Peng Xi, Chen Yangmei, Wang Zezhi, Zhang Xinbo, Wang Bi, Jin Lang, Wang Xiaoli, Yuan Na, Hu Xiaojing, Wang Xiaomu, Liu Yonghong
Department of Neurology, Xijing Hospital of the Air Force Military Medical University, Xi'an, Shaanxi, 710032, China.
Department of Neurology, The Second Affiliated Hospital of Chong Qing Medical University, Chongqing, 400010, China.
Acta Epileptol. 2024 Aug 1;6(1):25. doi: 10.1186/s42494-024-00158-3.
Epileptic spasms followed by a tonic component have been frequently observed in patients with late-onset spasms (LOS). However, there is a lack of comprehensive analysis and summary of clinical data related to tonic spasms (TS), including seizures, video-electroencephalogram (V-EEG), synchronous electromyography (EMG) and follow-up data.
To investigate the characteristics of TS, we prospectively collected the clinical data, including 24-h V-EEG and synchronous EMG data of 32 enrolled patients who suffered from epileptic spasms followed by a TS onset at least once during the 24-h V-EEG in the epilepsy center of Xijing Hospital between June 2015 and July 2020. The patients were prescribed anti-seizure medications (ASMs) and followed up for 2-7 years.
The average age of epilepsy onset was 48.06 ± 16.07 months (range: 25 to 88 months). Among the enrolled patients, 22 patients presented with mild intellectual deficits. During the 24-h video-EEG monitoring, an average of 6.94 TS events (range: 3 to 21) were recorded, and these TS seizures often occurred in clusters. In addition to TS, 26 patients experienced generalized tonic-clonic seizures (GTCS), atypical absence seizures, myoclonic seizure, and epileptic spasms. None of the 32 patients with TS displayed hypsarrhythmia during the 24-h video-EEG recording. A total of 28 patients showed normal EEG backgrounds. Interictal epileptic discharges, including slow waves (SW), spike/sharp slow waves (SSW), and spikes, often displayed multifocally. Notably, two patients achieved seizure freedom for more than 2 years through monotherapy with oxcarbazepine (OXC), which was associated with normalization of the EEG.
It is difficult to classify the patients with TS as any existing epileptic syndromes, which were distinct from West syndrome or Lennox-Gastaut syndrome. TS might be an underreported seizure type and further studies are needed to gain a more comprehensive understanding of the electro-clinical features and appropriate choice of ASMs for treating tonic spasms.
迟发性痉挛（LOS）患者中经常观察到癫痫性痉挛后伴有强直成分。然而，缺乏对与强直痉挛（TS）相关的临床数据的全面分析和总结，包括发作、视频脑电图（V-EEG）、同步肌电图（EMG）和随访数据。
为了研究TS的特征，我们前瞻性地收集了临床数据，包括2015年6月至2020年7月在西京医院癫痫中心接受24小时V-EEG监测期间至少有一次TS发作的32例癫痫性痉挛患者的24小时V-EEG和同步EMG数据。患者接受抗癫痫药物（ASMs）治疗，并随访2至7年。
癫痫发作的平均年龄为48.06±16.07个月（范围：25至88个月）。在纳入的患者中，22例有轻度智力缺陷。在24小时视频脑电图监测期间，平均记录到6.94次TS发作（范围：3至21次），这些TS发作常成簇出现。除TS外，26例患者还经历了全身强直阵挛发作（GTCS）、非典型失神发作、肌阵挛发作和癫痫性痉挛。32例TS患者在24小时视频脑电图记录期间均未显示高度失律。共有28例患者脑电图背景正常。发作间期癫痫放电，包括慢波（SW）、棘慢波（SSW）和棘波，常呈多灶性显示。值得注意的是，两名患者通过奥卡西平（OXC）单药治疗实现了超过2年的无发作，这与脑电图正常化有关。
将TS患者归类为任何现有的癫痫综合征都很困难，这些综合征与West综合征或Lennox-Gastaut综合征不同。TS可能是一种报告不足的发作类型，需要进一步研究以更全面地了解其电临床特征以及治疗强直痉挛的ASMs的适当选择。
