线粒体脑肌病伴高乳酸血症和卒中样发作综合征患者的脑磁共振成像改变动态演变规律  

Dynamic evolution of brain magnetic resonance imaging findings in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes syndrome

作　　者：赵丹华[1] 王朝霞[1] 于磊[2] 肖江喜[2] 谢晟[2] 袁云[1] 黄一宁[1] 

机构地区：[1] 北京大学第一医院神经内科,100034 [2]北京大学第一医院医学影像科,100034

出　　处：《中华神经科杂志》2014年第4期229-231,共3页Chinese Journal of Neurology

基　　金：科技部＂十二五＂重大专项课题资助项目（2011ZX09307-001-07）;国家自然科学基金资助项目（30870864）

摘　　要：目的 分析线粒体脑肌病伴高乳酸血症和卒中样发作（MELAS）综合征患者的脑MRI改变动态演变规律.方法 收集经肌肉病理检查和（或）基因检测确诊的58例MELAS患者的资料,于卒中样发作（SLE）后60 d内进行重复脑MRI检查,分析其病灶的演变规律；计算31例患者缓解期头MRI的脑萎缩指数以及与其年龄及病程之间的相关性.结果 SLE后30 d内64.1％ （25/39）的病灶扩大,10.2％ （4/39）的病灶转移,17.9％（7/39）的病灶缩小.在30 ～60 d内13％（3/23）的病灶扩大,21.7％（5/23）的病灶转移和56.5％（13/23）的病灶缩小.31例患者SLE缓解期脑萎缩指数为15.2％±2.8％,与年龄、总病程、脑内症状出现后病程的相关系数分别为r =0.329（P=0.043）、0.405（P=0.012）和0.649（P =0.000）.结论 MELAS综合征患者随病程发展出现脑萎缩和脑内病灶缩小,但病灶的转移持续存在.Objective To analyze the dynamic evolution of brain MRI in patients with mitochondrial myopathy,encephalopathy,lactic acidosis,and stroke-like episodes （MELAS） syndrome.Methods A retrospective study was performed on 58 MELAS cases with pathologically and （or） molecularly confirmed diagnosis.MRI were repeated within 60 days after the onset of stroke-like episodes （SLE） and the evolution changes of cerebral lesions were accessed.Brain atrophy index （BAI） was calculated in the remission stage from 31 patients with MELAS,and the correlation between BAI,age and disease duration was analyzed.Results The proportion of lesions expansion,migration and shrink within 30 days after the onset of SLE was 64.1% （25/39）,10.2% （4/39）,17.9% （7/39）,respectively,and 13% （3/23）,21.7% （5/23）,56.5% （13/23）,between 30-60 days after the onset of SLE respectively.In the recovery stage of SLE,the BAI in 31 patients with MELAS was 15.2% ±2.8%.The correlation coefficient between BAI and the age,total disease course and duration of encephalopathy was 0.329 （P =0.043）,0.405 （P =0.012） and 0.649 （P =0.000）.Conclusions Brain atrophy in the studied MELAS patients gradually develops and strokelike lesions shrink with progression of the disease.However,the migration of lesions is persistent.

关 键 词：MELAS综合征 磁共振成像 萎缩 脑 

分 类 号：R746[医药卫生—神经病学与精神病学] R743.3[医药卫生—临床医学]