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线粒体脑肌病伴高乳酸血症和卒中样发作(MELAS)患者在发作期和缓解期自发性脑活动的改变:超越卒中样病灶。

Altered spontaneous brain activity at attack and remission stages in patients with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS): Beyond stroke-like lesions.

机构信息

Shanghai Institution of Medical Imaging, Shanghai 200032, China; Department of Radiology, HuaShan Hospital, Fudan University, Shanghai 200040, China; Institute of Functional and Molecular Medical Imaging, Fudan University, Shanghai 200040, China.

Department of Radiology, HuaShan Hospital, Fudan University, Shanghai 200040, China; Institute of Functional and Molecular Medical Imaging, Fudan University, Shanghai 200040, China.

出版信息

Mitochondrion. 2020 Sep;54:49-56. doi: 10.1016/j.mito.2020.07.002. Epub 2020 Jul 15.

DOI:10.1016/j.mito.2020.07.002
PMID:32679169
Abstract

Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) may cause whole-brain functional changes due to mitochondrial dysfunction. Our purpose is to comprehensively evaluate the alterations of spontaneous brain activity in MELAS patients at stroke-like episodes (SLE) attack and remission stages using resting-state functional magnetic resonance imaging. Forty MELAS patients at attack stage (n = 20) and remission stage (n = 20) and 22 healthy controls were enrolled in this study. We suggested that MELAS patients presented decreased spontaneous brain activities beyond the areas of stroke-like lesions (SLLs), with a downward trend from SLE attack stage to remission stage. In addition, the regional spontaneous activity of SLL, an inherent change in MELAS, was less than that in unaffected areas. Furthermore, the fractional amplitude of low frequency fluctuations value in left precuneus may be used as a promising neuroimaging biomarker for monitoring the disease status of MELAS.

摘要

线粒体脑肌病、乳酸酸中毒和卒中样发作(MELAS)可能由于线粒体功能障碍导致全脑功能改变。本研究旨在采用静息态功能磁共振成像技术全面评估卒中样发作(SLE)发作期和缓解期 MELAS 患者的自发性脑活动改变。本研究纳入了 40 例 SLE 发作期(n=20)和缓解期(n=20)的 MELAS 患者以及 22 名健康对照者。我们发现 MELAS 患者在卒中样病灶(SLL)区域以外存在自发性脑活动减少,且从 SLE 发作期到缓解期呈下降趋势。此外,MELAS 固有改变的 SLL 区域的自发性活动减少。此外,左侧楔前叶低频波动值的分数幅度可能可作为监测 MELAS 疾病状态的有前途的神经影像学生物标志物。

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Mitochondrial Syndromes Revisited.线粒体综合征再探讨
J Clin Med. 2021 Mar 17;10(6):1249. doi: 10.3390/jcm10061249.