Tireli H, Karlikaya G, Tutkavul K, Akpinar A, Okay T
Haydarpaşa Numune Education and Research Hospital, 2nd Department of Neurology, Istanbul, Turkey.
Acta Myol. 2004 Dec;23(3):140-5.
Myasthenia Gravis is an acquired autoimmune disorder caused by a neuromuscular transmission defect which is clinically characterized by fluctuating weakness of voluntary muscles and fatigability. It can be diagnosed by clinical features, clinical, pharmacological and electrophysiological tests and serological evaluation. Treatment modalities include symptomatic treatment in the form of cholinesterase inhibitors and plasmapheresis and immunotherapy in the form of immunosuppressant medications, immunomodulating therapy and thymectomy. No single regimen is appropriate for all patients and up to now no mode of therapy has been proven to be clearly superior. The response to any form of treatment is difficult to assess because the severity of symptoms fluctuate. We retrospectively analyzed the clinical records of 33 myasthenia gravis patients which were managed at our clinic between 1995-2003. All patients were treated with anticholinesterase medications sometime during their treatment. Most patients recieved immunosupressant and/or immunomodulator therapy. Patients were referred for thymectomy when indicated. We evaluated the outcome with different treatment modalities, focusing on the role of thymectomy. We also investigated the possible correlations between clinicopathological features and clinical outcome. We conclude that as for the medical treatment of myasthenia gravis azathioprine plus steroid improves the outcome; and for the surgical treatment, early thymectomy should be performed in all generalize myasthenia patients.
重症肌无力是一种由神经肌肉传递缺陷引起的获得性自身免疫性疾病，其临床特征为随意肌波动性无力和易疲劳性。可通过临床特征、临床、药理学和电生理检查以及血清学评估进行诊断。治疗方式包括胆碱酯酶抑制剂形式的对症治疗、血浆置换以及免疫抑制剂药物、免疫调节治疗和胸腺切除术形式的免疫治疗。没有单一的治疗方案适用于所有患者，到目前为止，尚无一种治疗方式被证明明显更优。由于症状严重程度波动，难以评估对任何形式治疗的反应。我们回顾性分析了1995年至2003年在我们诊所接受治疗的33例重症肌无力患者的临床记录。所有患者在治疗期间的某个时间都接受了抗胆碱酯酶药物治疗。大多数患者接受了免疫抑制和/或免疫调节治疗。有指征时患者被转诊进行胸腺切除术。我们评估了不同治疗方式的结果，重点关注胸腺切除术的作用。我们还研究了临床病理特征与临床结果之间可能的相关性。我们得出结论，对于重症肌无力的药物治疗，硫唑嘌呤加类固醇可改善结果；对于手术治疗，所有全身型重症肌无力患者均应尽早进行胸腺切除术。
