Sanders Donald B, Guptill Jeffrey T
Continuum (Minneap Minn). 2014 Oct;20(5 Peripheral Nervous System Disorders):1413-25. doi: 10.1212/01.CON.0000455873.30438.9b.
This article reviews the clinical presentations, diagnostic findings, and treatment options for autoimmune myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome.
Immunologic research is unraveling the immunopathology of MG and identifying targets for novel immune-based therapy of this condition. MG patients with antibodies to muscle-specific tyrosine kinase (MuSK) frequently present with symptoms and clinical findings that suggest nerve or muscle disease.
Early diagnosis and treatment have a marked effect on outcome in these diseases. In most cases, the diagnosis of MG or Lambert-Eaton myasthenic syndrome can be made from the history, supplemented with directed questions, and a physical examination designed to demonstrate variable weakness in affected muscle groups. Appropriate confirmatory tests almost always establish the diagnosis. Although several novel treatment modalities for MG are under investigation, currently available therapies produce substantial improvement in function and quality of life in most patients with this condition. Knowledge about the dosing, adverse effects, and costs of immunomodulatory therapies is essential for the effective management of patients with MG and Lambert-Eaton myasthenic syndrome.
本文综述自身免疫性重症肌无力(MG)和兰伯特 - 伊顿肌无力综合征的临床表现、诊断结果及治疗选择。
免疫学研究正在揭示MG的免疫病理学,并确定针对这种疾病的新型免疫疗法靶点。患有肌肉特异性酪氨酸激酶(MuSK)抗体的MG患者常出现提示神经或肌肉疾病的症状和临床发现。
早期诊断和治疗对这些疾病的预后有显著影响。在大多数情况下,MG或兰伯特 - 伊顿肌无力综合征的诊断可依据病史,并辅以针对性问题,以及旨在证明受累肌肉群存在可变无力的体格检查。适当的确诊检查几乎总能明确诊断。尽管针对MG的几种新型治疗方式正在研究中,但目前可用的疗法能使大多数患有这种疾病的患者在功能和生活质量方面有显著改善。了解免疫调节疗法的剂量、不良反应和成本对于有效管理MG和兰伯特 - 伊顿肌无力综合征患者至关重要。