摘要
目的:分析进行性肌阵挛癫(PME)的临床特点。方法:回顾性分析7例PME患者病案。结果:①PME于童年或青少年起病。②随病情进展所有患者均有不同程度的智能减退。③癫发作形式:全身性、局灶性或节段性的肌阵挛,无规律、不同步、不对称的;通常合并全身强直阵挛发作或部分性发作。④有小脑、锥体束等神经系统受累症状。结论:PME主要临床表现除癫、肌阵挛和进行性神经功能衰退外,视觉发作也是比较明显的症状,脑电图检查是必不可少的。对某些患者及亲属进行乳酸运动试验有助于本病中线粒体病(MERRF)型的诊断。
Aim： To analyse clinical characteristics of patients with progressive myoclonus epilepsies （PME）. Methods： The medical records of 7 cases with PME were retrospectively reviewed. Results： ①The onset is in chihlhood or adolescence. ②All patients have mental deterioration culminating in dementia. ③A myoclonus syndrome involves a combination of focal or segmental , arrhythmic, asynchronous, asymmetric myoclonus and massive myoclonias. Epilepsy usually is generalized with tonic-clonic or partial seizures.④ Neurological syndrome nearly always includes cerebellar and pyramidal tract manifestations. Conclusion： In clinical features of progressive myoclniic epilepsies besides epilepsy and myoclonus and progressive neurologic dererioration, sight paroxysm is one of obvious features. In making the diagnosis of progressive myoclonus epilepsises, EEG is necessary and serum lactic acid levels are usuful at before and after motion for some patients and their relatives.
出处
《中国临床神经科学》
2006年第1期61-64,共4页
Chinese Journal of Clinical Neurosciences
