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大脑血流和白质变化在成年人与基尿症

Leonie Steiner¹, Raphaela Muri², Dilmini Wijesinghe³

¹Department of Diabetes, Endocrinology, Nutritional Medicine and Metabolism, Inselspital, Bern University Hospital and University of Bern, Switzerland; Division of Neuropaediatrics, Development and Rehabilitation, Department of Paediatrics, Inselspital, Bern University Hospital and University of Bern, Switzerland.

NeuroImage. Clinical

|December 13, 2023

概括

基尿症 (PKU) 患者在特定的大脑区域的脑血流 (CBF) 减少,这与白质病变有关. 这项研究强调了血管健康和PKU中大脑变化之间的复杂关系.

科学领域:

神经科学是一个神经科学.
放射学放射学是一门学科.
代谢障碍代谢障碍代谢障碍

背景情况:

基尿症 (PKU) 是一种影响氨转化的代谢障碍.
PKU与认知缺陷和大脑结构变化有关.
在PKU中,大脑血流 (CBF) 了解得很少.

研究的目的:

在早期治疗PKU的成年人中调查CBF.
检查CBF,认知,白质病变和代谢参数之间的关联.

主要方法:

动脉旋转标记输液MRI测量CBF. 动脉旋转标记输液MRI测量CBF.
H光谱检测大脑中的氨 (Phe) 水平.
扩散张力成像 (DTI) 检测白质完整性.
认知评估 (智商,执行功能,注意力,处理速度).

主要成果:

全球CBF在PKU患者和对照人群之间没有差异.
在左中和后脑动脉区域观察到减少的CBF,特别是前脑动脉.
白质病变与受影响区域的CBF减少相关 (例如,尾叶病变和左PCACBF).
在ACA和PCA中与CBF积极相关的轴向扩散率 (DTI).
CBF与认知表现或代谢参数没有相关性.

结论:

在PKU中,CBF和白质变化之间存在复杂的相互作用.
血管健康对于了解PKU对大脑的影响至关重要.
多因素模型对于PKU大脑研究很重要.

关键词:

动脉旋转标签的标签大脑的血液流动.认知认知是一种认知.基尿症基尿症频谱学是一种光谱学.白质是白质的组成部分.

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