神经退行与脑铁积累障碍和视网膜神经血管结构的神经退行
Elahe Amini1,2, Mohammad Rohani2,3, Alfonso Fasano4
1ENT and Head and Neck Research Center, The Five Senses Health Institute, Iran University of Medical Sciences, Tehran, Iran.
Movement disorders : official journal of the Movement Disorder Society
|November 10, 2023
概括
脑铁积累的神经退行症 (NBIA) 患者表现出显著的视网膜神经血管变化,包括视力敏度降低和视网膜层变化. 这些发现突出了NBIA亚型的潜在生物标志物.
科学领域:
- 眼科医生 眼科 眼科
- 神经学 神经学
- 神经科学是一个神经科学.
背景情况:
- 视网膜为大脑病理提供了一个窗口,但神经退行与大脑铁积累 (NBIA) 研究是有限的.
- 研究NBIA中的视网膜变化可以揭示神经退行性机制的洞察力.
研究的目的:
- 调查NBIA患者的神经和眼科表现.
- 为了确定NBIA的潜在视网膜生物标志物.
主要方法:
- 对基因确诊的NBIA患者和对照者的横截面研究.
- 利用光谱域-光学连贯性断层扫描 (SD-OCT) 和OCT血管学用于视网膜成像.
- 进行了 funduscopy,电网膜学 (ERG),视觉唤起潜力 (VEP) 和神经评估 (PKAN-DRS).
主要成果:
- NBIA患者表现出视力敏度下降,视网膜层较薄,心脏内膜厚度增加 (CCT),毛细血管密度降低.
- 在32.4%和45.9%的患者中,分别观察到异常VEP和ERG.
- 视觉敏度和神经纤维层之间发现的相关性,以及PKAN-DRS得分和疾病持续时间/ERG幅度.
结论:
- 在NBIA患者中,病态结构和功能神经血管变化是明显的.
- 视网膜生物标志物可能有助于理解NBIA机制和区分亚型.
- 需要进一步的研究,以探索NBIA亚型中的视网膜差异生物标志物.
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