Lampert P W
Am J Pathol. 1978 Apr;91(1):176-208.
Patterns of demyelination are described in several autoimmune and virus-induced demyelinating diseases of the peripheral and central nervous system. Myelin can be destroyed by injuries that affect either the myelin-supporting cells and/or the myelin lamellae. After destruction of the supporting cells, the related disintegrating sheaths are stripped off axons by invading phagocytes. Virus-induced cytolysis can occur with or without participation of immune responses, as demonstrated in subacute sclerosing panencephalitis and progressive mutlifocal leukoencephalopathy, respectively. Autoimmune demyelination is characterized by disintegration of myelin sheaths in periventular, mononuclear cell infiltrates. Myelin lamellae rather than the myelin-supporting cells are the target of the allergic reaction. The lamellae are lysed in focal areas when in contact with presumably sensitized mononuclear cells. The damaged sheaths are then removed in a nonspecific manner by invading macrophages that strip the myelin remnant off the axons. This sequence of changes is best revealed in experimental and human autoimmune demyelination of peripheral nerves, ie, allergic neuritis and idiopathic polyneutris (the Guillain-Barré syndrome). Autoimmune demyelination triggered by virus infection is described in Marek's disease and postinfectious Theiler's virus myelitis. Changes in canine distemper are discussed with reference to both autoimmune and virus-induced demyelination. The observations are compared with lesions in multiple sclerosis, the most common human demyelinating disease of unknown etiology.
在几种外周和中枢神经系统的自身免疫性及病毒诱导的脱髓鞘疾病中,脱髓鞘模式已有描述。髓磷脂可因影响髓磷脂支持细胞和/或髓磷脂板层的损伤而被破坏。支持细胞被破坏后,相关的崩解鞘会被侵入的吞噬细胞从轴突上剥离。病毒诱导的细胞溶解可在有无免疫反应参与的情况下发生,分别如亚急性硬化性全脑炎和进行性多灶性白质脑病所示。自身免疫性脱髓鞘的特征是脑室周围有髓鞘崩解,伴有单核细胞浸润。过敏反应的靶标是髓磷脂板层而非髓磷脂支持细胞。当与可能致敏的单核细胞接触时,板层在局部区域会被溶解。然后受损的鞘会被侵入的巨噬细胞以非特异性方式清除,巨噬细胞会将髓磷脂残余物从轴突上剥离。这种变化序列在实验性和人类外周神经自身免疫性脱髓鞘中最为明显,即过敏性神经炎和特发性多发性神经炎(格林-巴利综合征)。在马立克氏病和感染后泰勒氏病毒脊髓炎中描述了由病毒感染引发的自身免疫性脱髓鞘。参照自身免疫性和病毒诱导的脱髓鞘对犬瘟热的变化进行了讨论。将这些观察结果与多发性硬化症(最常见的病因不明的人类脱髓鞘疾病)中的病变进行了比较。