Fujiwara T, Watanabe M, Nakamura H, Kudo T, Yagi K, Seino M
National Epilepsy Center, Shizuoka Higashi Hospital.
Jpn J Psychiatry Neurol. 1988 Sep;42(3):497-508. doi: 10.1111/j.1440-1819.1988.tb01343.x.
Based on the clinicoelectrographic data of 28 patients (14 children and 14 adults) with absence status epilepticus thoroughly documented by CCTV/EEG, it was found that there were significant differences between the children and adults. In childhood, absence status tended to occur in those who had experienced individual short-lived atypical absence seizures and also other types of generalized seizure. In contrast, there was a general tendency for absence status in adulthood to occur in females without individual absence seizure. With respect to the clinicoelectrographical manifestations, absence status with a decreased postural tone was prone to be associated with a more profound clouding of consciousness, whereas in those with myoclonic components there was a less profound clouding. The former was found solely in children while the latter was both in children and in adults. During absence status, the focal motor features with or without secondarily generalized convulsions were observed in 8 adult patients (57%). On the other hand, no focal motor manifestations were observed during absence status in children. Absence status is composed of two modalities: either a prolongation or repetition of absence seizures. It was demonstrated that, in children, either the prolongation or repetition of individual absence seizures developed into absence status. The short-lived absence was of an atypical nature, whereas in adults, absence status consisted of the prolongation of an absence seizure which occurred in patients with no experience of individual absence seizures. With respect to the drug treatment, antiabsence drugs had some effect in children, while none of the adult patients responded either to the antiabsence drugs or other antiepileptic drugs. The long-term seizure prognosis was not necessarily poor in children but invariably poor in adults. Four adult patients showed diffuse, but unilateral frontally accentuated asymmetrical paroxysmal activity during the status. Three of them showed initial localized spike-wave discharges in the unilateral frontal region followed by a generalized spike-wave rhythm. Furthermore, all of these 4 patients with focally accentuated ictal EEGs have shown partial motor seizures intermingled with absence status.(ABSTRACT TRUNCATED AT 400 WORDS)
基于28例失神癫痫持续状态患者（14名儿童和14名成人）的临床脑电图数据，这些数据经闭路电视/脑电图全面记录，发现儿童和成人之间存在显著差异。在儿童期，失神癫痫持续状态倾向于发生在曾经历过个别短暂性非典型失神发作以及其他类型全身性发作的患者中。相比之下，成年期失神癫痫持续状态总体上倾向于发生在无个别失神发作的女性中。关于临床脑电图表现，伴有姿势张力降低的失神癫痫持续状态更容易伴有意识更深度的模糊，而伴有肌阵挛成分的患者意识模糊程度较轻。前者仅在儿童中发现，而后者在儿童和成人中均有。在失神癫痫持续状态期间，8名成年患者（57%）观察到有或无继发性全身性惊厥的局灶性运动特征。另一方面，儿童失神癫痫持续状态期间未观察到局灶性运动表现。失神癫痫持续状态由两种形式组成：失神发作的延长或重复。结果表明，在儿童中，个别失神发作的延长或重复发展为失神癫痫持续状态。短暂性失神是非典型性的，而在成人中，失神癫痫持续状态由无个别失神发作经历的患者中发生的失神发作延长组成。关于药物治疗，抗失神药物对儿童有一定效果，而成年患者对抗失神药物或其他抗癫痫药物均无反应。儿童的长期癫痫预后不一定差，但成人则总是很差。4名成年患者在癫痫持续状态期间表现出弥漫性但单侧额叶突出的不对称阵发性活动。其中3名患者最初在单侧额叶区域出现局灶性棘波放电，随后出现全身性棘波节律。此外，所有这4名脑电图发作期局灶性突出的患者均表现出部分运动性发作与失神癫痫持续状态相混合。（摘要截选至400字）
