基于立体定向脑电图的难治性惊吓发作致痫网络的研究

目的研究难治性惊吓癫痫的电-临床特点，探讨惊吓发作的致痫网络。方法回顾性分析2014年1月—2020年8月清华大学玉泉医院收治的经立体定向脑电图（SEEG）置入的4例难治性惊吓发作术后无发作患者，分析其电-临床特点，通过发作期SEEG数据研究其发作起源及致痫网路。结果4例患者以声音诱发发作为主，除病例1为单侧肢体感觉先兆-强直发作外，余3例患者均以轴肌、肢体近端肌肉短暂强直为主。发作期脑电图（EEG）均为广泛性电压抑制或伪差掩盖EEG，无侧向性和定位性提示。4例患者SEEG发作期起始均以相对广泛低波幅快活动起始，其中病例1为围外侧裂周围多微小脑回多触点略提前，病例2为左侧颞横回-岛叶后部略提前，病例3为左侧辅助感觉运动区（SMA）、中扣带回略提前，而病例4为左侧岛叶后部-颞顶盖电极略提前。高频致痫指数脑表面图显示惊吓发作致痫网路累及额叶内侧面、运动区-运动前区皮质、岛叶-岛盖皮质。节律性声音刺激或讲话均可诱发颞横回响应，颞横回诱发电位波幅150~400 μV。结论惊吓发作可以起源于额叶内侧面SMA，也可以起源岛叶-外侧裂周围皮质，其致痫网络主要累及额叶内侧面、运动区-运动前区皮质、岛叶-岛盖皮质。惊吓发作可能存在两种不同的致痫网络机制。

Objective To study the electro-clinical characteristics of intractable startle epilepsy, and to explore the epileptogenic network of startle seizures. Methods The electro-clinical characteristics of 4 patients admitted to Tsinghua University Yuquan Hospital from January 2014 to August 2020 with refractory startle epilepsy who had no seizures after operation were analyzed retrospectively. The seizure origin and epileptogenic network were studied by the data of stereotactic electroencephalography(SEEG). Results Four patients were mainly induced by voice. Except case 1, which was unilateral limb sensory aura-tonic seizure, the other 3 cases were characterized by transient axial muscle and proximal limb muscle rigidity. During the seizure period, the electroencephalogram(EEG) showed generalized voltage suppression or artifact masking EEG, without lateral and location cues. The onset of SEEG in 4 patients all started with relatively diffused low voltage fast activity. In case 1, multiple around peri-insular micro gyri were slightly advanced. In case 2, left posterior insula and transverse temporal gyrus was slightly advanced. In case 3, left supplementary sensorimotor area(SMA) and middle cingulate gyrus were slightly advanced. And in case 4, left posterior insular-opercular cortex was slightly advanced. High frequency epileptogenicgraphy showed that the seizure network involved the medial frontal lobe, motor-premotor cortex and peri-insulaopercular cortex. Rhythmic sound stimulation or speech could induce the response of transverse temporal gyrus, and the amplitude of evoked potential of transverse temporal gyrus was 150-400 μV. ConclusionsStartle seizure may originate from SMA in the medial frontal lobe, or from the cortex peri-insular auditory cortex. The epileptogenic network of startle seizure mainly involves the medial frontal lobe, the motor premotor cortex, and the insular-opercular cortex. There may be two different epileptogenic network mechanisms in startle seizure.