Tiacci C, D'Alessandro P, Cantisani T A, Bartocci A, Ibba A, Ferroni A
Unità Organica di Neurofisiopatologia, Ospedale Policlinico, Perugia, Italy.
Ital J Neurol Sci. 1995 Jun;16(5):279-94. doi: 10.1007/BF02249103.
In 1945, Lennox was the first to describe the epileptic states mainly expressed by various degrees of consciousness disturbance, which have their onset in children who present epileptic absences correlated with ictal EEG patterns of spike-wave complex discharges at about 3 Hz. As the clinical picture seemed to be similar to an uninterrupted series of absences, this led to the definition "Petit Mal Status" (PMS). Many authors have subsequently reported that PMS can occur in epileptic subjects who have never presented absences (and even in subjects without a previous history of epilepsy) and that the related EEG pictures were characterised by paroxysmal generalized activity of various morphology, but hardly ever consisted of the continuous rhythmic spike-wave or polyspike-wave complexes at 3 Hz found in petit mal absences. Finally, in reporting the onset and recurrence of this condition typically in adults and the elderly, some authors have proposed the existence of a particular form of PMS (dependent on different types of pathologic factors and characterising a specific syndrome of this age) that is different from that of the "real PMS" typical of childhood and related to petit mal absences. This paper describes fifteen patients in whom the onset of the condition occurred at different ages, and who seem to exemplify the various possible clinical expressions of PMS, with the aim of making a contribution towards the better nosographic definition of this epileptic condition. On the basis of our study, we sustain that the so-called PMS is a seizure type of Idiopathic Generalized Epilepsy which may appear at nearly all ages, and may occur in isolation or in association with other epileptic manifestations, but cannot itself be considered as characterising one or more age-dependent syndromes.
1945年,伦诺克斯首次描述了主要以不同程度意识障碍为表现的癫痫状态,其发病于儿童,这些儿童出现癫痫失神发作,发作期脑电图模式为约3赫兹的棘慢复合波放电。由于临床表现似乎类似于一系列不间断的失神发作,这导致了“癫痫小发作状态”(PMS)这一定义。许多作者随后报告称,PMS可发生于从未出现过失神发作的癫痫患者(甚至是既往无癫痫病史的患者),且相关脑电图表现以各种形态的阵发性广泛性活动为特征,但几乎从不包括癫痫小发作失神中所见的3赫兹连续节律性棘慢或多棘慢复合波。最后,在报告这种情况通常在成人和老年人中的起病及复发时,一些作者提出存在一种特殊形式的PMS(取决于不同类型的病理因素,且是该年龄特定综合征的特征),它不同于典型的儿童期“真正PMS”,且与癫痫小发作失神有关。本文描述了15例患者,其病情在不同年龄起病,似乎体现了PMS的各种可能临床表型,目的是为更好地对这种癫痫状况进行疾病分类定义做出贡献。基于我们的研究,我们认为所谓的PMS是特发性全身性癫痫的一种发作类型,它几乎可出现在所有年龄段,可单独出现或与其他癫痫表现合并出现,但它本身不能被视为一种或多种年龄依赖性综合征的特征。