Halász Péter, Kelemen Anna
Pázmány Péter Catholic University, Faculty of Information Technology, Budapest.
Ideggyogy Sz. 2009 Nov 30;62(11-12):366-80.
The aim of this work is to show explicitly why the "idiopathic generalized epilepsy" concept becomes outfashioned and untenable. As the concept of "generalized epilepsies" is from long ago closely related to the thalamo-cortical system, we briefly summarize the functional anatomy, the double working mode of the thalamo-cortical system in different vigilance states and it's role in development of the spike-wave pattern. The next part shows weaknesses of this concept from the EEG, seizure semiology, and neuroimaging point of view. Further experimental and clinical arguments are accumulated from the reflex epileptic features in IGE, indicating local/regional cortical hyperexcitability. A separate part is devoted to genetic aspects of the question. Lastly implications to epilepsy classification are shown and an outlook toward a unified epilepsy concept is provided. The epileptic disorder of the thalamo-cortical system is responsible for the development of "generalized", synchronous spike-wave paroxysms as the common neurophysiological background in "primary" - idiopathic and in "secondary" generalized epilepsies. This disorder is specifically related to the burstfiring working mode of the thalamo-cortical system during NREM sleep (is an epileptic exageration of it). The "generalized" epilepsy category should be abandoned, being misleading. Epilepsies are proposed to be classified according to their network properties and relations to different physiological systems of the brain. The different phenotypes, named earlier idiopathic (primary) generalized, or symptomatic (secondary) generalized (with encephalopathic features), should be delineated depending on the following factors: 1. speed and extent of syncronization within the thalamo-cortical system, 2. the way how the thalamo-cortical system is involved, 3. which kind of cortical triggers play role, 4. the degree and level of the disorder (restricted to the molecular level or extended to the level of structural alterations - in the cortex or more diffusely, 5. genetic targets and features.
这项工作的目的是明确展示为何“特发性全身性癫痫”这一概念已过时且站不住脚。由于“全身性癫痫”这一概念长期以来与丘脑 - 皮质系统密切相关,我们简要总结其功能解剖、丘脑 - 皮质系统在不同觉醒状态下的双重工作模式及其在棘波模式形成中的作用。下一部分从脑电图、发作症状学和神经影像学角度展示了这一概念的弱点。从特发性全身性癫痫的反射性癫痫特征中积累了更多实验和临床依据,表明存在局部/区域皮质兴奋性过高。单独一部分专门探讨了该问题的遗传学方面。最后展示了对癫痫分类的影响,并对统一的癫痫概念进行了展望。丘脑 - 皮质系统的癫痫性障碍是“全身性”同步棘波阵发形成的原因,这是“原发性”(特发性)和“继发性”全身性癫痫共同的神经生理背景。这种障碍与非快速眼动睡眠期间丘脑 - 皮质系统的爆发式放电工作模式密切相关(是其癫痫性增强)。“全身性”癫痫类别应被摒弃,因其具有误导性。建议根据癫痫的网络特性及其与大脑不同生理系统的关系对癫痫进行分类。应根据以下因素来界定先前命名为特发性(原发性)全身性或症状性(继发性)全身性(伴有脑病特征)的不同表型:1. 丘脑 - 皮质系统内同步化的速度和程度;2. 丘脑 - 皮质系统的参与方式;3. 哪种皮质触发因素起作用;4. 障碍的程度和水平(局限于分子水平还是扩展到结构改变水平——在皮质或更广泛区域);5. 遗传靶点和特征。