Section Clinical Neuroanatomy and Biobanking, Department of Anatomy and Neurosciences, Amsterdam UMC Location Vrije Universiteit Amsterdam, De Boelelaan 1118, Amsterdam, The Netherlands.
Amsterdam Neuroscience, Neurodegeneration, Amsterdam, The Netherlands.
Transl Neurodegener. 2023 Jan 20;12(1):3. doi: 10.1186/s40035-022-00328-8.
Increased neurofilament levels in biofluids are commonly used as a proxy for neurodegeneration in several neurodegenerative disorders. In this study, we aimed to investigate the distribution of neurofilaments in the cerebral cortex of Parkinson's disease (PD), PD with dementia (PDD) and dementia with Lewy bodies (DLB) donors, and its association with pathology load and MRI measures of atrophy and diffusivity.
Using a within-subject post-mortem MRI-pathology approach, we included 9 PD, 12 PDD/DLB and 18 age-matched control donors. Cortical thickness and mean diffusivity (MD) metrics were extracted respectively from 3DT1 and DTI at 3T in-situ MRI. After autopsy, pathological hallmarks (pSer129-αSyn, p-tau and amyloid-β load) together with neurofilament light-chain (NfL) and phosphorylated-neurofilament medium- and heavy-chain (p-NfM/H) immunoreactivity were quantified in seven cortical regions, and studied in detail with confocal-laser scanning microscopy. The correlations between MRI and pathological measures were studied using linear mixed models.
Compared to controls, p-NfM/H immunoreactivity was increased in all cortical regions in PD and PDD/DLB, whereas NfL immunoreactivity was increased in the parahippocampal and entorhinal cortex in PDD/DLB. NfL-positive neurons showed degenerative morphological features and axonal fragmentation. The increased p-NfM/H correlated with p-tau load, and NfL correlated with pSer129-αSyn but more strongly with p-tau load in PDD/DLB. Lastly, neurofilament immunoreactivity correlated with cortical thinning in PD and with increased cortical MD in PDD/DLB.
Taken together, increased neurofilament immunoreactivity suggests underlying axonal injury and neurofilament accumulation in morphologically altered neurons with increased pathological burden. Importantly, we demonstrate that such neurofilament markers at least partly explain MRI measures that are associated with the neurodegenerative process.
生物体液中神经丝水平的升高通常被用作几种神经退行性疾病中神经退行性变的替代标志物。在这项研究中,我们旨在研究帕金森病(PD)、PD 伴痴呆(PDD)和路易体痴呆(DLB)供体大脑皮质中神经丝的分布,及其与病理负荷和 MRI 测量的萎缩和弥散度的关系。
使用亚个体死后 MRI-病理学方法,我们纳入了 9 例 PD、12 例 PDD/DLB 和 18 例年龄匹配的对照供体。在原位 3T MRI 中分别从 3DT1 和 DTI 中提取皮质厚度和平均弥散度(MD)指标。尸检后,在七个皮质区域定量检测神经丝轻链(NfL)和磷酸化神经丝中链和重链(p-NfM/H)免疫反应性以及病理标志物(pSer129-αSyn、p-tau 和淀粉样β负荷),并使用共聚焦激光扫描显微镜进行详细研究。使用线性混合模型研究 MRI 和病理测量之间的相关性。
与对照组相比,PD 和 PDD/DLB 所有皮质区的 p-NfM/H 免疫反应性均增加,而 PDD/DLB 的海马旁回和内嗅皮质区的 NfL 免疫反应性增加。NfL 阳性神经元表现出退行性形态特征和轴突断裂。增加的 p-NfM/H 与 p-tau 负荷相关,而 NfL 与 PDD/DLB 中的 pSer129-αSyn 相关,但与 p-tau 负荷相关性更强。最后,神经丝免疫反应性与 PD 中的皮质变薄和 PDD/DLB 中的皮质 MD 增加相关。
总的来说,神经丝免疫反应性增加表明存在轴突损伤和形态改变神经元中的神经丝积累,伴有增加的病理负荷。重要的是,我们证明了这些神经丝标志物至少部分解释了与神经退行性过程相关的 MRI 测量结果。