儿童Jeavons综合征4例报告

Four cases report of Jeavons syndrome in childhood

目的：探讨儿童Jeavons综合征的临床、脑电图特征及药物治疗效果。方法回顾性分析4例儿童Jeavons综合征患儿的临床、脑电图特征及治疗效果等临床资料。结果4例患儿中女性3例、男性1例，起病年龄1~6岁。临床表现为反复特征性眼睑肌阵挛，伴或不伴失神发作。视频脑电图（VEEG）监测发作期脑电图，2例为全导对称同步3~6 Hz棘慢波或多棘慢波阵发，以闭眼诱发明显；2例为全导3.0~3.5 Hzδ节律发放。采用丙戊酸治疗2例、左乙拉西坦1例、丙戊酸联合左乙拉西坦1例。结果发作控制1例，发作减轻2例，发作仍频繁1例。结论 Jeavons综合征是一种特发性全面性癫综合征，发作主要为眼睑肌阵挛伴或不伴失神。VEEG对明确诊断有重要价值，丙戊酸钠或左乙拉西坦对控制发作有效。

Objective To explore the clinical manifestations, electroencephalographic characteristics and therapeutic effect of drugs in children with Jeavons syndrome. Methods The clinical and electroencephalographic characteristics and therapeutic effect of drugs were analyzed in 4 children with Jeavons syndrome. Results Among the four children there were 3 female and 1 male. The age at the onset of the disease was from 1 to 6 years. The typical clinical manifestations of this disease were brief, fast and repeated eyelid myoclonia （EM） with or without absence seizure. The typical electroencephalography （EEG） in two patients showed 3-6 Hz generalized spike and waves and polyspikes burst, and the eye closure and intermittent photic stimulation helped to induce discharges and clinical events. The typictal EEG in the other two patients showed 3.0-3.5 Hz generalizedδslow wave rhythm burst. The drugs of choice for treatment was sodium valproate monotherapy in two cases, levetiracetam in one case, sodium valproate combined with levetiracetam in one case. During the follow-up, seizures were controlled in one case, decreased in frequency in two cases and were still frequent in one case. Conclusions Jeavons syndrome is one of the idiopathic and generalized epileptic syndromes and characterized by EM with or without absence seizure. Video EEG monitoring plays an important role in the diagnosis of this disease. Sodium valproate and levetiracetam were effective for this disease.