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巴洛同心性硬化:在一位复发缓解型临床病程患者中存活的正常髓鞘。

Baló's concentric sclerosis: surviving normal myelin in a patient with a relapsing-remitting dinical course.

作者信息

Moore G R, Berry K, Oger J J, Prout A J, Graeb D A, Nugent R A

机构信息

Department of Pathology and Laboratory Medicine (Neuropathology), Vancouver Hospital and Health Services Centre, BC, Canada.

出版信息

Mult Scler. 2001 Dec;7(6):375-82. doi: 10.1177/135245850100700606.

DOI:10.1177/135245850100700606
PMID:11795459
Abstract

Baló's concentric sclerosis is a demyelinating disorder in which bands of demyelination alternate with concentric bands of myelin preservation. The pathogenesis of the lesion is unknown. Previous reports using modern histopathologic techniques have shown the bands of myelin preservation to be comprised of remyelinated or partially demyelinated myelin. Here we report a case of Baló's concentric sclerosis in a 24-year-old East Indian patient with a previous history of relapsing-remitting multiple sclerosis (MS). Pathologically, the bands of myelin preservation showed myelin sheaths of normal thickness, with focal areas of demyelination. The findings, taken together with those of previously reported cases, suggest that Baló's concentric sclerosis is a variant of MS, and the concentric lesion may be an intermediary form in evolution of a chronic active MS plaque. The pathogenesis of this concentric lesion may be explained by periodic suppression of demyelination in the rapidly expanding border, allowing remyelination or only transient incomplete demyelination to occur.

摘要

巴洛同心性硬化是一种脱髓鞘疾病,其中脱髓鞘带与髓鞘保留的同心带交替出现。该病变的发病机制尚不清楚。以往使用现代组织病理学技术的报告显示,髓鞘保留带由再髓鞘化或部分脱髓鞘的髓鞘组成。在此,我们报告一例24岁东印度患者的巴洛同心性硬化病例,该患者既往有复发缓解型多发性硬化(MS)病史。病理上,髓鞘保留带显示髓鞘厚度正常,有局灶性脱髓鞘区域。这些发现与先前报道病例的发现一起表明,巴洛同心性硬化是MS的一种变体,同心性病变可能是慢性活动性MS斑块演变过程中的一种中间形式。这种同心性病变的发病机制可以通过快速扩展边缘处脱髓鞘的周期性抑制来解释,从而允许再髓鞘化或仅发生短暂的不完全脱髓鞘。

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