Cortical demyelination in PML and MS - Similarities and differences
Cortical demyelination in PML and MS - Similarities and differences
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DOI：
10.1212/01.wnl.0000284601.54436.e4
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发表时间：
2008-01-29
期刊：
影响因子：
9.9
通讯作者：
Ransohoff, R. M.
中科院分区：
文献类型：
--
作者：
Moll, N. M.;Rietsch, A. M.;Ransohoff, R. M.
目的：为了解多发性硬化（MS）和进行性多灶性白质脑病（PML）患者大脑皮质的病理变化，方法：13例PML患者、4例MS患者、2例HIV脑病患者和1例无神经病理学改变的患者的脑组织进行尸检。髓鞘蛋白，炎症细胞和神经丝的免疫组织化学进行评估皮质病变的分布，其炎症活动，和神经炎病理。结果：多发性硬化症患者脑组织中存在皮质内、皮质内和软膜下的脱髓鞘改变。在PML脑组织中，观察到皮质内和皮质白质病变，但未观察到软膜下病变。PML和MS中的皮质病变比白色物质中的脱髓鞘区域含有更少的炎性细胞。皮质PML病变中的神经炎病理表现为营养不良和横断的神经突。在PML的神经炎过程中的病理修改是更明显的高度发炎的白色物质比在灰质脱髓鞘的领域，让人想起以前的报告神经炎病理MS。JC病毒感染的细胞与PML白色物质，leukocortical和intracorticallesions.Conclusions：皮质病理学代表了一个独特的功能进行性多灶性白质脑病。注意到多发性硬化症皮质病理学的相似性和差异，并可能提供有关这两种疾病发病机制的信息。
Objective: To characterize pathologic changes in the cerebral cortex of patients with multiple sclerosis ( MS) and progressive multifocal leukoencephalopathy ( PML).Methods: Autopsy brain tissue was obtained from 13 patients with PML, 4 patients with MS, 2 patients with HIV encephalopathy, and 1 subject without neurologic pathology. Immunohistochemistry for myelin proteins, inflammatory cells, and neurofilaments was performed to evaluate the distribution of cortical lesions, their inflammatory activity, and neuritic pathology. Confocal microscopy was applied to examine pathologic changes in neurites in PML cortex.Results: Leukocortical, intracortical, and subpial patterns of cortical demyelination were represented in MS brain tissue. In PML brain tissue intracortical and leukocortical but not subpial lesions were observed. Cortical lesions in PML and MS contained fewer inflammatory cells than demyelinated areas in the white matter. Neuritic pathology in cortical PML lesions was represented by dystrophic and transected neurites. Pathologic modifications in neuritic processes in PML were more evident in highly inflamed white matter than in gray matter areas of demyelination, reminiscent of previous reports of neuritic pathology in MS. JC virus-infected cells were associated with PML white matter, leukocortical and intracortical lesions.Conclusions: Cortical pathology represents a distinct feature of progressive multifocal leukoencephalopathy. Similarities and differences with regard to multiple sclerosis cortical pathology were noted and may be informative regarding the pathogenesis of both disorders.