Department of Neuropediatrics, Tokyo Metropolitan Neurological Hospital, Fuchu, Tokyo, Japan.
Department of Neurology, Kyoto University Graduate School of Medicine, Sakyo-ku, Kyoto, Japan.
Epileptic Disord. 2023 Jun;25(3):416-421. doi: 10.1002/epd2.20047. Epub 2023 May 24.
This report documents the clinical features of supplementary motor area seizures with voluntary movements in two patients. The first case describes a 13-year-old boy with a 2-year history of nocturnal seizures, characterized by an asymmetrical brief tonic posture followed by bilateral rapid hand shaking, but without impaired awareness. Magnetic resonance imaging revealed no abnormalities. Video electroencephalogram indicated interictal focal spikes and ictal activity 2 s before clinical onset in the frontal midline area. The patient stated that he purposely shook his hands to lessen the seizure-induced upper limb stiffness. The second case describes a 43-year-old man with a 33-year history of nocturnal seizures, characterized by an asymmetric brief tonic posture, with the right hand grabbing to hold this posture, but without impaired awareness. Video electroencephalogram indicated that he voluntarily moved his right hand during the latter part of the seizures; however, no clear ictal electroencephalogram change was noted. Magnetic resonance imaging revealed a mass lesion in the right medial superior frontal gyrus. Fluorodeoxyglucose-positron emission tomography and ictal single-photon emission computed tomography indicated ictal focus in the mesial frontal area, as confirmed by invasive electroencephalogram and seizure freedom after surgery. Both patients had typical supplementary motor area seizures, except they could perform voluntary movements in the body parts. The co-occurrence of supplementary motor area seizures and voluntary movements is clinically useful, as it may help avoid the inaccurate and misleading diagnosis of non-epileptic events such as psychogenic non-epileptic seizures.
本报告记录了两名患者伴随意运动的辅助运动区发作的临床特征。第一例描述了一名 13 岁男孩，有 2 年夜间发作史，表现为非对称短暂强直姿势，随后双侧快速手抖动，但意识无障碍。磁共振成像无异常。视频脑电图显示发作间期额中线区棘波和发作前 2s 的发作期活动。患者表示，他故意晃动双手以减轻因癫痫发作引起的上肢僵硬。第二例描述了一名 43 岁男性，有 33 年夜间发作史，表现为非对称短暂强直姿势，右手抓握以保持此姿势，但意识无障碍。视频脑电图显示，在发作后期，他自愿移动右手；然而，没有明显的脑电图发作改变。磁共振成像显示右侧额上回内侧有一肿块病变。氟脱氧葡萄糖正电子发射断层扫描和发作期单光子发射计算机断层扫描显示额中线区的发作灶，经有创脑电图和手术后癫痫无发作证实。两名患者均有典型的辅助运动区发作，只是身体部位可以进行随意运动。辅助运动区发作伴随意运动的共存具有临床意义，因为它可能有助于避免非癫痫性事件（如心因性非癫痫性发作）的不准确和误导性诊断。
