Research Service (151), Department of Veterans Affairs, Edward Hines Jr. VA Hospital, Hines, IL 60141, USA; Department of Ophthalmology, Loyola University Health Science Division, Maywood, IL 60153, USA.
Exp Neurol. 2020 Sep;331:113385. doi: 10.1016/j.expneurol.2020.113385. Epub 2020 Jun 17.
Healthy peripheral nerves encounter, with increased frequency, numerous chemical, biological, and biomechanical forces. Over time and with increasing age, these forces collectively contribute to the pathophysiology of a spectrum of traumatic, metabolic, and/or immune-mediated peripheral nerve disorders. The blood-nerve barrier (BNB) serves as a critical first-line defense against chemical and biologic insults while biomechanical forces are continuously buffered by a dense array of longitudinally orientated epineural collagen fibers exhibiting high-tensile strength. As emphasized throughout this Experimental Neurology Special Issue, the BNB is best characterized as a functionally dynamic multicellular vascular unit comprised of not only highly specialized endoneurial endothelial cells, but also associated perineurial cells, pericytes, Schwann cells, basement membrane, and invested axons. The composition of the BNB, while anatomically distinct, is not functionally dissimilar to that of the well characterized neurovascular unit of the central nervous system. While the BNB lacks a glial limitans and an astrocytic endfoot layer, the primary function of both vascular units is to establish, maintain, and protect an optimal endoneurial (PNS) or interstitial (CNS) fluid microenvironment that is vital for proper neuronal function. Altered endoneurial homeostasis as a secondary consequence of BNB dysregulation is considered an early pathological event in the course of a variety of traumatic, immune-mediated, or metabolically acquired peripheral neuropathies. In this review, emerging experimental advancements targeting the endoneurial microvasculature for the therapeutic management of immune-mediated inflammatory peripheral neuropathies, including the AIDP variant of Guillain-Barré syndrome, are discussed.
健康的周围神经经常会遇到多种化学、生物和生物力学的力量。随着时间的推移和年龄的增长，这些力量共同导致了一系列创伤性、代谢性和/或免疫介导的周围神经疾病的病理生理学变化。血神经屏障 (BNB) 是抵御化学和生物侵袭的第一道重要防线，而生物力学力量则被密集排列的纵向神经外膜胶原纤维不断缓冲，这些胶原纤维具有高强度的拉伸强度。正如本实验神经病学特刊所强调的那样，BNB 最好被描述为一个功能动态的多细胞血管单位，不仅包括高度特化的神经内膜内皮细胞，还包括相关的神经外膜细胞、周细胞、施万细胞、基底膜和被包裹的轴突。BNB 的组成虽然在解剖上是不同的，但在功能上与中枢神经系统中特征明确的神经血管单位并无不同。虽然 BNB 缺乏胶质界膜和星形细胞足突层，但这两个血管单位的主要功能都是建立、维持和保护最佳的神经内膜（PNS）或间质（CNS）液微环境，这对于神经元的正常功能至关重要。BNB 调节紊乱导致的神经内膜内环境稳态的改变被认为是多种创伤性、免疫介导或代谢获得性周围神经病变发展过程中的早期病理事件。在这篇综述中，讨论了针对免疫介导的炎症性周围神经病的治疗管理的神经内膜微血管的新兴实验进展，包括格林-巴利综合征的 AIDP 变体。
