The pathology of multiple sclerosis is the result of focal inflammatory demyelination with axonal damage
The pathology of multiple sclerosis is the result of focal inflammatory demyelination with axonal damage
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DOI：
10.1007/s00415-005-5002-7
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发表时间：
2005-11-01
影响因子：
6
通讯作者：
Brück, W
中科院分区：
文献类型：
--
作者：
Brück, W
多发性硬化是一种中枢神经系统慢性炎性脱髓鞘疾病，其形态学表现为炎症、脱髓鞘、轴突缺失和神经胶质增生。炎性病变的特征在于免疫系统的细胞和可溶性介质的异质群体的大量浸润，所述介质包括T细胞、B细胞、巨噬细胞和小胶质细胞，以及广泛的细胞因子、趋化因子、抗体、补体和其它有毒物质。这种病变的出现与临床复发有关。最近对早期急性病变的详细免疫病理学研究揭示了脱髓鞘模式和所涉及的免疫系统因素的深刻异质性。在缓解期间，炎症的消退是导致患者临床改善的主要因素。然而，免疫系统在这个阶段可以发挥有益的作用，可能通过产生生长因子如BDNF来促进髓鞘再生。相比之下，多发性硬化症中的进行性不可逆神经功能缺损与导致轴突和神经元损失的神经退行性过程相关。对多发性硬化症病变中轴突损伤背后的机制知之甚少。然而，具有突出轴突损失的区域和含有炎性浸润的区域（例如，T细胞、巨噬细胞）提示轴突损伤与炎症密切相关。免疫反应的不同可溶性或细胞介质已显示在实验系统中损伤轴突，并且这些可能是人类疾病中神经变性的原因。
Multiple sclerosis is a chronic inflammatory demyelinating disease of the central nervous system manifested morphologically by inflammation, demyelination, axonal loss and gliosis. The inflammatory lesions are characterized by massive infiltration by a heterogeneous population of cellular and soluble mediators of the immune system, including T cells, B cells, macrophages and microglia, as well as a broad range of cytokines, chemokines, antibodies, complement and other toxic substances. The appearance of such lesions is associated with clinical relapses. Recent detailed immunopathological studies of early, acute lesions revealed profound heterogeneity in the patterns of demyelination and the factors of the immune system involved. During remission, resolution of inflammation is the main factor which leads to clinical improvement of patients. However, the immune system can play a beneficial role at this stage, promoting remyelination perhaps by production of growth factors such as BDNF. In contrast, the progressive irreversible neurological deficit in multiple sclerosis is associated with neurodegenerative processes resulting in axonal and neuronal loss. The mechanisms behind damage to axons in multiple sclerosis lesions are poorly understood. However, the close proximity of areas with prominent axonal loss and areas containing inflammatory infiltrates (e.g., T cells, macrophages) suggest that axonal damage is closely associated with inflammation. Different soluble or cellular mediators of the immune response have been shown to damage axons in experimental systems, and these maybe responsible for neurodegeneration in human disease.