以癫(癎)持续状态起病的线粒体脑肌病合并乳酸血症与脑卒中样发作的临床特征分析.pdfVIP

Chi n J Clin Ne urosci 2012, 20 ( L) , 37-42 . 37 . r__..φ_.呻￥ • • j 论著j t舶…0.• 以癫病持续状态起病的线粒体脑肌病合并乳酸血症与 脑卒中样发作的临床特征分析 吴i句映 ，朱国行， 王晋扬 ， 王明，徐岚， 王剑虹， 洪震 (复叹大学附属华山医院神经科200040) 关键词 癫摘持续状态;线粒体脑肌病: 脑电图 摘要 目的: 探i寸以癫踊持续状态起病的线粒体脑肌病合并乳酸IÚU缸与脑卒中样发作(MELAS)患者的11备床、脑电图、 影像学及肌肉病理等特点。 方法: 对收治并最终确诊为MELAS 的6 例(年龄 13-40 岁)病例进行总结、分析其临床与脑 电图相关特征。结果: 6 例患者均以癫刑将续状态、起病，头颅MRI 提示脑实质内异常病灶，发{乍阅期脑电图检查提示 相应区域有;Ji可样放电，肌肉活检见破碎主[纤维(RRF)。 结论: 对青少年及成人起病的难治性或进展性癫脯， 1临床诊断 应考虑线位体疾病的可能。 MELAS 患者由干存在线校体功能损害与ATP 能量供应障碍， 其神经细胞更易遭受兴俗除 毒性侵害，应尽早选择合适的抗癫脯药物干预以阻止癫脯持续状态的发生。 A GlOUp ofMitochondlial Encephalopathy 飞~thLactic Acidosis and Stroke with Onset of Status Epileptics: Study of Clinical Featur宅s vf气I及~n- Yi, ZHU Guo-Xìng, WANG Jin-yiαng， WANG Yue, XU Lan, WANG Jiαn­ Hong, HONG Zhen Department of Neurology, Huashan Hospital, Fudan University, Shanghai 200040, China KEY WORDS status epileptics; mitochondrial encephalopathy; electroencephalogram ABSTRACT Aim: To investigate the clinical features of a gr oup of mitochondrial encephalomyopathy with lactic acidosis and stroke 仙tlELAS) patients with onset of status epileptics. Methods: AII patients with diagnosis of MELAS by muscle biopsy were followed up. Their seizures, EEGs, image findings , and therap巳utic data were reviewed to analyze their semi ological characters. Results: Patients in this study were onset with staωs epileptics. 岛仅1 showed hyperinte nse gyriform lesion on T2 and bilateral