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一名急性多发性硬化症患者正常外观白质中的轴突损失。

Axonal loss in normal-appearing white matter in a patient with acute MS.

作者信息

Bjartmar C, Kinkel R P, Kidd G, Rudick R A, Trapp B D

机构信息

Department of Neurosciences, Lerner Research Institute, Cleveland Clinic Foundation, OH 44195, USA.

出版信息

Neurology. 2001 Oct 9;57(7):1248-52. doi: 10.1212/wnl.57.7.1248.

Abstract

BACKGROUND

Brain imaging studies detect abnormalities in normal-appearing white matter in patients with MS.

OBJECTIVE

To investigate the histopathologic basis for these changes in autopsy tissue from a patient with MS with 9 months' disease duration and a terminal brain stem lesion.

METHODS

The brain stem and spinal cord were analyzed ultrastructurally and immunocytochemically for axons, myelin, and activated microglia/macrophages.

RESULTS

Pathologic findings were consistent with a terminal inflammatory demyelinated lesion at the cervicomedullary junction. The ventral spinal cord column, containing descending tracts, exhibited 22% axonal loss at segment C7, but grossly normal immunostaining for myelin. Confocal and electron microscopy revealed myelin sheaths without axonal content and initial stages of myelin degradation by activated microglia/macrophages among intact myelinated axons. Axonal number and appearance was normal in ascending sensory tracts.

CONCLUSIONS

These studies confirm axonal degeneration in the absence of myelin loss as one histopathologic correlate to abnormal MR findings in patients with MS.

摘要

背景

脑成像研究发现多发性硬化症(MS)患者正常外观的白质存在异常。

目的

研究一名病程9个月且患有终末期脑干病变的MS患者尸检组织中这些变化的组织病理学基础。

方法

对脑干和脊髓进行超微结构分析以及针对轴突、髓鞘和活化小胶质细胞/巨噬细胞的免疫细胞化学分析。

结果

病理结果与颈髓交界处的终末期炎性脱髓鞘病变一致。包含下行束的脊髓腹侧柱在C7节段显示22%的轴突损失,但髓鞘免疫染色大体正常。共聚焦显微镜和电子显微镜显示髓鞘鞘膜内无轴突成分,且在完整的有髓轴突中存在活化小胶质细胞/巨噬细胞导致的髓鞘降解初期阶段。上行感觉束中的轴突数量和外观正常。

结论

这些研究证实,在无髓鞘损失的情况下轴突变性是MS患者异常磁共振成像结果的一种组织病理学关联。

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