青少年肌阵挛癫痫的临床、EEG与SPECT的研究

目的：探索青少年肌阵挛癫痫（JME）的临床、脑电图（EEG）和发作间期单光子发射断层扫描（SPECT）的特点.方法：总结40例JME病例的临床、EEG和 SPECT显像等资料.结果：年龄6～32岁.病程2周至19年,平均（4.12±3.95）年.病因：家族史7例.临床表现：有肌阵挛发作性抽动40例（100%）,兼有全面性强直阵挛发作36例（90%）,兼有失神发作12例（30%）.常规EEG检查13例,正常5例,异常8例 长程EEG监测27例（V-EEG 21例,AEEG 6例）均异常,总体异常35例（87%）.异常者中,广泛性31例（89%）,局灶性异常4例（11%） 多棘慢波31例（89%）,高波幅慢波2例（6%）.发作间期SPECT正常11例（28%）,异常29例（72%）.低灌注12例（41%）,高灌注17例（59%）.异常灌注部位41个,额叶占40%.影像学异常5%.结论： 有关JME的诊断,在把握好临床表现的基础上,长程EEG监测显得很重要,SPECT显像的额叶异常灌注灶的证据也具有重要意义.

A study of the clinic,EEG and interictal SPECT in patients with juvenile myoclonic epilepsy

Objective：To explore the characteristics of clinic,EEG and interictal SPECT of juvenile myoclonie epilepsy（JME）. Methods： Clinical data of 40 patients with JME, EEG, interietai SPECT and CT/MRI were analized. Results： Age range ： 6 - 32 years old ; the disease course ： 2 weeks to 19 years （average 4.12± 3.95 years） ;family history in 7 cases;three seizure types：GTCS, myoclonic seizure and absence seizure ; abnormal EEG in 35 cases （88 %） , including extensive abnormality 89 % and focal 11 %, ab normal features including multiple spike and slow wave complex in 89 % and high amplitude waves in 60 %. All long term monitoring EgG were abnormal. Interictal SPECT imaging： normal in 28 %, abnor mal in 72% with hypoperfusion in 41 % and hypertransfusion 59 %. 40 abnormal locations of perfusion and 40 % in frontal lobe. and abnormal CT/MRI in 5 %. Conclusion： In the clinical diagnosis of J ME,long term monitoring EEG,and the data of SPECT imaging abnormal frontal lobe perfusion focus have significant value.