眼睑肌阵挛失神综合征

Eyelid myoclonia with absences: two cases report and review of literature

目的探讨眼睑肌阵挛失神的临床、脑电图特点及治疗。方法回顾性分析我院诊治的2例眼睑肌阵挛失神患者的临床资料,并复习文献。结果2例均儿童起病,表现为反复的眼睑肌阵挛,伴或不伴短暂失神,脑电图特征为泛化性的双侧对称的3～6Hz棘慢波或多棘慢波发放,常由特征性的闭眼或闪光刺激诱发或发作加重和时间延长。结论眼睑肌阵挛失神由于症状复杂和抗癫痫药物对脑电图变化的遮蔽,常被漏诊。Video-EEG对眼睑肌阵挛失神的诊断是必需的。本病需长期适当的抗癫痫药物治疗。

Objective To explore the clinical manifestations, electroencephalography(EEG) findings and treatment of eyelid myoclonia with absences (EMA). Methods The clinical data of two patients with eyelid myoclonia with absences were analyzed retrospectively. Results Both two patients onsetted in childhood, and presented recurrance of eyelid myoclonia with or without transient absences. EEG features of two patients were generalized discharges of paroxysmal 3～6 Hz polyspikes and slow waves which were brief and precipitated primarily by eye closure and photosensitivity. Conclusion Eyelid myoclonia with absences is likely to be misdiagnosed due to complicated symptoms and masking effect of EEG changes by anti- epileptic drugs. Video-EEG is necessary to diagnosis of EMA. The patients should take anti- epileptic drugs for long time.