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[从内科医生角度看重症肌无力]

[Myasthenia, from the internist's point of view].

作者信息

Eymard B

机构信息

Unité de pathologie neuromusculaire, service de neurologie 2, centre de référence de pathologie neuromusculaire Paris Est, hôpital de la Pitié-Salpêtrière, institut de myologie, bâtiment Babinski, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France.

出版信息

Rev Med Interne. 2014 Jul;35(7):421-9. doi: 10.1016/j.revmed.2013.08.013. Epub 2013 Oct 7.

Abstract

Myasthenia gravis is an autoimmune disease due to specific antibodies inducing a neuromuscular transmission defect causing muscle fatigability. If onset of the disease may be at any age, myasthenia gravis concerns mostly young adults, in majority females. The disease characteristic features are the following: ocular symptoms (ptosis or diplopia) as main initial manifestation, extension to other muscles in 80 % of the cases, variability of the deficit, effort induced worsening, successive periods of exacerbation during the disease course, severity depending on respiratory and swallowing impairment (if rapid worsening, a myasthenic crisis is to be suspected), association with thymoma in 20 % of patients and with other various autoimmune diseases, most commonly hyperthyroidism and Hashimoto's disease. Diagnosis relies on the clinical features, improvement with cholinesterase inhibitors, detection of specific autoantibodies (anti-AChR or anti-MuSK), and significant decrement evidenced by electrophysiological tests. The points concerning specifically the internist have been highlighted in this article: diagnostic traps, associated autoimmune diseases, including inflammatory myopathies that may mimic myasthenia gravis, adverse effects of medications commonly used in internal medicine, some of them inducing myasthenic syndromes. The treatment is well codified: the treatment is well codified: (1) respect of adverse drugs contra-indications, systematically use of cholinesterase inhibitors, (2) thymectomy if thymoma completed with radiotherapy if malignant, (3) corticosteroids or immunosuppressive agent in severe or disabling form, (4) intensive care unit monitoring, plasmapheresis or intravenous immunoglobulins for patients with myasthenic crisis.

摘要

重症肌无力是一种自身免疫性疾病,由于特定抗体导致神经肌肉传递缺陷,引起肌肉疲劳。虽然该病可在任何年龄发病,但主要累及年轻成年人,多数为女性。该病的特征如下:眼部症状(上睑下垂或复视)为主要初始表现,80%的病例会扩展至其他肌肉,症状有可变性,用力会导致症状加重,病程中会有连续的病情加重期,严重程度取决于呼吸和吞咽功能障碍(如果病情迅速恶化,则怀疑为重症肌无力危象),20%的患者伴有胸腺瘤,还可与其他多种自身免疫性疾病相关,最常见的是甲状腺功能亢进和桥本氏病。诊断依靠临床特征、胆碱酯酶抑制剂治疗有效、检测特定自身抗体(抗乙酰胆碱受体或抗肌肉特异性激酶)以及电生理检查显示的显著递减。本文特别强调了内科医生关注的要点:诊断陷阱、相关自身免疫性疾病,包括可能酷似重症肌无力的炎性肌病、内科常用药物的不良反应,其中一些会诱发重症肌无力综合征。治疗方法已明确规范:(1)注意药物禁忌,系统性使用胆碱酯酶抑制剂;(2)如有胸腺瘤则行胸腺切除术,若为恶性则术后辅以放疗;(3)对重症或致残患者使用皮质类固醇或免疫抑制剂;(4)对重症肌无力危象患者进行重症监护病房监测、血浆置换或静脉注射免疫球蛋白。

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