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数据
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图1 视频脑电图
第一天:第一个脑电图进行排除nonconvulsive地位是含有过量的运动和肌肉肌电图人工制品。底层,脑电图显示弥漫性背景衰减nonrecruiting bifrontal 2-3Hz慢波(和一些峰值混杂在右frontocentral-temporal);这是部分响应抗癫痫药物。最后2通道EMG polygraphy左翼和右翼的三角肌。第三天:下周的焦点运动发作都被记录下来,与一些显示保留意识;这些都是很难描述的更加明显和侵入性运动障碍。的阵发性抽搐发作记录由下肢,有时成为两国。有人看见一个中线局灶性发作的脑电图关联(一致的大幅波在Cz(顶点)前每个阵挛]与符号学和整合。背景显示弥漫性节奏放缓峰值和慢波。最后用绿色通道是EMG polygraphy从左边显示左腿股四头肌阵挛。 Day 9: In the last recording over a 50-minute period, 14 self-terminating intermittent focal clonic seizures were recorded, involving the right lower limb. Many other movements did not have an ictal EEG correlate. The last channel in orange is EMG polygraphy showing clonus of the right foot. Preceding Cz sharp wave is seen. Overall, the EEG evolution was in keeping with a genetic developmental and epileptic encephalopathy with a movement disorder.
(一)轴向t2加权和矢状t1影像扫描获得3岁。(B)对应的图像获得14岁年渐进显示小脑萎缩(箭头),在深小脑白质异常信号和小脑中间总花梗。(C) Diffusion-weighted成像显示双边的皮质扩散限制和小脑的光谱学先生显示异常升高乳酸双重峰(红圈)。
![Video EEG Day 1: The first EEG performed to exclude nonconvulsive status was contaminated with excess movement and muscle EMG artefact. Underlying this, the EEG showed diffuse background attenuation with nonrecruiting bifrontal 2–3Hz slow waves (and some spikes intermixed over the right frontocentral-temporal); this was partially responsive to antiepileptics. The last 2 channels are EMG polygraphy from the right and left deltoid. Day 3: Over the course of the next week, focal motor seizures were recorded, with some showing retained awareness; these were very difficult to delineate from the more pronounced and intrusive movement disorder. The seizures recorded consisted of clonic jerking of either lower limb, at times becoming bilateral. A midline focal ictal EEG correlate was seen [consistent sharp wave over Cz (vertex) preceding each clonus] and concordant with the semiology. Background shows diffuse rhythmic slowing with spikes and slow waves. The last channel in green is EMG polygraphy from the left quadriceps showing left leg clonus. Day 9: In the last recording over a 50-minute period, 14 self-terminating intermittent focal clonic seizures were recorded, involving the right lower limb. Many other movements did not have an ictal EEG correlate. The last channel in orange is EMG polygraphy showing clonus of the right foot. Preceding Cz sharp wave is seen. Overall, the EEG evolution was in keeping with a genetic developmental and epileptic encephalopathy with a movement disorder.](http://www.ez-admanager.com/content/neurology/100/1/30/F1.large.jpg?width=800&height=600&carousel=1){kind=link}
