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以孤立性眼肌症状起病的重症肌无力临床多态性;五年回顾性分析

[Clinical polymorphism of myasthenia gravis beginning with isolated ocular symptoms; a five years retrospective analysis].

作者信息

Boumendil J, Clermont-Vignal C, Gout O, Fechner C, Dufier J-L, Morax S

机构信息

Service d'ophtalmologie, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France.

出版信息

J Fr Ophtalmol. 2010 Dec;33(10):728-38. doi: 10.1016/j.jfo.2010.09.005. Epub 2010 Nov 18.

Abstract

INTRODUCTION

myasthenia gravis is a neuromuscular junction disorder that can jeopardize the patient's life and has a high clinical polymorphism that makes it difficult to diagnose.

PATIENTS AND METHODS

after reviewing the disease physiology, its clinical symptoms, and the different means to diagnose and treat it, we present a 15-patient series that we cared for at the Rothschild ophthalmologic foundation from 2002 to 2007 for myasthenia gravis that began with isolated ocular symptoms, so as to highlight the clinical diversity of this pathology.

RESULTS

when the disease was diagnosed, 11 patients out of 15 had a ptosis with diplopia, two had an isolated ptosis, and two had isolated diplopia. After investigations, we discovered that three patients had a malignant thymoma and one had thymic hyperplasia. An autoimmune disease association was found in two patients: the first one had Hashimoto thyroiditis and the second one developed optical neuromyelitis a few years after his myasthenia gravis. Only three patients secondarily developed a generalized myasthenia gravis.

DISCUSSION

our series of patients has a low disease generalization rate in comparison with the published data in the medical literature, indicating that two-thirds of patients with ocular myasthenia gravis should develop generalized myasthenia gravis within approximately 2 years after the beginning of their illness. This could be explained by the early consultation of these patients and the common prescription of an immunosuppressive therapy, reducing the risk of secondarily generalized myasthenia gravis according to some studies.

CONCLUSION

despite the small number of patients, this study underlines the clinical polymorphism of ocular myasthenia gravis and the risks it may cause. Close collaboration between ophthalmologists and neurologists is needed to ensure good care for these patients.

摘要

引言

重症肌无力是一种神经肌肉接头疾病,可危及患者生命,且临床多态性高,诊断困难。

患者与方法

在回顾了该疾病的生理学、临床症状以及诊断和治疗的不同方法后,我们呈现了一组15例患者的病例系列,这些患者于2002年至2007年在罗斯柴尔德眼科基金会接受治疗,均为以孤立性眼部症状起病的重症肌无力患者,以突出这种疾病的临床多样性。

结果

确诊时,15例患者中有11例出现上睑下垂伴复视,2例仅有上睑下垂,2例仅有复视。经检查,我们发现3例患者患有恶性胸腺瘤,1例患有胸腺增生。在2例患者中发现了自身免疫性疾病关联:第一例患有桥本甲状腺炎,第二例在重症肌无力发病几年后患上视神经脊髓炎。只有3例患者继发全身性重症肌无力。

讨论

与医学文献中公布的数据相比,我们的患者系列疾病泛化率较低,这表明三分之二的单纯眼肌型重症肌无力患者在发病后约2年内应发展为全身性重症肌无力。根据一些研究,这可能是由于这些患者早期就诊以及免疫抑制治疗的普遍使用,降低了继发全身性重症肌无力的风险。

结论

尽管患者数量较少,但本研究强调了单纯眼肌型重症肌无力的临床多态性及其可能导致的风险。眼科医生和神经科医生需要密切合作,以确保对这些患者的良好护理。

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