Engel J
UCLA School of Medicine, Los Angeles, California 90095-1769, USA.
Epilepsia. 1998 Sep;39(9):1014-7. doi: 10.1111/j.1528-1157.1998.tb01453.x.
Recent advances in neurodiagnostic technology, new insights into fundamental neuronal mechanisms of epilepsy, and rapid developments in molecular genetics have greatly improved our understanding of epileptic seizures and epileptic disorders since 1981, when the current International Classification of Epileptic Seizures was adopted, and since 1989, when the International Classification of Epilepsies, Epileptic Syndromes, and Related Disorders was adopted. Although both have been universally accepted and have proven to be of considerable clinical value, it has become clear that more is needed for specific applications of growing importance, such as presurgical evaluation, clinical pharmacology trials, and epidemiological studies. Therefore, an International League Against Epilepsy (ILAE) task force has evaluated the need for revising our current classification and terminology and has begun developing four specific documents. The first is a descriptive terminology for ictal phenomena, incorporating some of the concepts included in the preceding paper by Dr. Hans Lüders; however, this will be a glossary, not a classification. The second is a classification of epileptic seizures based on known or presumed pathophysiological and anatomic substrates, to replace the current classification, which is based entirely on phenomenology. The third is a classification of epileptic syndromes and epileptic diseases, adapted from the current Classification of Epilepsies, Epileptic Syndromes, and Related Disorders, which will take into account the fact that disorders with unique etiologies, such as a single gene, should be considered diseases, and which will be reorganized for maximum practical application to differential diagnosis. The fourth is a new classification of functional disability due to seizures or epilepsy, based on a new impairment classification for neurological disorders being developed by the World Health Organization. Working groups are currently in the process of developing each of these documents, and their chairs would benefit from any comments or suggestions from those who ultimately will be using these systems of classification and terminology.
自1981年采用现行国际癫痫发作分类法以及自1989年采用国际癫痫、癫痫综合征及相关障碍分类法以来，神经诊断技术的最新进展、对癫痫基本神经元机制的新认识以及分子遗传学的快速发展，极大地增进了我们对癫痫发作和癫痫性疾病的理解。尽管这两种分类法都已被普遍接受并已证明具有相当大的临床价值，但显然对于日益重要的特定应用，如术前评估、临床药理学试验和流行病学研究，还需要更多内容。因此，国际抗癫痫联盟（ILAE）的一个特别工作组评估了修订我们现行分类和术语的必要性，并已开始编写四份具体文件。第一份是发作期现象的描述性术语，纳入了汉斯·吕德斯博士在前一篇论文中提出的一些概念；然而，这将是一个术语表，而不是一个分类。第二份是基于已知或推测的病理生理和解剖学基础的癫痫发作分类，以取代完全基于现象学的现行分类。第三份是癫痫综合征和癫痫疾病的分类，改编自现行的癫痫、癫痫综合征及相关障碍分类，它将考虑到具有独特病因（如单一基因）的疾病应被视为疾病这一事实，并将进行重新组织以最大程度地实际应用于鉴别诊断。第四份是基于世界卫生组织正在制定的神经系统疾病新损伤分类的癫痫发作或癫痫所致功能残疾的新分类。目前各工作组正在编写这些文件中的每一份，其主席将受益于最终使用这些分类和术语系统的人员提出的任何意见或建议。
