Pathology of multiple sclerosis: where do we stand?
Pathology of multiple sclerosis: where do we stand?
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DOI：
10.1212/01.con.0000433291.23091.65
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发表时间：
2013-08-01
期刊：
影响因子：
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通讯作者：
Lucchinetti, Claudia F
中科院分区：
文献类型：
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作者：
Popescu, Bogdan F Gh;Pirko, Istvan;Lucchinetti, Claudia F
综述目的：本文总结了多发性硬化症（MS）和其他炎症性脱髓鞘疾病的病理特征，并讨论了神经病理学研究对脱髓鞘潜在机制的新见解。最近发现：多发性硬化症的病理特征包括中枢神经系统内的局灶性脱髓鞘斑块，伴有不同程度的炎症、胶质瘤和神经退行性变。活动性MS病变表现出深刻的病理异质性，具有四种主要的免疫病理模式，这表明MS损伤的目标和脱髓鞘的机制可能在不同的疾病亚群中不同。最近的病理学研究表明，蛛网膜下腔和皮层可能是MS疾病过程的起始部位和靶点，炎性皮质脱髓鞘在MS早期就存在，脑膜炎症可导致部分MS患者皮质和白质损伤。总结：多发性硬化症在临床、遗传、放射学和病理特征方面是异质性的；需要开发四种免疫模式的替代MRI，临床，遗传，血清学和/或CSF标记物，以便在一般未经活检的MS人群中识别它们。炎症性皮质脱髓鞘是多发性硬化症发病机制的重要早期事件，可能由脑膜炎症驱动。这些观察结果强调了开发能够捕获早期炎性皮质脱髓鞘的成像技术的重要性，以便更好地了解疾病发病机制并确定潜在的疾病修饰疗法对皮质的影响。
PURPOSE OF REVIEW: This article summarizes the pathologic features of multiple sclerosis (MS) and other inflammatory demyelinating diseases and discusses neuropathologic studies that have yielded novel insights into potential mechanisms of demyelination.RECENT FINDINGS: The pathologic hallmark of MS consists of focal demyelinated plaques within the CNS, with variable degrees of inflammation, gliosis, and neurodegeneration. Active MS lesions show a profound pathologic heterogeneity with four major patterns of immunopathology, suggesting that the targets of injury and mechanisms of demyelination in MS may be different in different disease subgroups. Recent pathologic studies have suggested that the subarachnoid space and cortex may be initial sites and targets of the MS disease process, that inflammatory cortical demyelination is present early in MS, and that meningeal inflammation may drive cortical and white matter injury in some MS patients.SUMMARY: MS is heterogeneous with respect to clinical, genetic, radiographic, and pathologic features; surrogate MRI, clinical, genetic, serologic, and/or CSF markers for each of the four immunopatterns need to be developed in order to recognize them in the general nonbiopsied MS population. Inflammatory cortical demyelination is an important early event in the pathogenesis of MS and may be driven by meningeal inflammation. These observations stress the importance of developing imaging techniques able to capture early inflammatory cortical demyelination in order to better understand the disease pathogenesis and to determine the impact of potential disease-modifying therapies on the cortex.