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接受胸腺切除术的重症肌无力患者中的视神经脊髓炎。

Neuromyelitis optica in patients with myasthenia gravis who underwent thymectomy.

作者信息

Kister Ilya, Gulati Sandeep, Boz Cavit, Bergamaschi Roberto, Piccolo Giovanni, Oger Joel, Swerdlow Michael L

机构信息

Department of Neurology, Albert Einstein College of Medicine, Bronx, NY 10467, USA.

出版信息

Arch Neurol. 2006 Jun;63(6):851-6. doi: 10.1001/archneur.63.6.851.

DOI:10.1001/archneur.63.6.851
PMID:16769866
Abstract

BACKGROUND

Myasthenia gravis (MG) and neuromyelitis optica (NMO, also known as Devic disease) are rare autoimmune disorders, with upper-limit prevalence estimates in the general population of 15 per 100,000 and 5 per 100,000, respectively. To our knowledge, an association between these diseases has not been previously reported.

OBJECTIVES

To describe 4 patients with MG who developed NMO after thymectomy and to analyze possible causes of apparent increased prevalence of NMO among patients with MG.

DESIGN

Case series.

PATIENTS

Four patients with MG who underwent thymectomy.

INTERVENTIONS

None.

RESULTS

The prevalence of MG within the published cohort of patients with NMO is more than 150 times higher than that in the general population.

CONCLUSION

Dysregulation of B-cell autoimmunity in myasthenia, possibly exacerbated by loss of control over autoreactive cells as a result of thymectomy, may predispose patients to the development of NMO.

摘要

背景

重症肌无力(MG)和视神经脊髓炎(NMO,也称为德维克病)是罕见的自身免疫性疾病,普通人群中的患病率估计上限分别为每10万人15例和每10万人5例。据我们所知,此前尚未报道过这些疾病之间存在关联。

目的

描述4例重症肌无力患者在胸腺切除术后发生视神经脊髓炎的情况,并分析重症肌无力患者中视神经脊髓炎明显患病率增加的可能原因。

设计

病例系列。

患者

4例行胸腺切除术的重症肌无力患者。

干预措施

无。

结果

已发表的视神经脊髓炎患者队列中重症肌无力的患病率比普通人群高150倍以上。

结论

重症肌无力中B细胞自身免疫失调,可能由于胸腺切除导致对自身反应性细胞的控制丧失而加剧,可能使患者易患视神经脊髓炎。

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