眼肌型重症肌无力的诊疗进展  

Progress in the diagnosis and treatment of ocular myasthenia gravis

作　　者：景筠[1] JING Yun(Department of Neurology,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China)

机构地区：[1]首都医科大学附属北京同仁医院神经内科,北京100730

出　　处：《中国眼耳鼻喉科杂志》2024年第5期360-367,共8页Chinese Journal of Ophthalmology and Otorhinolaryngology

摘　　要：眼肌型重症肌无力(OMG)的诊断主要依赖于临床病史及床旁试验(如冰袋试验、疲劳试验、睡眠试验等)、血清抗体(乙酰胆碱受体抗体、肌肉特异性络氨酸激酶抗体和低密度脂蛋白受体相关蛋白4抗体)测定及电生理学检查(重复神经刺激、单纤维肌电图)。不同辅助检查的合理组合有助于提高其对OMG诊断的敏感性和特异性。OMG的治疗方法有对症治疗和免疫抑制(IS)治疗。眼肌麻痹患者通常需要糖皮质激素(GC)治疗。GC停药后复发或对GC不耐受的患者可能需要其他IS疗法,包括传统免疫抑制剂甚至靶向生物制剂。对症治疗疗效不好、不愿接受IS治疗或有IS治疗禁忌证、或表现为难治性乙酰胆碱受体抗体+OMG的患者,早期胸腺切除也是一种积极的治疗选择。本文对近年来国内外文献在OMG诊断、治疗及预后方面的进展进行了综述,旨在提高临床医师对OMG的认识,促使临床医师对OMG尽可能实行早期诊断、合理治疗,以获得更好的治疗结局。The diagnosis of ocular myasthenia gravis(OMG)relies primarily on clinical history,bedside tests(such as ice pack test,fatigue test,sleep test,etc.),serum antibody measurement(AChR,MuSK,and LRP-4 antibody),and electrophysiological examination(RNS,SFEMG).A reasonable combination of different ancillary tests can enhance their sensitivity and specificity for diagnosing OMG.Treatment for OMG includes symptomatic treatment and immunosuppressive(IS)therapy.Patients with ophthalmoplegia typically require glucocorticoid(GC)therapy.Those who experience relapse after discontinuing GC or are intolerant to them may necessitate alternative is therapies such as traditional immunosuppressants or even targeted biologics.Early thymectomy is also an active treatment option for patients who do not respond well to symptomatic therapy or are unwilling to undergo IS therapy or have contraindications to it,or present with refractory AChR-Ab+OMG.This article reviews the recent literature progress on the diagnosis,treatment and prognosis of OMG,aiming to enhance clinicians’understanding of OMG and encourage early diagnosis and appropriate treatment in order to achieve better outcomes.

关 键 词：眼肌型重症肌无力 诊断试验 免疫抑制 靶向生物制剂 胸腺切除术 

分 类 号：R774[医药卫生—眼科]