The Pathology of Multiple Sclerosis and Its Variants
The Pathology of Multiple Sclerosis and Its Variants
复制标题
多发性硬化症及其变异的病理学
DOI：
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发表时间：
2005
期刊：
影响因子：
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通讯作者：
R. Herndon
中科院分区：
文献类型：
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作者：
R. Herndon
各种病因的疾病。其范围从明显的感染性疾病（如进行性多灶性白质脑病）到遗传性代谢性疾病（如肾上腺白质营养不良和异染性白质营养不良），再到一组病因不明的疾病（表14.1）。多发性硬化症（MS）及其变体是否包括一种具有高度变化的病程的单一疾病或一组不同病因的综合征仍然存在争议，尽管最近的证据表明它实际上涉及至少四种不同的病理变体（1）。许多临床脱髓鞘疾病可能是MS谱的一部分，或者可能代表具有相似临床和病理特征的不同疾病。多年来，许多临床医生认为原发性进行性MS和Devic综合征（视神经肌萎缩症）与复发缓解型和继发进行性MS不同。Balo同心圆硬化症被认为是一种不同的疾病，但最近的证据表明，它是复发缓解型MS的一种变异。无论是马尔堡变异型和急性播散性脑脊髓炎（ADEM），是MS谱的一部分还是代表不同的疾病实体仍然是一个争论的问题。最近的免疫病理学研究开始澄清这些问题，但最终，澄清的变异相互之间的关系将不得不等待这些条件的病因的确定。免疫病理学的最新发展表明，MS是几种不同的疾病，具有共同的临床表现（1）。这些免疫病理学上不同的疾病可能具有不同的病因。格林-巴利综合征已被证明有几种由不同病原体引起的变体，包括空肠弯曲菌、巨细胞病毒、EB病毒、肺炎支原体、猪流感疫苗，可能还有其他几种（2-4）。典型的复发缓解型MS现在似乎有几种变体，这些变体可能具有不同的病因。不同病例中的炎性脱髓鞘具有彼此充分不同的免疫学特征，表明这确实是一组疾病（1）。这些脱髓鞘疾病似乎有中央髓鞘和形成中央髓鞘的细胞，少突胶质细胞，作为攻击目标。MS通常不影响外周髓鞘，尽管存在临床和病理上与MS无法区分的疾病病例，其中也存在外周脱髓鞘，其模式与慢性炎性脱髓鞘性多发性神经病无法区分（5-7）。因为有很好的证据表明分子模拟与格林-巴利综合征有关（3），共同表位的分子模拟可能导致这种罕见的cen-
of illnesses with varied etiology. They range from clearly infectious diseases such as progressive multifocal leucoencephalopathy to hereditary metabolic disorders such as adrenoleucodystrophy and metachromatic leucodystrophy to a group of disorders of unknown etiology (Table 14.1). Whether multiple sclerosis (MS) and its variants comprise a single disease with a highly varied course or a group of syndromes of diverse etiology remains controversial, although recent evidence suggests that it actually involves at least four different pathologic variants (1). Numerous clinical demyelinating disorders may be part of the MS spectrum or may represent different diseases with similar clinical and pathologic features. For years, many clinicians have felt that primary progressive MS and Devic syndrome (neuromyelitis optica) were distinct from relapsing remitting and secondary progressive MS. Balo concentric sclerosis was felt to be a different disease, but recent evidence has suggested that it is a variant of relapsing remitting MS. Whether the Marburg variant and acute disseminated encephalomyelitis (ADEM) are part of the MS spectrum or represent different disease entities remains a matter of debate. Recent immunopathologic studies are beginning to clarify these issues, but, ultimately, clarification of the relationship of the variants to each other will have to await determination of the etiology of these conditions. Recent developments in immunopathology have suggested that MS is several different diseases with a common clinical presentation (1). These immunopathologically different disorders are likely to have different etiologies. Guillian-Barre syndrome has been shown to have several variants caused by different agents including Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumonia, swine flu vaccine, and probably several others (2–4). Typical relapsing remitting MS now appears to have several variants, and these are likely to have different etiologies. The inflammatory demyelination in different cases has immunologic features that are sufficiently different from each other to suggest that this is really a group of diseases (1). These demyelinating disorders appear to have central myelin and the cells that form central myelin, the oligodendrocytes, as the target of attack. MS usually does not affect peripheral myelin, although there are cases of a disease clinically and pathologically indistinguishable from MS in which there is also peripheral demyelination in a pattern indistinguishable from chronic inflammatory demyelinating polyneuropathy (5–7). Because there is good evidence that molecular mimicry is involved in Guillian-Barre syndrome (3), molecular mimicry of a shared epitope could cause this rare combination of cen-
影响因子：
14.5
作者：
Lucchinetti, CF;Mandler, RN;Lassmann, H
通讯作者：
Lassmann, H