Ocular Myasthenia Gravis in Slovak Republic

Česká verzia

Authors: I. Martinka ¹; F. Cibulčík ¹; J. Bednařík ²; P. Špalek ¹
Authors place of work: Centrum pre neuromuskulárne ochorenia, Neurologická klinika SZU a UN Bratislava ¹; Neurologická klinika LF MU a FN Brno ²
Published in the journal: Cesk Slov Neurol N 2017; 80/113(2): 190-196
Category: Original Paper
doi: https://doi.org/10.14735/amcsnn2017190

Summary

Background:
Ocular symptoms are the most common initial symptoms of myasthenia gravis (MG) and they generalize rapidly in the majority of MG patients. In purely ocular MG (OMG), impairment of neuromuscular transmission remains limited to extraocular muscles even after three years from OMG onset.

Aim:
We performed retrospective longitudinal study in 946 MG patients with initial ocular symptoms (oMG) registered between 1978 and 2015 at the Slovak Centre for Neuromuscular Diseases. The aim of this study was to analyze clinical assessments, laboratory findings and prognostic factors in oMG.

Material and methods:
We analyzed data and findings in medical records of oMG patients including age at onset, sex, autoantibodies against acetylcholine receptors (AChRs) and muscle-specific kinase (MuSK), a type of clinical symptomatology. We evaluated the therapies used, clinical status at the last examination and prognosis in this initial clinical pattern.

Results:
Impairment of ocular muscles as the first MG sign occurred in 946 patients (M = 487, F = 459). oMG generalized in due course in 554 (58.6%) patients. We identified male gender (RR 1.23), age at onset of over 50 years (RR 1.23), association with other autoimmune diseases (RR 1.57), AChR - seropositivity (RR 2.09) and thymoma (RR 15.71) as the risk factors of oMG generalization. We identified female gender (RR 1.2), AChR - seronegativity (RR 1.58), oMG generalization (RR 1.63) and age at onset of below 50 years (RR 2.08) as predictive factors of unfavourable outcome (repeated exacerbations, unfavourable post-intervention status).

Conclusion:
To improve oMG prognosis, it is important to recognize the oMG patients with high risk of generalization and/or unfavourable outcome and to select appropriate therapy for these patients.

Key words:
myasthenia gravis – ocular myasthenia gravis – diagnosis – prognosis – generalization

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Chinese summary - 摘要

斯洛伐克共和国眼科重症肌无力

背景：

眼部症状通常是重症肌无力（MG）的最初表现，并且它们在大多数MG患者中发展的很迅速。在纯粹的眼部MG（OMG）中，即使在OMG发病三年后，仍然存在有限的存在损伤的神经肌肉传播到眼外肌。

目标：

我们对1978年至2015年间在斯洛伐克神经肌肉疾病中心登记的946名MG患者进行了追溯性纵向研究。本研究的目的是分析oMG中的临床评估，实验室检查结果和预后因素。

材料和方法：

我们分析了oMG患者的病历数据，包括发病年龄，性别，乙酰胆碱受体（AChR）和肌肉特异性激酶（MuSK）的一种临床症状学类型。我们评估了使用的治疗方法，最后一次检查时的临床状态和初步临床模式的预后。

结果：

946例MG患者均首次出现了眼肌损伤的征兆（男 = 487，女 = 459）。 oMG在554例（58.6％）患者中适时推广。我们确定男性性别（RR 1.23），50岁以上发病（RR 1.23），与其他自身免疫性疾病（RR 1.57），AChR-血清阳性（RR 2.09）和胸腺瘤（RR 15.71）的关系作为oMG的危险因素。我们确定女性性别（RR 1.2），AChR - 血清反应性（RR 1.58），oMG泛化（RR 1.63）和50岁以下发病（RR 2.08）作为不良后果的预测因素（重复加重，不良干预后状态）。

结论：

为了改善oMG预后，重要的是要识别具有广泛性风险和/或不利后果的oMG患者，并为这些患者选择合适的治疗方法。

关键词：

重症肌无力 - 重症肌无力 - 诊断 - 预后 - 泛化

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