摘要：

目的 总结儿童发作性运动诱发性运动障碍的视频脑电图特点及其临床意义。 方法 回顾分析9 例发作性运动诱发性运动障碍患儿的视频脑电图资料。 结果 9 例患儿中男性8 例、女性1 例，年龄6.25 ~ 15.17 岁、平均（7.10 ± 3.24）岁，病程1 ~ 12 个月、平均（6.12 ± 2.58）个月。9 例患儿共监测到45 次临床发作，发作持续时间5 ~ 35 s、平均（9.21 ± 4.35）s，临床表现为舞蹈样动作并手足徐动10 次、肌张力障碍并站立不稳6 次、舞蹈样动作并手足徐动和肌张力障碍并站立不稳29 次；均由突发性运动诱发，其中2 例（2/9）亦由过度换气诱发、1 例（1/9）由惊吓刺激诱发；4 例（4/9）发作前存在肢体僵硬、肢体麻木或其他感觉异常等先兆。9 例患儿发作期呈现正常背景节律或被大量运动伪差覆盖，未见样放电、背景节律改变或局限性慢波节律等异常征象；发作间期背景活动正常，2 例（2/9）呈非特异性异常，表现为额区或枕区间断性慢活动，1 例（1/9）可见中央?颞区（Rolandic 区）棘波。5 例患儿（5/9）富脯氨酸跨膜蛋白2（PRRT2）基因突变阳性。 结论 儿童发作性运动诱发性运动障碍发作间期视频脑电图可见非特异性异常和样放电，发作期临床表现和同步脑电图对明确诊断意义重大。

关键词: 运动障碍, 脑电描记术, 儿童

Abstract:

Objective  To investigate the features and clinical significance of video electroencephalogram (VEEG) about paroxysmal kinesigenic dyskinesia (PKD) in children. Methods  The clinical data of 9 children with PKD in our hospital from January 2015 to January 2017 were reviewed and analyzed.  Results  Nine patients were 8 boys and one girl, with age of 6.25-15.17 years, average (7.10 ± 3.24) years, and duration 1-12 months, average (6.12 ± 2.58) months. All patients were monitored 45 times of clinical paroxysms, with duration lasting for 5-35 s, average (9.21 ± 4.35) s. Clinical manifestations included 10 times of simple choreoathetosis, 6 times of dystonia and unsteady stand, 29 times of choreoathetosis, dystonia and unsteady stand. Clinical paroxysms of all patients were induced by sudden movement, among whom 2 cases (2/9) were induced by hyperventilation and one case (1/9) by starle. Four cases (4/9) had aura such as rigidity and numbness of limbs and paraesthesia before the onset. The ictal VEEG showed normal background rhythm or was covered by a large number of motion artifacts. Abnormal signs such as epileptic discharges, background rhythm changes or local slow waves were not seen. During interictal phase, background activities were normal. Two cases (2/9) showed nonspecific abnormalities, including frontal or occipital discontinuous slow waves, one case (1/9) showed centro?temporal (Rolandic area) spikes. Five cases (5/9) were positive for proline-rich transmembrane protein 2 (PRRT2) gene.  Conclusions  There may be interictal nonspecific abnormalities or epileptic discharges in VEEG of children with PKD. The clinical manifestations and synchronous VEEG during the attack period are of great significance to make clear diagnosises.

Key words: Movement disorders, Electroencephalography, Child