肿瘤性病变,包括海马硬化、皮质发育畸形、斯特奇-韦伯综合征和拉斯穆森脑炎,并综述了关于介导兴奋性过高的潜在分子发病机制和细胞机制的当前知识。