Yuan Yujun, Yang Fenghua, Huo Liang, Fan Yuying, Liu Xueyan, Wu Qiong, Wang Hua
Department of Pediatrics, Shengjing Hospital of China Medical University, Shenyang, China.
Front Pediatr. 2021 Apr 22;9:671732. doi: 10.3389/fped.2021.671732. eCollection 2021.
Eyelid myoclonus with or without absence epilepsy is a rare and usually misdiagnosed disease in the neurology department. It is an idiopathic general epileptic syndrome, the onset period is 6-8 years, and is more common in girls. It is characterized by rapid abnormal eye blinking, accompanied by upward rolling of the eye and slight backward movement of the head, with eye closure sensitivity and photosensitivity. The seizure is frequent and short, dozens or even hundreds of times a day; a small number of patients may have eyelid myoclonus status. We report a patient who visits the hospital for the first time with eyelid myoclonic problem; the patient continued to wink the eyes, eye rolled up, and backward movement of the head, accompanied by impairment of consciousness. Video electroencephalography (VEEG) suggests continued spike slow-wave, polyspike slow-wave. After the patient had 2, 4, 6, 8, 10, 12, and 14 Hz of intermittent photic stimulation (IPS), her seizures and epileptic discharges reduced or stopped. Seven min after giving stimulation at 20 Hz, the child developed an occipital-initiated tonic-clonic seizure, which demonstrated that after sufficient IPS stimulation, the occiput cortex became excited and initiated a brain network, leading to diffuse brain discharge and tonic-clonic seizures. At 1 h after onset, the child developed a nonconvulsive state, with impairment of consciousness despite no eyelid myoclonic movements, and VEEG suggested a large number of epileptic discharges. After 10 min of administrating midazolam, the patient's EEG immediately became normal, and the patient regained consciousness. Therefore, this paper presents an eyelid myoclonus status patient with occipital origin seizure, we recorded the whole course of the disease and the treatment effect, and reviewed the literature accordingly.
伴或不伴失神发作的眼睑肌阵挛是神经内科一种罕见且常被误诊的疾病。它是一种特发性全身性癫痫综合征,发病年龄为6 - 8岁,多见于女孩。其特征为快速异常眨眼,伴有眼球上翻和头部轻微后移,存在闭眼敏感和光敏感现象。发作频繁且短暂,每天发作数十次甚至上百次;少数患者可能出现眼睑肌阵挛持续状态。我们报告1例因眼睑肌阵挛问题首次就诊的患者;该患者持续眨眼、眼球上翻及头部后移,伴有意识障碍。视频脑电图(VEEG)提示持续棘慢波、多棘慢波。患者在接受2、4、6、8、10、12和14Hz的间歇性光刺激(IPS)后,发作及癫痫放电减少或停止。在给予20Hz刺激7分钟后,患儿出现枕叶起始的强直 - 阵挛发作,这表明在充分的IPS刺激后,枕叶皮质兴奋并启动脑网络,导致全脑放电和强直 - 阵挛发作。发作后1小时,患儿出现非惊厥状态,虽无眼睑肌阵挛动作但有意识障碍,VEEG提示大量癫痫放电。给予咪达唑仑10分钟后,患者脑电图立即恢复正常,患者意识恢复。因此,本文报告1例伴枕叶起源发作的眼睑肌阵挛持续状态患者,记录了疾病全过程及治疗效果,并相应复习了文献。