Niedzielska K, Kuran W, Romaniak A
Zakładu EEG/EMG, Instytutu Psychiatrii i Neurologii w Warszawie.
Neurol Neurochir Pol. 1995 Sep-Oct;29(5):675-85.
18 patients (9 females, 9 males) with juvenile myoclonic epilepsy (JME) were studied. Despite a fairly long duration of the disease (from 1 to 34 years--mean 9.5 years) the patients had not been properly diagnosed. In all cases routine serial EEG examinations, 24-hour EEG with Medilog System 9000, and polygraphic Video/EEG/EMG recording were done (Videometry Processor, Glonner). Typical attack triads occurred in 7 cases (39%), grand mal seizures and myoclonic attacks in 10 cases (56%), one patient had only myoclonic seizures. EEG demonstrated already in routine recording occurrence of seizure activity with spike/polyspike-slow wave 3-6 Hz complexes. 24-hour EEG made possible demonstration of these complexes in the remaining 4 cases. Seizure activity during clinical myoclonic seizures were recorded in 8 cases, and during absences in 2 cases. A valuable method was also Video/EEG/EMG recording which showed occurrence of both myoclonic seizures (in 5 cases) and absences (9 cases). Three patients with absences were not aware of their seizures. In 8 patients EEG findings demonstrated focal abnormalities which contributed to previous diagnostic errors. The described clinical and electrophysiological features met fully the diagnostic criteria of JME. In the Polish literature this is the first report on such a large groups of JME cases. Attention is called to diagnostic difficulties due to poor knowledge of this disease and its manifestations which leads to inaccurate history taking. Another cause is non-availability of full neurophysiological diagnostic facilities in many centers.
对18例青少年肌阵挛性癫痫(JME)患者(9例女性,9例男性)进行了研究。尽管病程较长(1至34年,平均9.5年),但这些患者此前未得到正确诊断。所有病例均进行了常规系列脑电图检查、使用Medilog System 9000的24小时脑电图检查以及多导睡眠图视频/脑电图/肌电图记录(Videometry处理器,格洛纳)。典型的发作三联征出现在7例患者中(39%),大发作和肌阵挛发作出现在10例患者中(56%),1例患者仅有肌阵挛发作。脑电图在常规记录中已显示出伴有3 - 6赫兹棘波/多棘波 - 慢波复合波的癫痫活动。24小时脑电图使其余4例患者也能显示出这些复合波。8例患者在临床肌阵挛发作时记录到癫痫活动,2例患者在失神发作时记录到癫痫活动。视频/脑电图/肌电图记录也是一种有价值的方法,它显示了肌阵挛发作(5例)和失神发作(9例)的发生情况。3例失神发作患者未意识到自己的发作。8例患者的脑电图结果显示有局灶性异常,这导致了之前的诊断错误。所描述的临床和电生理特征完全符合JME的诊断标准。在波兰文献中,这是关于如此大量JME病例的首次报告。由于对这种疾病及其表现了解不足导致病史采集不准确,从而引起了对诊断困难的关注。另一个原因是许多中心无法提供完整的神经生理学诊断设施。