Kroczka Sławomir, Pietrzyk Jacek A, Steczkowska Małgorzata, Skowronek-Bała Barbara
Klinika Neurologii Dzieciecej Katedry Neurologii Dzieci i Młodziezy, Kraków.
Przegl Lek. 2010;67(11):1213-6.
Myasthenia gravis (MG) is a disease with autoimmune background. Impaired neuromuscular transmission is caused by blockage of acetylcholine receptors on postsynaptic membrane by circulating specific antibodies. Recognition of myasthenia gravis in children, especially its ocular type, may be difficult due to occurrence of similar clinical symptoms in other diseases like ecephalomyopathies. MG is characterized by variety of clinical symptoms and their alternations during excercise and rest.
Case report of nearly 18-year-old girl with generalized type of MG.
Before hospitalization the girl had been treated psychiatrically for 6 months due to suspicion of conversion disorders. After performance of clinical test and electro-neurophysiological examinations mysathenia gravis was diagnosed and conservative treatment was instituted. Additionally, on the ground of low effectiveness of the treatment, sterydotherapy and immunosuppressive treatment were instituted without marked clinical improvement. Thymectomy was also low effective. Thymic inflammation was recognised histopathologically. Only after performance of 5 plasmapheresis was significant clinical improvement achieved.
Plasmapheresis may be used not only in the treatment of myasthenic crisis but also in the treatment of drug-resistant mysthenia gravis.
重症肌无力（MG）是一种具有自身免疫背景的疾病。循环中的特异性抗体阻断突触后膜上的乙酰胆碱受体，导致神经肌肉传递受损。由于在其他疾病如脑肌病中会出现类似的临床症状，儿童重症肌无力，尤其是眼肌型，可能难以识别。重症肌无力的特点是临床症状多样，且在运动和休息时会交替出现。
报告一名近18岁的全身型重症肌无力女孩的病例。
住院前，该女孩因疑似转换障碍接受了6个月的精神科治疗。在进行临床检查和神经电生理检查后，诊断为重症肌无力并开始进行保守治疗。此外，由于治疗效果不佳，开始使用类固醇疗法和免疫抑制治疗，但临床改善不明显。胸腺切除术效果也不佳。组织病理学检查发现胸腺炎症。仅在进行5次血浆置换后才取得了显著的临床改善。
血浆置换不仅可用于治疗重症肌无力危象，也可用于治疗耐药性重症肌无力。
