Lucchinetti C F, Brück W, Rodriguez M, Lassmann H
Department of Neurology; Mayo Clinic Foundation; Rochester, Minnesota, USA.
Brain Pathol. 1996 Jul;6(3):259-74. doi: 10.1111/j.1750-3639.1996.tb00854.x.
Multiple sclerosis is an inflammatory demyelinating disease of the central nervous system. The hallmark of its pathology is the demyelinated plaque with reactive glial scar formation. However, a detailed analysis of the patterns of demyelination, oligodendroglia cell pathology and the reaction of other tissue components suggests that the pathogenesis of myelin destruction in this disease may be heterogeneous. In this review we present a new classification scheme of lesional activity on the basis of the molecular composition of myelin degradation products in macrophages. When these criteria are used, different patterns of demyelination can be distinguished, including demyelination with relative preservation of oligodendrocytes, myelin destruction with concomitant and complete destruction of oligodendrocytes or primary destruction or disturbance of myelinating cells with secondary demyelination. Furthermore, in some cases a primary selective demyelination may be followed by a secondary oligodendrocyte loss in the established lesions. Finally, some extraordinarily severe conditions may result in destructive lesions with loss of myelin, oligodendrocytes, axons and astrocytes. This heterogeneity of plaque pathology is discussed in the context of recent experimental models of inflammatory demyelination, which show that different immunological pathways may lead to the formation of demyelinated plaques that reveal the diverse structural aspects described above. Our data indicate, that the demyelinated plaques of multiple sclerosis may reflect a common pathological end point of a variety of different immunological mechanisms of myelin destruction in this disease.
多发性硬化症是一种中枢神经系统的炎性脱髓鞘疾病。其病理学特征是伴有反应性胶质瘢痕形成的脱髓鞘斑块。然而,对脱髓鞘模式、少突胶质细胞病理学以及其他组织成分反应的详细分析表明,该疾病中髓鞘破坏的发病机制可能是异质性的。在本综述中,我们基于巨噬细胞中髓鞘降解产物的分子组成,提出了一种新的病变活动分类方案。当使用这些标准时,可以区分不同的脱髓鞘模式,包括少突胶质细胞相对保留的脱髓鞘、少突胶质细胞同时完全破坏的髓鞘破坏或髓鞘形成细胞的原发性破坏或紊乱伴继发性脱髓鞘。此外,在某些情况下,原发性选择性脱髓鞘可能会在已形成的病变中继发少突胶质细胞丢失。最后,一些极其严重的情况可能导致伴有髓鞘、少突胶质细胞、轴突和星形胶质细胞丢失的破坏性病变。在最近的炎性脱髓鞘实验模型的背景下讨论了斑块病理学的这种异质性,这些模型表明不同的免疫途径可能导致脱髓鞘斑块的形成,这些斑块揭示了上述不同的结构方面。我们的数据表明,多发性硬化症的脱髓鞘斑块可能反映了该疾病中多种不同髓鞘破坏免疫机制的共同病理终点。