Wolf Peter
Danish Epilepsy Centre, Dianalund, Denmark.
Epilepsia. 2005;46 Suppl 9:7-9. doi: 10.1111/j.1528-1167.2005.00308.x.
Early in these proceedings, the origin of the three terms in the title, "idiopathic generalized epilepsy," is discussed with respect to their significance over time, and typical misunderstandings. In the mid-20th century, a rather chaotic use of a multitude of often loosely defined terms had developed, which increasingly became an obstacle to a meaningful international discussion. The International League against Epilepsy (ILAE) took the initiative to develop an internationally accepted terminology with a classification system consisting of a classification of seizures (1981) and a classification of syndromes (1989). The Idiopathic Generalized Epilepsies are one of its four major groups emerging from a double dichotomy of generalized versus localization-related and idiopathic versus symptomatic. The inclusion of biologic aspects such as syndrome-specific ages of onset ("age-related syndromes") or syndrome-specific relations of seizure occurrence to the sleep-wake cycle ("Epilepsy with Grand Mal on Awaking") meant that the syndrome classification merged the more biological views of the German school with the more neurophysiological ones of the French. Apart from establishing a common international language concerning epilepsy, the International Classification of Epilepsies and Epileptic Syndromes became an important stimulator of research, especially concerning the idiopathic epilepsies. In particular, genetic and functional imaging investigations aim at a better understanding of these conditions. It is now understood that most idiopathic syndromes have a--sometimes complex--genetic background, but we are also becoming aware of the inappropriateness of the time-honored term "generalized" and part of our dichotomies. Both localization-related and "generalized" idiopathic epilepsies seem to share a principle of ictogenesis based on functional anatomic pathogenic networks, and we seem to move toward understanding them as functional system disorders of the brain.
在这些讨论的早期,标题中“特发性全身性癫痫”这三个术语的起源就其随时间的意义以及典型的误解进行了探讨。在20世纪中叶,出现了对众多定义往往很松散的术语相当混乱的使用情况,这日益成为有意义的国际讨论的障碍。国际抗癫痫联盟(ILAE)主动制定了一种国际通用的术语,并建立了一个分类系统,该系统包括发作分类(1981年)和综合征分类(1989年)。特发性全身性癫痫是从全身性与局灶性相关以及特发性与症状性的双重二分法中产生的四个主要类别之一。纳入生物学方面的内容,如综合征特异性的发病年龄(“年龄相关综合征”)或发作与睡眠 - 觉醒周期的综合征特异性关系(“觉醒时大发作癫痫”)意味着综合征分类将德国学派更多的生物学观点与法国学派更多的神经生理学观点融合在一起。除了建立关于癫痫的通用国际语言外,《国际癫痫分类和癫痫综合征》还成为研究的重要推动因素,特别是关于特发性癫痫的研究。尤其是基因和功能成像研究旨在更好地理解这些病症。现在人们认识到,大多数特发性综合征都有一个——有时很复杂的——遗传背景,但我们也开始意识到由来已久的“全身性”术语以及我们部分二分法的不恰当性。局灶性相关和“全身性”特发性癫痫似乎都基于功能性解剖致病网络共享一个发作起源原则,而且我们似乎正朝着将它们理解为大脑的功能性系统障碍的方向发展。