Mirsky A F, Duncan C C, Myslobodsky M S
J Clin Neurophysiol. 1986 Jul;3(3):179-208.
Petit mal (absence) epilepsy remains one of the most enigmatic of neurological disorders, and there is no widely accepted theory of its etiology. This review covers some of the current issues concerned with the disorder, including treatment and prognosis, neurochemical research, behavioral and psychophysiological effects of wave-spiked discharges, and EEG studies of seizure control. With respect to treatment, although effective drug therapy (valproic acid, ethosuximide) exists for the "pure" form of absence epilepsy, other forms, in which there is an admixture of grand mal seizures, are less amenable to pharmacotherapy. Moreover, the frequency of fatal hepatic toxicity following valproic acid therapy has been estimated at 1 in 20,000. With respect to prognosis, follow-up studies indicate that many patients do not outgrow the disorder but continue to suffer absence seizures well into adulthood. In recent years, there has been considerable research on the neurochemical basis of absence epilepsy. Current theories, including those that implicate gamma-aminobutyric acid, catecholamines, and "endogenous" epileptogens, are summarized; and requirements for an experimentally induced animal model of absence epilepsy are discussed. The majority of behavioral studies of the disorder have concerned the effects of petit mal-type discharges on sensory and cognitive processes. Some of these studies are reviewed; and recent work bearing on these issues, involving event-related brain potentials, is presented. Our review concludes with a discussion of research aimed at the development of electrophysiologically based approaches to the reduction of seizure frequency in patients with absence epilepsy.
失神性癫痫（小发作）仍然是最神秘的神经系统疾病之一，其病因尚无被广泛接受的理论。本综述涵盖了与该疾病相关的一些当前问题，包括治疗和预后、神经化学研究、棘波放电的行为和心理生理影响以及癫痫发作控制的脑电图研究。关于治疗，虽然对于“单纯”形式的失神性癫痫存在有效的药物治疗（丙戊酸、乙琥胺），但其他伴有大发作混合形式的癫痫对药物治疗的反应较差。此外，丙戊酸治疗后致命性肝毒性的发生率估计为两万分之一。关于预后，随访研究表明，许多患者无法摆脱该疾病，而是在成年后仍继续遭受失神发作。近年来，对失神性癫痫的神经化学基础进行了大量研究。本文总结了当前的理论，包括那些涉及γ-氨基丁酸、儿茶酚胺和“内源性”致痫原的理论；并讨论了建立实验性失神性癫痫动物模型的要求。对该疾病的大多数行为学研究关注小发作型放电对感觉和认知过程的影响。本文回顾了其中一些研究；并介绍了近期涉及事件相关脑电位的有关这些问题的研究工作。我们的综述最后讨论了旨在开发基于电生理学方法以降低失神性癫痫患者癫痫发作频率的研究。
