Oguni Hirokazu, Hirano Yoshiko, Nagata Satoru
Department of Pediatrics, Tokyo Women's Medical University, Tokyo.
Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Yotsugi Medical Center for the Severely Disabled, Tokyo, Japan.
Epileptic Disord. 2020 Feb 1;22(1):67-72. doi: 10.1684/epd.2020.1128.
We report two patients with Panayiotopoulos syndrome (PS) who developed encephalopathy related to status epilepticus during slow sleep (ESES) at the peak of their clinical course. Clinical charts and EEG data were reviewed. The patients exhibited nocturnal autonomic seizures and occipital EEG foci, the latter of which later evolved into multifocal EEG foci with synchronous frontopolar and occipital spikes (Fp-O EEG foci), and finally into continuous spikes-waves during sleep (CSWS; spike-wave index >85% based on whole-night sleep recording) at eight years and seven years of age, respectively. The occipital spikes always preceded frontopolar spikes by 30∼50 mseconds based on the analysis of CSWS. Neuropsychological ability, including IQ, deteriorated during the CSWS period in both patients. The autonomic seizures and focal to bilateral tonic-clonic seizures were initially resistant to antiepileptic drugs (AEDs), and occurred more than 10 times in both patients. However, the seizures and EEG findings gradually resolved, and AEDs were successfully terminated in both patients. PS can progress to ESES if the clinical course exhibits atypical evolution. The initial autonomic symptom of the seizures and interictal Fp-O EEG foci should be carefully monitored in patients with CSWS or ESES.
我们报告了两名患有帕纳约托普洛斯综合征（PS）的患者，他们在临床病程高峰期的慢波睡眠期癫痫持续状态（ESES）期间出现了与脑病相关的症状。我们回顾了临床病历和脑电图数据。这两名患者均表现出夜间自主神经性发作和枕叶脑电图病灶，后者随后演变为多灶性脑电图病灶，并伴有同步的额极和枕叶棘波（Fp-O脑电图病灶），最终分别在8岁和7岁时发展为睡眠期持续棘慢波（CSWS；基于整夜睡眠记录的棘慢波指数>85%）。根据对CSWS的分析，枕叶棘波总是比额极棘波提前30至50毫秒出现。在CSWS期间，两名患者的神经心理能力，包括智商，均出现恶化。自主神经性发作和局灶性至双侧强直阵挛性发作最初对抗癫痫药物（AEDs）耐药，且两名患者的发作次数均超过10次。然而，发作和脑电图表现逐渐缓解，两名患者的AEDs均成功停用。如果临床病程呈现非典型演变，PS可进展为ESES。对于患有CSWS或ESES的患者，应仔细监测发作的初始自主神经症状和发作间期Fp-O脑电图病灶。
