肌萎缩侧索硬化(ALS)是一种罕见的致命性运动神经元疾病。尽管已经提出了许多潜在的机制,该疾病的病理生理学仍然未知。目前可用的治疗方法只能延缓疾病的进展,并将预期寿命延长几个月。ALS还没有确定的治疗方法,由于缺乏对引发和促进神经变性的潜在生物过程的了解,新疗法的发展受到限制。一些科学结果表明,ALS中的神经血管损害为开发新的生物标志物和治疗方法提供了前景。在这篇文章中,我们使用PRISMA指南进行了系统审查,包括PubMed,Embase,GoogleScholar,和WebofScienceCoreCollection分析了2000年至2021年之间发表的科学文献,讨论了ALS的神经心血管受累和眼科异常。总的来说,纳入了122篇文章来建立这一系统综述。的确,微血管病理学似乎与ALS有关,影响所有的神经血管单元组件。最近还突出了视网膜变化,而视觉通路没有明显改变。尽管视网膜的周边位置,它被认为是中枢神经系统(CNS)的延伸,因为它显示出与大脑的相似性,内部的血-视网膜屏障,还有血脑屏障.这表明,在许多中枢神经系统疾病中,眼睛可以被视为进入大脑的窗口。因此,研究脑部病变的眼部表现在理解神经退行性疾病方面似乎非常有希望,主要是ALS。因此,光学相干断层扫描血管造影(OCT-A)可能是探索视网膜微血管形成的有力方法,可以获得新的ALS诊断和预后生物标志物。
Amyotrophic lateral sclerosis (ALS) is a rare fatal motor neuron disease. Although many potential mechanisms have been proposed, the pathophysiology of the disease remains unknown. Currently available treatments can only delay the progression of the disease and prolong life expectancy by a few months. There is still no definitive cure for ALS, and the development of new treatments is limited by a lack of understanding of the underlying biological processes that trigger and promote neurodegeneration. Several scientific results suggest a neurovascular impairment in ALS providing perspectives for the development of new biomarkers and treatments. In this article, we performed a systematic
review using PRISMA guidelines including PubMed, EmBase, GoogleScholar, and Web of Science Core Collection to analyze the scientific literature published between 2000 and 2021 discussing the neurocardiovascular involvement and ophthalmologic abnormalities in ALS. In total, 122 articles were included to establish this systematic
review. Indeed, microvascular pathology seems to be involved in ALS, affecting all the neurovascular unit components. Retinal changes have also been recently highlighted without significant alteration of the visual pathways. Despite the peripheral location of the retina, it is considered as an extension of the central nervous system (CNS) as it displays similarities to the brain, the inner blood-retinal barrier, and the blood-brain barrier. This suggests that the eye could be considered as a \'window\' into the brain in many CNS disorders. Thus, studying ocular manifestations of brain pathologies seems very promising in understanding neurodegenerative disorders, mainly ALS. Optical coherence tomography angiography (OCT-A) could therefore be a powerful approach for exploration of retinal microvascularization allowing to obtain new diagnostic and prognostic biomarkers of ALS.