Clinical Spectrum and Therapeutic Outcome of Myasthenia Gravis in a Tertiary Care Hospital: A Retrospective Study.

Q3 Medicine
Sujaya Raghavendra, Jawahar Marimuthu, Daisy Precilla Senthilathiban, Balasubramanian Samivel
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Abstract

Introduction: Myasthenia gravis (MG) is characterized by defective transmission of electric impulses across the neuromuscular junction. To date, there is a lack of real-world data to understand its management, specifically in the Indian context. To bridge this gap in clinical knowledge, the current study was designed to understand the clinical management of MG in the Indian population.

Materials and methods: This retrospective study included clinical data of MG patients at Madras Medical College, Chennai (2021-2023). Patients were assessed for demographics, clinical characteristics, therapeutic interventions, and clinical outcome. Statistical analysis was performed using GraphPad Prism v.8.0.1.

Results: A total of 49 MG cases were observed, and 14 patients presented with myasthenic crisis (MC). Young onset was observed in 26 cases. Clinical features included ptosis (90%), diplopia (90%), bulbar symptoms (60%), and respiratory illness (19.2%). Bedside tests depicted a decremental response to repetitive nerve stimulation (RNS) (93.8%), acetylcholine receptor (AChR) antibody positivity (45%), and MUSK Ab positivity (3.8%). Treatment comprised intravenous immunoglobulin (IVIG) (10.79%), plasmapheresis (PLEX) for impending crisis or thymoma (3.5%), and combination therapy using acetylcholine esterase inhibitors with immunomodulators (63.26%). Mortality was observed in 4 cases (8.1%), while the remaining 45 MG patients (91.84%) exhibited stable vitals with a median hospital stay of 14 days. Thymectomy had potentially reduced the risk of crisis.

Discussion and conclusion: Early detection of MG can prevent the onset of crisis. Electrophysiological studies were crucial in the diagnosis of atypical cases. Rituximab successfully normalized bulbar and respiratory functions in PLEX/IVIG-resistant patients, enhancing crisis response. Combining AChE inhibitors with immunomodulators ensured good drug compliance and, thereby, reduced the risk of crisis and enabled effective management of MG. Centric studies like this may serve as reference points for designing more extensive studies on MG involving multiple centers nationwide.

三级医院重症肌无力的临床谱和治疗结果:回顾性研究。
简介:重症肌无力(MG)的特点是电脉冲在神经肌肉交界处的传输有缺陷。迄今为止,缺乏真实世界的数据来了解其管理,特别是在印度的背景下。为了弥补临床知识上的差距,本研究旨在了解印度人群中MG的临床管理。材料和方法:本回顾性研究纳入了金奈马德拉斯医学院(Madras Medical College) 2021-2023年MG患者的临床资料。评估患者的人口统计学、临床特征、治疗干预和临床结果。使用GraphPad Prism v.8.0.1进行统计分析。结果:共观察49例MG患者,14例出现肌无力危象(MC)。年轻发病26例。临床特征包括上睑下垂(90%)、复视(90%)、球症状(60%)和呼吸系统疾病(19.2%)。床边试验显示对重复神经刺激(RNS)的反应减少(93.8%),乙酰胆碱受体(AChR)抗体阳性(45%),MUSK Ab阳性(3.8%)。治疗包括静脉注射免疫球蛋白(IVIG)(10.79%),危重症或胸腺瘤的血浆置换(PLEX)(3.5%),以及乙酰胆碱酯酶抑制剂和免疫调节剂的联合治疗(63.26%)。死亡4例(8.1%),其余45例(91.84%)MG患者生命体征稳定,中位住院时间为14天。胸腺切除术潜在地降低了危机的风险。讨论与结论:早期发现MG可以预防危机的发生。电生理研究对非典型病例的诊断至关重要。利妥昔单抗成功地使PLEX/ ivig耐药患者的球和呼吸功能正常化,增强了危机反应。乙酰胆碱酯酶抑制剂联合免疫调节剂确保了良好的药物依从性,从而降低了危机风险,使MG得到有效管理。这样的中心研究可以作为设计涉及全国多个中心的更广泛的MG研究的参考点。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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CiteScore
0.80
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