University Vita-Salute San Raffaele, Milan, Italy.
Department of Neurology and Institute of Experimental Neurology (INSPE), San Raffaele Hospital Scientific Institute, Milan, Italy.
Sci Rep. 2019 Jul 17;9(1):10371. doi: 10.1038/s41598-019-46251-3.
Early detection of neuromyelitis optica spectrum disorders (NMOSD), especially after optic neuritis, a presenting manifestation commonly observed also in multiple sclerosis (MS), is crucial for timely treatment and prognosis. Integrated visual pathway assessment with optical coherence tomography (OCT) and visual evoked potentials (VEP) may help in this task, showing in vivo different pathophysiological backgrounds. We evaluated combined VEP and OCT in a cross-sectional, single-centre study assessing 50 consecutive NMOSD patients, 57 MS patients and 52 healthy controls. After optic neuritis, VEP were more frequently absent in NMOSD compared to MS; most NMOSD eyes with recordable VEP showed prolonged latency, but extreme latency delays were less common than in MS. OCT showed predominantly axonal involvement in NMOSD, with 88% eyes (95% CI: 69-97%) displaying retinal nerve fibre layer thickness <60 µm even after first optic neuritis episode. Accuracy of OCT was further enhanced by combination with VEP into a new Z-score derived OCT-VEP index, measuring prevalence of axonal damage or demyelination. Our results suggest that integrated optic nerve assessment may elucidate differences in optic neuritis pathophysiology; conduction slowing with relatively preserved nerve fibre layer suggests MS, while severe neuroaxonal loss after optic neuritis, often hindering VEP response, characterizes NMOSD.
视神经脊髓炎谱系疾病(NMOSD)的早期检测至关重要,特别是在视神经炎后,这是多发性硬化症(MS)常见的首发表现。视神经炎后,NMOSD 患者的 VEP 较 MS 患者更常缺失;大多数 NMOSD 患者的 VEP 记录潜伏期延长,但与 MS 相比,极端潜伏期延迟的情况较少见。OCT 显示 NMOSD 主要累及轴突,即使在首次视神经炎发作后,88%的眼(95%CI:69-97%)的视网膜神经纤维层厚度<60µm。OCT 与 VEP 相结合,形成一种新的基于 Z 分数的 OCT-VEP 指数,可以更准确地评估视神经损伤,该指数可测量轴突损伤或脱髓鞘的发生率。我们的研究结果表明,综合评估视神经可能有助于阐明视神经炎的病理生理学差异;MS 表现为神经传导速度减慢,而 NMOSD 则表现为视神经炎后严重的神经轴突丢失,常导致 VEP 反应缺失。