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重症肌无力和兰伯特-伊顿肌无力综合征的治疗。

Therapy in myasthenia gravis and Lambert-Eaton myasthenic syndrome.

作者信息

Newsom-Davis John

机构信息

Department of Clinical Neurology, University of Oxford, Radcliffe Infirmary, Woodstock Road, Oxford OX2 6HE, United Kingdom.

出版信息

Semin Neurol. 2003 Jun;23(2):191-8. doi: 10.1055/s-2003-41135.

Abstract

Myasthenia gravis (MG) is a heterogeneous disorder, a fact that needs to be kept in mind when considering treatment. Most patients benefit from pyridostigmine. In nonthymomatous ocular MG, prednisolone is often effective. Thymectomy is indicated for thymoma and is an option for acetylcholine receptor antibody-positive patients with generalized weakness developing under the age of 45 years. In older patients and in those failing to respond to thymectomy, prednisone alone or combined with azathioprine is the treatment of choice. Mycophenolate mofetil is an option in those intolerant of azathioprine. Lambert-Eaton myasthenic syndrome (LEMS) can exist in paraneoplastic (P-) and nonparaneoplastic (NP-) forms. Most patients benefit from 3,4-diaminopyridine. In P-LEMS, treatment of the tumor often results in neurological improvement. In both forms, prednisone alone is an option or combined with azathioprine in NP-LEMS. In both MG and LEMS, where weakness is severe, plasma exchange or intravenous immunoglobulin treatment may provide short-term benefit.

摘要

重症肌无力(MG)是一种异质性疾病,在考虑治疗时需要牢记这一事实。大多数患者从吡啶斯的明中获益。在非胸腺瘤性眼肌型MG中,泼尼松龙通常有效。胸腺切除术适用于胸腺瘤,对于45岁以下出现全身无力的乙酰胆碱受体抗体阳性患者也是一种选择。在老年患者以及对胸腺切除术无反应的患者中,单独使用泼尼松或与硫唑嘌呤联合使用是首选治疗方法。霉酚酸酯是不能耐受硫唑嘌呤患者的一种选择。兰伯特-伊顿肌无力综合征(LEMS)可分为副肿瘤性(P-)和非副肿瘤性(NP-)两种形式。大多数患者从3,4-二氨基吡啶中获益。在P-LEMS中,肿瘤的治疗通常会导致神经功能改善。在两种形式中,单独使用泼尼松是一种选择,在NP-LEMS中可与硫唑嘌呤联合使用。在MG和LEMS中,当肌无力严重时,血浆置换或静脉注射免疫球蛋白治疗可能会提供短期益处。

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