Usui Naotaka, Kotagal Prakash, Matsumoto Riki, Kellinghaus Christoph, Lüders Hans Otto
Sections of Pediatric and Adult Epilepsy, Department of Neurology, The Cleveland Clinic Foundation, Cleveland, OH 44195, USA.
Epilepsia. 2005 Oct;46(10):1668-76. doi: 10.1111/j.1528-1167.2005.00262.x.
A few reports have described focal electroencephalographic or clinical features or both of juvenile myoclonic epilepsy (JME), but without video-EEG documentation. We examined focal clinical and EEG features in patients with JME who underwent video-EEG monitoring.
Twenty-six patients (nine males and 17 females) who had seizures recorded during video-EEG monitoring were included. Age at seizure onset was 0 to 22 years (mean, 12.3 years), and age at monitoring was 12 to 44 years (mean, 26.5 years). In one patient with left parietooccipital epilepsy, primary generalized tonic-clonic seizures developed after resection of the parietal tumor. Two patients had both temporal lobe epilepsy and JME. Videotaped seizures in each patient were analyzed. Interictal and ictal EEG also were analyzed for any focal features.
Focal semiologic features were observed in 12 (46%) of 26 patients. Six patients had focal myoclonic seizures, and two had Figure 4 sign: one with version to the left, and another had left version followed by Figure 4 sign, and left arm clonic seizure. Their ictal EEGs were generalized at onset but with a lateralized evolution over the right hemisphere. The patient who had both JME and left parietooccipital epilepsy, right arm clonic seizure, and Figure 4 sign was seen during a generalized EEG seizure. Interictally, one patient had temporal sharp waves, and another had run of spikes in the right frontal region.
Fourteen (54%) of 26 patients with JME exhibited focal semiologic or electroencephalographic features or both. Video-EEG was essential in reaching a correct diagnosis and choosing an appropriate antiepileptic drug regimen.
有几份报告描述了青少年肌阵挛癫痫（JME）的局灶性脑电图或临床特征或两者兼有，但均无视频脑电图记录。我们对接受视频脑电图监测的JME患者的局灶性临床和脑电图特征进行了研究。
纳入26例在视频脑电图监测期间有发作记录的患者（9例男性，17例女性）。发作起始年龄为0至22岁（平均12.3岁），监测时年龄为12至44岁（平均26.5岁）。1例左顶枕叶癫痫患者在切除顶叶肿瘤后出现原发性全面性强直阵挛发作。2例患者同时患有颞叶癫痫和JME。对每位患者的发作录像进行分析。还分析了发作间期和发作期脑电图的任何局灶性特征。
26例患者中有12例（46%）观察到局灶性症状学特征。6例患者有局灶性肌阵挛发作，2例有图4征：1例向左扭转，另1例先向左扭转，随后出现图4征，并伴有左臂阵挛发作。他们的发作期脑电图起始时为全面性，但在右半球有侧化演变。在一次全面性脑电图发作期间观察到1例同时患有JME和左顶枕叶癫痫、右臂阵挛发作及图4征的患者。发作间期，1例患者有颞叶尖波，另1例在右额叶区域有一连串棘波。
26例JME患者中有14例（54%）表现出局灶性症状学或脑电图特征或两者兼有。视频脑电图对于做出正确诊断和选择合适的抗癫痫药物治疗方案至关重要。
