摘要
Abstract
Background There are certain challenges for imaging diagnosis of mitochondrial encephalomyopathy(ME).And the study of dynamic imaging data during the progression of the disease is still deficient.Objective To investigate the clinical and dynamic head CT and MRI manifestations in patients with ME,and MELAS(mitochondrial encephalomyopathy,lactic acidosis,and stroke-like episodes) syndrome in particular.Methods The clinical and dynamic cranial imaging manifestations during the 19 episodes of 9 patients with ME diagnosed in the First Affiliated Hospital of Wenzhou Medical University from 2010 to 2015 were reviewed,of which the signal changes of diffusion-weighted imaging(DWI) and apparent diffusion coefficient(ADC) during the progression of disease were observed dynamically.Results Most of the 9 cases were young males(6 cases),their clinical manifestations included seizures,stroke-like episodes,cognitive impairment,headaches or dizziness,decrease in hearing or vision,obvious muscular atrophy and so on.Nine patients were found with 23 lesions involving in the lobes of the brain.Head CT scanning found low-density lesions,and bilateral basal ganglia with high-density shadow.Head MRI scanning indicated that,the intracranial lesions had low signal intensity on T1 and opposite on T2;initially the lesions were swelling,the affected gyri had high signal intensity on DWI and low signal intensity on ADC;the adjacent subcortex was found with nebulously high signal intensity on ADC and that existed steadily for a long time;over time the signal intensity on DWI decreased slowly in gyri while that on ADC increased;in the progression of the disease,it was found that the range of partial lesions narrowed down,and there were irreversible findings including enlarged adjacent ventricle,or widened sulci and faveolate liquefaction in parenchyma when nervous tissue necrosis was involved.Head CT scanning detected that 4 cases had high-density shadow(iron deposition/calcification) in bilateral basal ganglia.Conclusion The clinical manifestations of these ME patients are complicated and various,most of them are cortex-involved.Head CT scanning can provide some information of iron deposition or calcification in nucleus.As for head MRI scanning,DWI and ADC can dynamically reflect the changing process of ME to some extent.关键词
线粒体脑肌病/MELAS综合征/弥散加权成像/表观扩散系数/脑萎缩/铁沉积Key words
Mitochondrial encephalomyopathies/MELAS syndrome/Diffusion-weighted imaging/Apparent diffusion coefficient/Encephalatrophy/Iron deposition分类
医药卫生
