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伴有广泛脑实质受累的视神经脊髓炎:一项尸检研究

Neuromyelitis optica with extensive active brain involvement: an autopsy study.

作者信息

Almekhlafi Mohammed A, Clark Arthur W, Lucchinetti Claudia F, Zhang Yunyan, Power Christopher, Bell Robert B

机构信息

Department of Internal Medicine, King Abdulaziz University, Jeddah, Saudi Arabia.

出版信息

Arch Neurol. 2011 Apr;68(4):508-12. doi: 10.1001/archneurol.2011.62.

DOI:10.1001/archneurol.2011.62
PMID:21482930
Abstract

OBJECTIVE

To describe the clinical, molecular, and neuropathological findings of a patient with aquaporin 4-positive relapsing myelitis who developed extensive brain involvement followed by death.

DESIGN

Case report.

SETTING

Foothills Medical Center, Calgary, Alberta, Canada.

PATIENT

A 51-year-old woman with neuromyelitis optica spectrum disorder.

RESULTS

Neuropathological examination disclosed neuromyelitis optica lesions, even in areas that appeared normal radiologically and grossly. Immunostaining confirmed the massive disintegration of astrocytes in the acute and chronic lesions, indicating that astrocytes are targeted early in the disease process. Induction of the immune response was demonstrated by reverse-transcriptase polymerase chain reaction analysis of relevant immune response genes.

CONCLUSIONS

This article supports and supplements current concepts of astrocyte disintegration in neuromyelitis optica and of immune mechanisms in its pathogenesis.

摘要

目的

描述1例水通道蛋白4阳性复发性脊髓炎患者出现广泛脑受累并最终死亡的临床、分子及神经病理学表现。

设计

病例报告。

地点

加拿大艾伯塔省卡尔加里市山麓医疗中心。

患者

一名51岁患有视神经脊髓炎谱系障碍的女性。

结果

神经病理学检查发现视神经脊髓炎病变,即使在放射学和大体检查看似正常的区域也存在。免疫染色证实急性和慢性病变中星形胶质细胞大量崩解,表明星形胶质细胞在疾病进程早期即成为靶点。通过对相关免疫反应基因进行逆转录酶聚合酶链反应分析证实了免疫反应的诱导。

结论

本文支持并补充了目前关于视神经脊髓炎中星形胶质细胞崩解及其发病机制中免疫机制的概念。

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