背景:后皮质萎缩(PCA)是一种罕见的疾病,其特征是早发性和进行性视力障碍。PCA患者有相对早发性和进行性痴呆,对早期检测提出了一定的需求。因此,本研究旨在探讨外视网膜和脉络膜结构和微血管改变与PCA神经影像学和临床特征的关系,以及载脂蛋白E(APOE)ε4等位基因对PCA参与者外视网膜和脉络膜改变的可能影响。检测潜在的眼部生物标志物进行PCA筛查。
方法:这项横断面研究包括2022年6月至2023年12月的PCA和年龄和性别匹配的健康对照参与者。所有使用PCA的参与者都完成了全面的神经系统评估。记录所有参与者的基线信息并进行眼科评估。使用扫频源光学相干断层扫描(SS-OCT)和血管造影(SS-OCTA)进行定量分析。在某些患者中进行了自适应光学扫描激光检眼镜(AO-SLO)。在PCA的参与者中,研究了APOEε4对体外视网膜和脉络膜改变的影响,以及体外视网膜和脉络膜改变与PCA神经影像学和PCA患者临床特征的相关性.
结果:本次研究共纳入了28名PCA患者(53只眼)和56名健康对照者(112只眼)。与健康对照参与者相比,PCA的参与者视网膜外厚度(ORT)显着降低(p<0.001),脉络膜毛细血管密度(VD)(p=0.007),脉络膜血管指数(CVI)(p=0.005)和脉络膜血管容积(CVV)(p=0.003)。在PCA的参与者中,APOEε4载体显示出较薄的ORT(p=0.009),脉络膜毛细血管病VD(p=0.004)和CVI(p=0.004)增加。PCA神经影像学特征与ORT呈正相关,CVI和CVV。此外,观察到PCA临床特征与CRT的差异相关性,CVV和CVI。
结论:我们的发现强调了外视网膜和脉络膜改变与PCA神经影像学和PCA参与者临床特征的关联。非侵入性SS-OCT和SS-OCTA可以为PCA的诊断和管理提供潜在的生物标志物。提高眼科医生对PCA综合征的认识,神经学家,和初级保健提供者。
BACKGROUND: Posterior cortical atrophy (PCA) is a rare condition characterized by early-onset and progressive visual impairment. Individuals with PCA have relatively early-onset and progressive dementia, posing certain needs for early detection. Hence, this study aimed to investigate the association of alterations in outer retinal and choroidal structure and microvasculature with PCA neuroimaging and clinical features and the possible effects of apolipoprotein E(APOE) ε4 allele on outer retinal and choroidal alterations in participants with PCA, to detect potential ocular biomarkers for PCA screening.
METHODS: This cross-sectional study included PCA and age- and sex-matched healthy control participants from June 2022 to December 2023. All participants with PCA completed a comprehensive neurological evaluation. All participants were recorded baseline information and underwent an ophthalmic evaluation. Quantitative analyses were performed using swept-source optical coherence tomography (SS-OCT) and angiography (SS-OCTA). Adaptive optics scanning laser ophthalmoscopy (AO-SLO) was performed in some patients. In participants with PCA, the influence of APOE ε4 on outer retinal and choroidal alterations and the correlation of outer retinal and choroidal alterations with PCA neuroimaging and clinical features in participants with PCA were investigated.
RESULTS: A total of 28 participants (53 eyes) with PCA and 56 healthy control participants (112 eyes) were included in the current study. Compared with healthy control participants, participants with PCA had significantly reduced outer retinal thickness (ORT) (p < 0.001), choriocapillaris vessel density (VD) (p = 0.007), choroidal vascular index (CVI) (p = 0.005) and choroidal vascular volume (CVV) (p = 0.003). In participants with PCA, APOE ε4 carriers showed thinner ORT (p = 0.009), and increased choriocapillaris VD (p = 0.004) and CVI (p = 0.004). The PCA neuroimaging features were positively associated with the ORT, CVI and CVV. Furthermore, differential correlations were observed of PCA clinical features with the CRT, CVV and CVI.
CONCLUSIONS: Our findings highlighted the association of outer retinal and choroidal alterations with PCA neuroimaging and clinical features in participants with PCA. Noninvasive SS-OCT and SS-OCTA can provide potential biomarkers for the diagnosis and management of PCA, improving awareness of PCA syndrome among ophthalmologists, neurologists, and primary care providers.