Zhou Zongpu, Gong Pan, Jiao Xianru, Niu Yue, Xu Zhao, Qin Jiong, Yang Zhixian
Department of Pediatrics, Peking University People's Hospital, Beijing, China; Epilepsy Center, Peking University People's Hospital, Beijing, China.
Department of Pediatrics, Peking University First Hospital, Beijing, China.
Clin Neurophysiol. 2024 Aug;164:24-29. doi: 10.1016/j.clinph.2024.04.011. Epub 2024 Apr 23.
To test the hypothesis that myoclonic seizures can evolve to tonic seizures, we documented the electroclinical features of this under-recognized seizure type.
We observed a distinct seizure pattern starting with myoclonus without returning to an interictal state, which subsequently evolved into generalized tonic seizures. The detailed symptomatic and electroencephalographic characteristics of this seizure were extracted, and the clinical manifestations, drug curative responses in patients with this seizure were reviewed and analyzed.
The onset of all seizures was characterized by a preceding period of myoclonus and bursts of generalized spike or poly-spike slow wave discharges with high amplitude. This was closely followed by the occurrence of tonic seizures, which were distinguished by bursts of generalized fast activity at 10 Hz or higher frequency. This under-recognized seizure type has been designated as myoclonic-to-tonic (MT) seizure. The number of patients identified with MT seizures in this study was 34. The prevalence rate of MT seizures was found to be higher in males. While MT seizures typically included a tonic component, it should be noted that some patients experiencing this seizure type never presented with isolated tonic seizures. Generalized Epilepsy not further defined (GE) accounted for approximately one-third of the diagnosed cases, followed by Lennox-Gastaut syndrome and Epilepsy with Myoclonic-Atonic seizures. In comparison to other types of epilepsy, GE with MT seizures demonstrated a more favorable prognosis.
The classification of myoclonic-to-tonic seizure represents a novel approach in comprehending the ictogenesis of generalized seizures and can provide valuable assistance to clinicians in epilepsy diagnosis.
为验证肌阵挛性发作可演变为强直性发作这一假说,我们记录了这种未被充分认识的发作类型的电临床特征。
我们观察到一种独特的发作模式,始于肌阵挛且未恢复到发作间期状态,随后演变为全身性强直性发作。提取了该发作的详细症状和脑电图特征,并回顾分析了该发作患者的临床表现及药物治疗反应。
所有发作均始于一段肌阵挛期,并伴有高波幅的全身性棘波或多棘慢波发放。随后紧接着出现强直性发作,其特征为10Hz或更高频率的全身性快速活动发放。这种未被充分认识的发作类型被命名为肌阵挛 - 强直性(MT)发作。本研究中确诊为MT发作的患者有34例。发现MT发作在男性中的患病率更高。虽然MT发作通常包括强直性成分,但应注意的是,一些经历这种发作类型的患者从未出现过孤立的强直性发作。未进一步明确的全身性癫痫(GE)约占确诊病例的三分之一,其次是Lennox - Gastaut综合征和肌阵挛 - 失张力性发作癫痫。与其他类型的癫痫相比,伴有MT发作的GE预后更佳。
肌阵挛 - 强直性发作的分类是理解全身性发作痫性发作机制的一种新方法,可为临床医生进行癫痫诊断提供有价值的帮助。