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在患有Leber遗传性视神经病变的患者中,脑血流异常

Ling Wang¹, Yi Ji², Hao Ding^2,3

¹Department of Medical Imaging, Henan Provincial People's Hospital, Zhengzhou, 450003, China.

Brain imaging and behavior

|June 27, 2023

概括

勒伯遗传性视神经病 (LHON) 患者在视觉和认知领域的脑血流 (CBF) 减少. 这种减少与疾病持续时间和神经眼科障碍相关.

科学领域:

神经科学是一个神经科学.
医疗成像医学成像
眼科医生眼科眼科

背景情况:

勒伯遗传性视神经病 (LHON) 是一种主要影响视神经的线粒体疾病.
在LHON中,大脑血流 (CBF) 的异常被怀疑是LHON,但没有很好的特征.
动脉旋转标签 (ASL) 提供了一种非侵入性方法来评估CBF.

研究的目的:

用动脉旋转标记 (ASL) 来量化Leber遗传性视神经病变 (LHON) 患者的脑血流 (CBF) 异常.
探索LHON中改变的CBF,疾病持续时间和神经眼科缺陷之间的关系.
为了区分急性和慢性LHON阶段的CBF模式.

主要方法:

对20名急性LHON,29名慢性LHON患者和37名健康对照进行了ASL输液成像.
协同变量分析 (ANCOVA) 用于比较各组之间的CBF.
线性和非线性回归模型分析了CBF,疾病持续时间和视力障碍指标之间的关联.

主要成果:

与对照人群相比,LHON患者在视觉和感觉运动大脑区域的CBF减少.
慢性LHON患者在左中额头环和感觉运动皮质的CBF明显低于急性LHON患者和对照组.
在左中额头环中的CBF与疾病持续时间负相关,与视网膜神经纤维层厚度正相关.

结论:

勒伯遗传性视神经病变与视觉,感觉运动和高阶认知领域的CBF减少有关.
疾病持续时间和神经眼科损伤的严重程度会影响非视觉大脑区域的CBF.
ASL是了解视神经之外的LHON病理生理学的宝贵工具.

关键词:

动脉旋转标签的标签大脑新陈代谢大脑新陈代谢大脑的血液流动.勒贝尔的遗传性视神经病变线粒体疾病是线粒体疾病.

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