线粒体脑肌病伴高乳酸血症和卒中样发作综合征的影像学特征及动态演变

发布时间：2018-07-31 16:07

【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)综合征的影像学特点及其动态演变过程。方法收集2011年1月-2016年2月我院经肌肉病理确诊的21例MELAS综合征的资料,对他们的头部CT、MRI、增强MRI、MRA和MRS表现进行回顾性分析。结果 19例患者行头部CT,其中8例显示双侧基底节区对称性钙化。卒中样发作急性期头部磁共振主要表现为T_1WI低信号、T_2WI和FLAIR高信号,DWI高信号或等信号,ADC高信号或低信号;增强MRI未见明显强化或线状强化,MRA未见明显异常,MRS可见N-乙酰天门冬氨酸峰(NAA)下降、乳酸峰(Lac)明显升高。19例(90.5%)病灶累及2个及2个以上脑叶,最常累及的部位是枕叶、颞叶和顶叶。病灶呈层状坏死,分布不符合脑血管的支配区域,动态观察具有"可逆性"、"游走性"和"进展性"。结论 MELAS综合征临床表现复杂,神经影像学具有一定的特征性,具有重要诊断价值。充分认识这些特征,有助于早期诊治、减少误诊。
[Abstract]:Objective to investigate the imaging features and dynamic evolution of mitochondrial encephalomyopathy with hyperlactoemia and apoplexy (MELAS) syndrome. Methods from January 2011 to February 2016, 21 cases of MELAS syndrome confirmed by muscle pathology in our hospital were collected, and the CT findings of their heads were analyzed retrospectively. Results CT was performed in 19 patients, of which 8 showed bilateral symmetrical calcification of basal ganglia. In the acute phase of apoplexy, the main Mr manifestations of head were T_1WI low signal T _ 2WI and FLAIR hyperintense or iso-signal. There was no obvious enhancement or linear enhancement on enhanced MRI and no obvious abnormality in Mrs. The peak (NAA) of N acetyl aspartate was decreased, and the peak (Lac) of lactic acid was significantly increased in 19 cases (90.5%). The lesions involved 2 or more than 2 cerebral lobes, the most common involvement was occipital lobe. Temporal lobe and parietal lobe. The lesion presented stratified necrosis, and the distribution was not in accordance with the innermost region of cerebral vessels. The dynamic observation showed "reversibility", "mobility" and "progression". Conclusion the clinical manifestations of MELAS syndrome are complicated, neuroimaging is characteristic and has important diagnostic value. To fully understand these characteristics is helpful to early diagnosis and treatment and reduce misdiagnosis.
【作者单位】：郑州大学第五附属医院神经内科、肌肉病理实验室;郑州大学第五附属医院护理部;
【基金】：河南省科技厅科技攻关项目(No.132102310092)
【分类号】：R746

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