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失神发作真的是全身性癫痫发作还是起源于或主要累及运动前区的部分性癫痫发作?一些临床和理论观察及其对癫痫发作分类的影响。

Are absences truly generalized seizures or partial seizures originating from or predominantly involving the pre-motor areas? Some clinical and theoretical observations and their implications for seizure classification.

作者信息

Craiu Dana, Magureanu Sanda, van Emde Boas Walter

机构信息

Carol Davila University of Medicine, Pediatric Neurology Clinic, Alexandru Obregia Hospital, Sos. Berceni 10, Sector 4, Bucharest, Romania.

出版信息

Epilepsy Res. 2006 Aug;70 Suppl 1:S141-55. doi: 10.1016/j.eplepsyres.2005.11.018. Epub 2006 Jul 18.

DOI:10.1016/j.eplepsyres.2005.11.018
PMID:16854562
Abstract

In both the current (1981) ILAE Classification of Epileptic Seizures and the recently Proposed Diagnostic Scheme for People with Epilepsy and Epileptic Seizures, typical absence seizures are defined as generalized seizures, implying widespread subcortical and cortical neuronal involvement from onset with impairment of consciousness as the clinical hallmark. Clinical observations from three patients and clinical and experimental data from the literature suggest, however, that: (1) consciousness is retained in many typical absences; (2) the true hallmark of these seizures is arrest of motor initiation due to disturbance of pre-motor area frontal-lobe function; (3) typical absences and partial seizures from these areas may show similar clinical and EEG features and involve the same neuronal circuits. The neuronal system primarily involved in these seizures consists of a relatively limited cortico-thalamo-cortical circuit, including the reticular thalamic nucleus, the thalamocortical relay and the predominantly anterior and mesial frontal cerebral cortex, with the cortex probably acting as the primary driving site. Typical absences thus should not be classified or defined as generalized seizures, particularly since neuropathological and imaging studies increasingly argue for localized structural abnormalities, even in idiopathic or primary generalized epilepsy. These observations further highlight the intrinsic weaknesses of the current classification system for seizures and support further adaptations of the diagnostic system currently under development.

摘要

在当前(1981年)的国际抗癫痫联盟(ILAE)癫痫发作分类以及最近提出的癫痫和癫痫发作患者诊断方案中,典型失神发作均被定义为全身性发作,这意味着从发作开始就有广泛的皮质下和皮质神经元受累,意识障碍是其临床标志。然而,来自三名患者的临床观察以及文献中的临床和实验数据表明:(1)许多典型失神发作时意识并未丧失;(2)这些发作的真正标志是由于运动前区额叶功能紊乱导致运动起始停止;(3)这些区域的典型失神发作和部分性发作可能表现出相似的临床和脑电图特征,并涉及相同的神经元回路。主要参与这些发作的神经元系统由一个相对有限的皮质 - 丘脑 - 皮质回路组成,包括丘脑网状核、丘脑皮质中继以及主要是额叶前部和内侧的大脑皮质,其中皮质可能是主要的驱动部位。因此,典型失神发作不应被归类或定义为全身性发作,特别是因为神经病理学和影像学研究越来越多地支持存在局部结构异常,即使在特发性或原发性全身性癫痫中也是如此。这些观察结果进一步凸显了当前癫痫发作分类系统的内在缺陷,并支持对目前正在开发的诊断系统进行进一步调整。

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