Jeavns综合征的临床及录像脑电图特征.doc

Jeavons综合征的临床及录像脑电图特征 刘金生张丽刘培莉 ［ ］目的分析jeavons纟宗合征的电-临床特征，提高认识，减少误诊。方 法收集2004年1月至2012年12月儿科确诊眼睑肌阵挛(EM)患儿14例，对其 录像脑电图(VEEG)及临床特点进行回顾分析。结果14例中女10例，男4例； 发病年龄4岁?7岁，10例为女性患儿，发病年龄为4岁~7岁，14例均以眼睑肌 阵挛伴或不伴失神起病，9例半年?1年后出现全面强直-阵挛发作(GTCS)和(或) 肌阵挛发作，4例因GTCS首次就诊，5例以眼睑肌阵挛伴或不伴失神为唯一发作 类型，6例围产期窒息史，3例生后1年内2次以上热性惊厥史，余正常；VEEG 监测时通过闭眼或闪光刺激(IPS)诱发，EM发作期脑电图为广泛对称同步 3Hz~6Hz棘慢波、多棘慢波阵发。结论jeavons综合征一种特发性全面性癫痫综 合征，表现为反复眼睑肌阵挛伴或不伴失祌发作，首次发作在童年早期，临床表 现形式多样，患儿及家长对存在眼部相关症状未察觉或不重视，易于误诊或漏诊, VEEG监测结合闭眼及闪光刺激试验可确诊。丙戊酸及其他新型的抗癫痫药对治 疗木病有效。jeavons综合征是一种终生性的疾病，发作有时能得到很好的控制， 发作控制不佳时对认知及智力会造成一定的损伤。 ［关键词］jeavons综合征眼脸肌阵挛失神录像脑电图 EEG characteristics of Jeavons syndrome Liu Jinsheng,Zhang Li,Liu Peili Abstract: Objective To investigate the electroencephalographic and clinical characteristics of Jeavons syndrome. Method 14 patients of eyelid myoclonus with absence seizures diagnosed in the authorrsquo;s hospital from 2004 to 2011 were collected to study their VEEG and clinical features. Result There were 10 girls and 4 boys. Age of rSseizure onset ranged from 4 to 7 years old. All the 14 Jeavons patients began their disease with eyelid myoclonus with or without absence, and among them 9 patients had generalized tonic-clonic (GTCS) and (or) myoclonic half a year to one year later, 4 visited the hospital because of GTCS, 5 were only attacked by EM or without absence, 6 had perinatal asphyxia and 3 had 2 occasions of febrile seizure one year within delivery. VEEG monitoring with eye closure and intermittent photic stimulation tests helped to induce discharges and seizure. EEG of Jeavons syndrome was characterized by generalized discharges of 3-6 Hz bilaterally symmetrical spike and ware waves or poly-spike and slow wave bursts. Conclusion Jeavons syndrome is one of the idiopathic generalized epilepsies characterized by EM with or without absence. The age of seizure onset was in early childhood, and Jeavons had various clinical manifestation forms. Since itrsquo;s not easy for children and parents to notice its syndrome, EM