Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany.
J Neurol. 2018 Apr;265(4):949-953. doi: 10.1007/s00415-018-8787-x. Epub 2018 Feb 20.
Changes in skin and muscle small blood vessels (SBVs) and microvascular structures of the brain have been reported in patients with amyotrophic lateral sclerosis (ALS). A direct assessment of brain SBVs in vivo is currently not feasible. Retinal vessels are considered a "mirror" of brain SBVs. In this study, we used optic coherence tomography (OCT)-based measurements to detect changes in retinal blood vessels of ALS patients compared to those of healthy controls.
We analysed Spectralis-OCT images of 34 ALS patients and 20 HCs. The inner wall thickness (IWT), outer wall thickness (OWT), and lumen diameter (LD) of retinal vessels were assessed using intensity-based measurements. In addition, the different retinal layers were analysed using automated segmentation software. The correlations between the various retinal layers and clinical parameters [e.g., disease duration and revised ALS functional rating scale (ALS-FRS-R)] were examined.
The OWT of retinal vessels was higher in ALS patients than in HCs (p = 0.04). There were no differences in the IWT, LD. ALS patients showed a thinning of the outer nuclear layer (ONL) compared to HCs (median 1.63 vs. 1.77, p = 0.002). The whole retinal thickness negatively correlated with the ALS-FRS scale (r = 0.3, p = 0.03).
Our study reports retinal vessel pathology in ALS patients. These changes may be related to those observed in SBVs in skin and muscle biopsies. Furthermore, we report a thinning of the ONL in ALS, revealing a possible affection of rods and cones function in ALS.
据报道,肌萎缩侧索硬化症(ALS)患者的皮肤和肌肉小血管(SBV)以及脑微血管结构发生了变化。目前尚无法直接评估脑 SBV。视网膜血管被认为是脑 SBV 的“镜子”。在这项研究中,我们使用基于光学相干断层扫描(OCT)的测量来检测 ALS 患者与健康对照者的视网膜血管变化。
我们分析了 34 名 ALS 患者和 20 名 HC 的 Spectralis-OCT 图像。使用基于强度的测量来评估视网膜血管的内管壁厚度(IWT)、外管壁厚度(OWT)和管腔直径(LD)。此外,还使用自动分割软件分析了不同的视网膜层。检查了各种视网膜层与临床参数(例如疾病持续时间和修订后的 ALS 功能评定量表(ALS-FRS-R))之间的相关性。
与 HC 相比,ALS 患者的视网膜血管 OWT 更高(p=0.04)。IWT、LD 无差异。与 HC 相比,ALS 患者的外核层(ONL)变薄(中位数 1.63 对 1.77,p=0.002)。整个视网膜厚度与 ALS-FRS 量表呈负相关(r=0.3,p=0.03)。
我们的研究报告了 ALS 患者的视网膜血管病理学。这些变化可能与皮肤和肌肉活检中观察到的 SBV 变化有关。此外,我们报告 ALS 中 ONL 的变薄,揭示了 ALS 中可能影响杆状细胞和锥状细胞功能。