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在一个未经过挑选的土耳其重症肌无力患者队列中重症肌无力危象的发生率和严重程度。

Occurrence and severity of myasthenic crisis in an unselected Turkish cohort of patients with myasthenia gravis.

作者信息

Ozyurt Kose Selen, Nazli Ezgi, Tutkavul Kemal, Gilhus Nils Erik

机构信息

Department of Clinical Neurophysiology, Marmara University Pendik Teaching and Research Hospital, Istanbul, Turkey.

Department of Neurology, Health Sciences University Haydarpasa Numune Teaching and Research Hospital, Istanbul, Turkey.

出版信息

Front Neurol. 2023 Jul 13;14:1201451. doi: 10.3389/fneur.2023.1201451. eCollection 2023.

Abstract

Myasthenia gravis (MG) is a disorder of the neuromuscular junction that can deteriorate into myasthenic crisis, involving weakness of bulbar and respiratory muscles. In this study, we describe the clinical manifestations of myasthenic crisis, identify risk factors, and examine treatments and outcomes. All 95 patients with generalized MG treated at our center during the last 10 years were included in this retrospective study. We collected data from the patients' records, including clinical follow-ups, muscle antibodies, thymic status, and treatments. The characteristics of patients who did and did not experience myasthenic crisis were compared. Features of all myasthenic crises were also assessed. Twelve patients (13%) developed myasthenic crisis during the observation period. Men were more often affected at older ages. Seven patients experienced multiple myasthenic crises. Thymoma increased the risk of a crisis, whereas thymic hyperplasia decreased the risk. Myasthenic crises were more common in the summer months. No patients died during a myasthenic crisis. Risk factors for myasthenic crisis were thymoma, older age, MuSK antibodies, and previous crises. Individualized and active immunosuppressive treatment and optimal intensive care during crises provide a good outcome for patients with generalized MG.

摘要

重症肌无力(MG)是一种神经肌肉接头疾病,可恶化为重症肌无力危象,累及延髓和呼吸肌的无力。在本研究中,我们描述了重症肌无力危象的临床表现,确定了危险因素,并研究了治疗方法和结果。本回顾性研究纳入了过去10年在我们中心接受治疗的所有95例全身型MG患者。我们从患者记录中收集数据,包括临床随访、肌肉抗体、胸腺状态和治疗情况。比较了发生和未发生重症肌无力危象的患者特征。还评估了所有重症肌无力危象的特征。12例患者(13%)在观察期内发生了重症肌无力危象。男性在年龄较大时更易受累。7例患者经历了多次重症肌无力危象。胸腺瘤增加了危象风险,而胸腺增生则降低了风险。重症肌无力危象在夏季更为常见。没有患者在重症肌无力危象期间死亡。重症肌无力危象的危险因素包括胸腺瘤、年龄较大、MuSK抗体和既往危象。个体化且积极的免疫抑制治疗以及危象期间的最佳重症监护可为全身型MG患者带来良好的结果。

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