受损的白质与患有基尿症的成年人的认知能力下降有关
Raphaela Muri1,2,3,4, Stephanie Maissen-Abgottspon1,4, Murray Bruce Reed5
1Department of Diabetes, Endocrinology, Nutritional Medicine and Metabolism, Inselspital, Bern University Hospital and University of Bern, 3010 Bern, Switzerland.
Brain communications
|June 2, 2023
概括
患有基尿症 (PKU) 的成年人的白质变化持续到成年期,显示出突出的后部白质变化,可能是由于轴突损伤. 这些微观结构差异与认知功能相关,而不是代谢控制.
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) 是一种影响大脑发育的代谢障碍.
- 虽然PKU对大脑结构的影响已知,但其对白质微观结构的影响以及与代谢控制和认知的关系仍然不清楚.
研究的目的:
- 量化评估PKU成年人的白质微观结构.
- 调查PKU患者白质特征,氨酸水平,代谢控制和认知表现之间的关系.
主要方法:
- 扩散张力成像 (DTI) 和1H光谱学用于30名早期治疗PKU的成年人和54名健康对照.
- 白质微观结构使用分数异构 (FA) 和扩散度指标 (MD,AD,RD) 进行分析.
- 认知功能 (执行功能,注意力,处理速度) 和氨酸水平 (大脑和血) 被评估.
主要成果:
- 与对照组相比,在PKU患者中观察到MD,AD和FA的广泛减少,特别是在光学辐射和后冠状体辐射中.
- 白质微观结构与认知表现有显著的关联,特定的扩散性和FA指标与抑制,认知灵活性和注意力分裂相关.
- 白质微观结构与并发或历史代谢控制之间没有发现显著的关联. 白质病变很普遍,但与认知或代谢控制无关.
结论:
- 早期治疗PKU的白质变化持续到成年期,其特征是突出的后部白质变化,暗示轴突损伤.
- 患有PKU的成年人的DTI指标与注意力和执行功能的表现有关,突出显示了PKU超出代谢控制的神经生物学影响.
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