摘要
目的观察线粒体脑肌病伴乳酸血症和卒中样发作(MELAS)综合征和Leigh病患者的影像学特点。方法对12例MELAS综合征、7例Leigh病和1例MELAS与Leigh病叠加患者的影像学特点进行系统分析。结果 12例MELAS综合征患者脑CT及MRI示病灶多位于枕、颞、顶叶皮质及皮质下,且病灶在左侧占优势;7例leigh病患者病变部位主要在双侧基底节、丘脑及脑干。4例MELAS综合征、4例Leigh病患者、1例叠加的核磁波谱检查示病变区乳酸水平明显增高。DWI仅显示新病灶,FLAIR可观察到所有新旧病灶,较T2像敏感。MELAS可见部分病变侧MRA血管增粗增多,且病情复发时病灶有迁徙,旧病变有萎缩的特点。结论 MELAS和Leigh的影像学特点有显著差异,前者以脑叶皮质及皮质下受累为主,病变范围较大,不符合大脑动脉供血区分布。Leigh患者主要病变部位在脑干、基底节,且病灶发展变化趋势有一定的规律性。FLAIR与DWI是不可缺少的扫描像位。MRS对线粒体脑病和线粒体脑肌病的诊断有重要价值,应作为本病常规扫描序列。
Objective To compare and summarize the neuroimaging features in the patients of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes(MELAS)and Leigh disease.Methods Systemic study was performed on the neuroimaging features of patients including 10 MELAS,7 Leigh,and 1 overlapping features of the 2 syndrome.Results Cranial CT showed calcification in basal ganglia and MRI showed cortex and subcortex lesions in 10 patients with MELAS.MRI of 8 patients with Leigh disease indicated that symmetric abnormal signals presented in bilateral basal ganglia and brain stem.MRS of 4 patients with MELAS and 4 with Leigh disease and 1 overlapping suggested that lactic acid level increased in affected areas.Conclusion The imaging features of MELAS and Leigh disease are significantly different.Lesions are easily referred to cerebral cortex and subcortex in patients with MELAS,which are relatively large in size and do not locate with blood supply from capsular arterial branches.However,the lesions in Leigh disease show symmetric abnormal signals in bilateral basal ganglia and brain stem.The trend of lesions may be orderliness.MRS could be very useful in the diagnosis of mitochondrial encephalomyopathy,which should be the regular inspections in its diagnosis.
出处
《脑与神经疾病杂志》
2010年第5期373-377,共5页
Journal of Brain and Nervous Diseases
基金
海后卫生部课题(07HW05)
关键词
线粒体脑肌病伴乳酸血症和卒中样发作
LEIGH病
影像学
Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes
Leigh disease
Clinical manifestation
Image
