线粒体脑肌病伴高乳酸血症和卒中样发作的临床和磁共振灌注加权成像特点  

Analysis on clinical features and functional MRI of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes

作　　者：时雅辉 赵建华[2] 宋金玲[2] 李月娟[2] 梁珂[2] 

机构地区：[1]新乡医学院研究生处,453003 [2]河南省人民医院神经内科

出　　处：《中华医学杂志》2016年第37期2969-2972,共4页National Medical Journal of China

摘　　要：目的：探讨线粒体脑肌病伴高乳酸血症和卒中样发作（MELAS）的临床和磁共振灌注加权成像（PWI）特点，提高早期诊断率。方法收集2012年1月至2016年6月在河南省人民医院住院治疗的46例最终确诊为 MELAS 的临床及影像学资料。结果首发症状为癫痫、偏瘫、语言障碍、视力下降等。病灶区弥散加权成像（DWI）呈高信号，表现扩散系数（ADC）图随病程发展可呈高信号、低信号及高低信号并存。磁共振血管造影（MRA）均无明显血管狭窄。 PWI 急性期灌注增高，慢性期灌注正常或减低。结论MELAS 临床表现复杂多样，缺乏特异性，常规磁共振成像（MRI）检查对于病变的筛选有很大的意义，PWI 具有特征性表现，有助于提高该病早期诊断率。Objective To explore the clinical and perfusion weighted imaging （ PWI ） characteristics of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes （MELAS） in order to improve the early diagnosis rate.Methods Clinical and imaging data of 46 patients with final diagnosis of MELAS treated in Henan Province People′s Hospital from January 2012 to June 2016 were collected.Results Patients presented with symptoms of epilepsy, hemiplegia, language disorders, and decreased visual acuity and so on.Diffusion-weighted imaging （DWI） of the lesion area showed a high signal, apparent diffusion coefficient （ADC） chart with the course of disease development can be a high signal, low signal, high and low signal.Vascular stenosis was not seen in all the patients by magnetic resonance angiography （MRA）.PWI perfusion increased in acute phase and was normal or decreased in the chronic phase.Conclusions The clinical manifestations of MELAS patients are complex and various, and lack of specificity.Routine MRI examination is of great significance for the screening of the lesion, which has a characteristic performance and is helpful to improve the early diagnosis rate of the disease.

关 键 词：MELAS 综合征 磁共振成像 磁共振血管造影 磁共振扩散加权成像 磁共振灌注加权成像 

分 类 号：R445.2[医药卫生—影像医学与核医学]