Myasthenia gravis: a clinical-immunological update

被引:97
作者
Binks, Sophie [1 ]
Vincent, Angela [1 ]
Palace, Jacqueline [1 ]
机构
[1] Univ Oxford, John Radcliffe Hosp, Nuffield Dept Clin Neurosci, Oxford OX3 9DU, England
关键词
Myasthenia gravis; MuSK; LRP4; IgG4; Cell-based assays; Neuromuscular junction; Thymectomy; TYROSINE KINASE MUSK; CLUSTERED ACETYLCHOLINE-RECEPTOR; NEUROMUSCULAR-JUNCTION; INCREASING INCIDENCE; ROBOTIC THYMECTOMY; THYMUS CHANGES; DOUBLE-BLIND; PROTEIN; ANTIBODY; AUTOANTIBODIES;
D O I
10.1007/s00415-015-7963-5
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Myasthenia gravis (MG) is the archetypic disorder of both the neuromuscular junction and autoantibody-mediated disease. In most patients, IgG1-dominant antibodies to acetylcholine receptors cause fatigable weakness of skeletal muscles. In the rest, a variable proportion possesses antibodies to muscle-specific tyrosine kinase while the remainder of seronegative MG is being explained through cell-based assays using a receptor-clustering technique and, to a lesser extent, proposed new antigenic targets. The incidence and prevalence of MG are increasing, particularly in the elderly. New treatments are being developed, and results from the randomised controlled trial of thymectomy in non-thymomatous MG, due for release in early 2016, will be of particular clinical value. To help navigate an evidence base of varying quality, practising clinicians may consult new MG guidelines in the fields of pregnancy, ocular and generalised MG (GMG). This review focuses on updates in epidemiology, immunology, therapeutic and clinical aspects of GMG in adults.
引用
收藏
页码:826 / 834
页数:9
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