乙酰胆碱受体抗体和肌联蛋白抗体双阳性重症肌无力患者的临床分析
The Clinical Analysis of Myasthenia Gravis Patients with AChR Antibody and Titin Antibody Double Positive
DOI: 10.12677/ACM.2022.12111570, PDF,   
作者: 陈丹丹：济宁医学院，山东 济宁；孔庆霞*：济宁医学院附属医院，山东 济宁
关键词: 重症肌无力；乙酰胆碱抗体；肌联蛋白抗体；双抗体阳性；临床特征；Myasthenia Gravis； AChR Antibody； Titin Antibody； Double Positive Antibody； Clinical Features
摘要: 目的：总结乙酰胆碱受体抗体(AChR-Ab)和肌联蛋白抗体(Titin-Ab)双阳性的重症肌无力(Myasthenia Gravis, MG)患者的临床特点、治疗效果及预后，以提高我们对此类患者诊治的正确认识。方法：回顾性收集2021年6月至2022年8月在济宁医学院附属医院神经内科确诊的AChR-Ab及Titin-Ab双阳性的重症肌无力患者的临床资料，总结分析其临床特点、抗体结果及治疗预后的情况。结果：共纳入患者23例，其中男10例，女13例；发病年龄平均(60.87 ± 2.86)岁。最常累及眼外肌、躯干肌、咽喉肌。其中5例(23.53%)提示胸腺病变。按照美国重症肌无力协会(MGFA)标准对患者疗效进行评估，治疗后临床症状达到药物缓解(PR) 4例、最小临床表现(MM) 11例，症状改善(I) 6例，症状不变(U) 1例，症状恶化(W) 1例，治疗有效率91.30%。结论：乙酰胆碱受体抗体(AChR-Ab)和肌联蛋白抗体(Titin-Ab)双阳性的重症肌无力大部分属于晚发型，眼外肌、躯干肌和咽喉肌受累最常见，胸腺病变发生率高，激素治疗、免疫治疗有效。
Abstract: Objective: To summarize the clinical characteristics, antibody results, therapeutic effect and prog-nosis of myasthenia gravis (MG) patients with double positive acetylcholine receptor antibody (AChR-Ab) and Titin antibody (Titin-Ab), so as to improve our correct understanding of the diagno-sis and treatment of this kind of patients. Methods: Clinical data of patients with myasthenia gravis who were diagnosed as double positive for AchR-Ab and Titin-Ab in the Department of Neurology, Affiliated Hospital of Jining Medical University from June 2021 to August 2022 were retrospectively collected, and their clinical characteristics, antibody results and treatment prognosis were summa-rized and analyzed. Results: A total of 23 patients with AchR-Ab and Titin-Ab double positive MG were enrolled, including 10 males and 13 females. The mean age of onset was 60.87 ± 2.86 years. The extraocular, trunk and throat muscles were most commonly involved. Five cases (23.53%) showed thymic lesions. According to the criteria of Myasthenia Gravis Society of America (MGFA), the clinical symptoms were relieved (PR) in 4 cases, minimal clinical manifestation (MM) in 11 cases, improved (I) in 6 cases, unchanged (U) in 1 case, worsened (W) in 1 case, and the effective rate was 91.30%. Conclusion: Myasthenia gravis with both AChR-Ab and Titin-Ab positive is mostly late-onset. Extraocular, trunk and throat muscles are the most common involved muscles. The ex-traocular, trunk and throat muscles were most commonly involved. Thymus lesions are common in myasthenia gravis. Hormone therapy and immunotherapy are effective.
文章引用：陈丹丹, 孔庆霞. 乙酰胆碱受体抗体和肌联蛋白抗体双阳性重症肌无力患者的临床分析[J]. 临床医学进展, 2022, 12(11): 10907-10913. https://doi.org/10.12677/ACM.2022.12111570

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