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持续性部分性癫痫(科热夫尼科夫综合征):一例报告。

Epilepsia partialis continua (Kojewnikow's syndrome): report of one case.

作者信息

Lin L H, Wang P J, Lin M Y, Sue W C, Shen Y Z

机构信息

Department of Pediatrics, National Taiwan University Hospital, Taipei, R.O.C.

出版信息

Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 1990 Jul-Aug;31(4):254-61.

PMID:2124775
Abstract

We describe a 12-year-old boy with EPC (Epilepsia Partialis Continua) who showed well localized myoclonic jerks in the left toes continuing for more than 19 months. Preceding partial clonic or tonic convulsions of the left leg or body were initially noted. Neurological examination disclosed mild muscle weakness of the left leg. During the course, neither mental deterioration nor progressive evolution was noted. Since the EEG revealed well defined midline central spike focus which represent the sensorimotor area of left leg and foot, we postulate that the pathophysiological mechanism of the EPC may be evoked by the focal epileptogenic activation of the motor cortex. EEG-EMG polygraphy proved the well localized myoclonic action potential volleys in the M. extensor hallucis longus of the left great toe. According to the clinical and electrophysiological findings, we classified our case into the group I of Bancaud's definition but failed to find out any demonstrable brain lesion throughout brain CT, MRI and angiography. Neurosurgical intervention is not practical for our case, and medical treatment gave somewhat improvement.

摘要

我们描述了一名患有持续性部分性癫痫(EPC)的12岁男孩,其左脚趾出现定位明确的肌阵挛性抽搐,持续超过19个月。最初发现左腿或身体先出现部分阵挛性或强直性惊厥。神经系统检查发现左腿轻度肌肉无力。在病程中,未发现精神衰退或病情进展。由于脑电图显示明确的中线中央棘波灶,代表左腿和足部的感觉运动区,我们推测EPC的病理生理机制可能是由运动皮层的局灶性致痫激活引起的。脑电图-肌电图多导记录证实左大脚趾拇长伸肌存在定位明确的肌阵挛动作电位群。根据临床和电生理检查结果,我们将该病例归类为班考定义的I组,但在整个脑部CT、MRI和血管造影检查中均未发现任何可证实的脑部病变。神经外科干预对我们的病例并不适用,药物治疗有一定改善。

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Epileptic negative myoclonus.癫痫性负性肌阵挛
Neurology. 1993 Jun;43(6):1078-83. doi: 10.1212/wnl.43.6.1078.

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