Aicardi J
National Institute of Health and Medical Investigation (INSERM), Hôpital des Enfants Malades, Paris, France.
Epilepsia. 1988;29 Suppl 3:S1-5. doi: 10.1111/j.1528-1157.1988.tb05803.x.
Epileptic syndromes are clusters of signs and symptoms regularly occurring together. These may include type of seizure(s), time and circumstances of occurrence, and neurological and EEG findings. Some syndromes have common signs and a predictable course (e.g., benign rolandic epilepsy). Others, such as absence seizures, are less specific and may include several subgroups with different outcomes and different associated features. Still others are rather loose collections of a few common characteristics irregularly linked together. Widely accepted syndromes in childhood include the West syndrome and Lennox-Gastaut syndromes, several myoclonic syndromes, febrile seizures of infancy, absence epilepsy, benign partial epilepsy, and juvenile myoclonic epilepsy. If the concept of epileptic syndromes is to be practically useful, it should be limited to clusters that are unequivocally identifiable. Heterogeneous epileptic syndromes such as West syndrome or absence epilepsies give only a limited guide to prognosis but may be important in determining investigations and treatment. A classification based on syndromes has the advantage of dispensing with most assumptions required by other systems. However, it cannot cover all the clinical aspects of epilepsy and gives no guide to the pathophysiology, genetics, and etiology of a seizure disorder. The ultimate goal remains the delineation of disease entities.
癫痫综合征是经常一起出现的一组体征和症状。这些可能包括癫痫发作类型、发作时间和情况，以及神经学和脑电图检查结果。有些综合征有共同的体征和可预测的病程（如良性罗兰多癫痫）。其他的，如失神发作，则不太具有特异性，可能包括几个具有不同预后和不同相关特征的亚组。还有一些则是由一些不规则联系在一起的共同特征组成的相当松散的集合。儿童期广泛认可的综合征包括韦斯特综合征、伦诺克斯 - 加斯东综合征、几种肌阵挛综合征、婴儿热性惊厥、失神癫痫、良性部分性癫痫和青少年肌阵挛癫痫。如果癫痫综合征的概念要具有实际用途，它应该仅限于能够明确识别的综合征群。像韦斯特综合征或失神癫痫这样的异质性癫痫综合征对预后的指导作用有限，但在确定检查和治疗方面可能很重要。基于综合征的分类具有无需其他系统所需的大多数假设的优点。然而，它不能涵盖癫痫的所有临床方面，也不能为癫痫发作障碍的病理生理学、遗传学和病因学提供指导。最终目标仍然是明确疾病实体。
