Ochoa-Urrea Manuela, Lacuey Nuria, Vilella Laura, Zhu Liang, Jamal-Omidi Shirin, Rani M R Sandhya, Hampson Johnson P, Dayyani Mojtaba, Hampson Jaison, Hupp Norma J, Tao Shiqiang, Sainju Rup K, Friedman Daniel, Nei Maromi, Scott Catherine, Allen Luke, Gehlbach Brian K, Reick-Mitrisin Victoria, Schuele Stephan, Ogren Jennifer, Harper Ronald M, Diehl Beate, Bateman Lisa M, Devinsky Orrin, Richerson George B, Zhang Guo-Qiang, Lhatoo Samden D
National Institute of Neurological Disorders and Stroke Center for Sudden Unexpected Death in Epilepsy Research (CSR), McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, United States.
Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, United States.
Front Neurol. 2021 Feb 12;12:643916. doi: 10.3389/fneur.2021.643916. eCollection 2021.
Seizure clusters may be related to Sudden Unexpected Death in Epilepsy (SUDEP). Two or more generalized convulsive seizures (GCS) were captured during video electroencephalography in 7/11 (64%) patients with monitored SUDEP in the MORTEMUS study. It follows that seizure clusters may be associated with epilepsy severity and possibly with SUDEP risk. We aimed to determine if electroclinical seizure features worsen from seizure to seizure within a cluster and possible associations between GCS clusters, markers of seizure severity, and SUDEP risk. Patients were consecutive, prospectively consented participants with drug-resistant epilepsy from a multi-center study. Seizure clusters were defined as two or more GCS in a 24-h period during the recording of prolonged video-electroencephalography in the Epilepsy monitoring unit (EMU). We measured heart rate variability (HRV), pulse oximetry, plethysmography, postictal generalized electroencephalographic suppression (PGES), and electroencephalography (EEG) recovery duration. A linear mixed effects model was used to study the difference between the first and subsequent seizures, with a level of significance set at < 0.05. We identified 112 GCS clusters in 105 patients with 285 seizures. GCS lasted on average 48.7 ± 19 s (mean 49, range 2-137). PGES emerged in 184 (64.6%) seizures and postconvulsive central apnea (PCCA) was present in 38 (13.3%) seizures. Changes in seizure features from seizure to seizure such as seizure and convulsive phase durations appeared random. In grouped analysis, some seizure features underwent significant deterioration, whereas others improved. Clonic phase and postconvulsive central apnea (PCCA) were significantly shorter in the fourth seizure compared to the first. By contrast, duration of decerebrate posturing and ictal central apnea were longer. Four SUDEP cases in the cluster cohort were reported on follow-up. Seizure clusters show variable changes from seizure to seizure. Although clusters may reflect epilepsy severity, they alone may be unrelated to SUDEP risk. We suggest a stochastic nature to SUDEP occurrence, where seizure clusters may be more likely to contribute to SUDEP if an underlying progressive tendency toward SUDEP has matured toward a critical SUDEP threshold.
癫痫发作丛集可能与癫痫性猝死（SUDEP）有关。在MORTEMUS研究中，11例接受监测的SUDEP患者中有7例（64%）在视频脑电图检查期间出现了两次或更多次全面性惊厥发作（GCS）。由此可见，癫痫发作丛集可能与癫痫严重程度相关，也可能与SUDEP风险相关。我们旨在确定在一个丛集中，从一次发作到下一次发作，电临床发作特征是否会恶化，以及GCS丛集、发作严重程度标志物和SUDEP风险之间可能存在的关联。患者是来自一项多中心研究的连续、前瞻性同意参与的耐药性癫痫患者。癫痫发作丛集被定义为在癫痫监测单元（EMU）进行长时间视频脑电图记录期间，24小时内出现两次或更多次GCS。我们测量了心率变异性（HRV）、脉搏血氧饱和度、体积描记法、发作后全面性脑电图抑制（PGES）和脑电图（EEG）恢复持续时间。使用线性混合效应模型研究首次发作与后续发作之间的差异，显著性水平设定为<0.05。我们在105例患者中识别出112个GCS丛集，共285次发作。GCS平均持续48.7±19秒（平均49秒，范围2 - 137秒）。184次（64.6%）发作出现了PGES，38次（13.3%）发作出现了惊厥后中枢性呼吸暂停（PCCA）。从一次发作到下一次发作，发作特征如发作期和惊厥期持续时间的变化似乎是随机的。在分组分析中，一些发作特征出现了显著恶化，而另一些则有所改善。与第一次发作相比，第四次发作时的阵挛期和惊厥后中枢性呼吸暂停（PCCA）明显缩短。相比之下，去大脑强直姿势持续时间和发作期中枢性呼吸暂停持续时间更长。随访期间报告了丛集队列中的4例SUDEP病例。癫痫发作丛集在每次发作之间表现出可变的变化。虽然丛集可能反映癫痫严重程度，但它们本身可能与SUDEP风险无关。我们认为SUDEP的发生具有随机性，如果存在潜在的向SUDEP发展的渐进趋势已经达到临界SUDEP阈值，那么癫痫发作丛集可能更有可能导致SUDEP。
