Myasthenia gravis

被引:568
作者
Gilhus, Nils Erik [1 ,2 ]
Tzartos, Socrates [3 ,4 ]
Evoli, Amelia [5 ,6 ]
Palaces, Jacqueline [7 ]
Burns, Ted M. [8 ]
Verschuuren, Jan J. G. M. [9 ]
机构
[1] Univ Bergen, Dept Clin Med, Bergen, Norway
[2] Haukeland Hosp, Dept Neurol, Bergen, Norway
[3] Hellenic Pasteur Inst, Dept Neurobiol, Athens, Greece
[4] Tzartos NeuroDiagnost, Athens, Greece
[5] IRCCS, Fdn Policlin A Gemelli, Ist Neurol, Rome, Italy
[6] Univ Cattolica Sacro Cuore, Rome, Italy
[7] Univ Oxford, Hosp Trust, Nuffield Dept Clin Neurosci, Oxford, England
[8] Univ Virginia, Dept Neurol, Charlottesville, VA USA
[9] Leiden Univ, Dept Neurol, Med Ctr, Leiden, Netherlands
关键词
QUALITY-OF-LIFE; NICOTINIC ACETYLCHOLINE-RECEPTOR; LATE-ONSET MYASTHENIA; CLINICAL CHARACTERISTICS; MYCOPHENOLATE-MOFETIL; PROTEIN; INTRAVENOUS IMMUNOGLOBULIN; IGG4; AUTOANTIBODIES; RANDOMIZED-TRIAL; STRUCTURAL BASIS;
D O I
10.1038/s41572-019-0079-y
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Myasthenia gravis (MG) is an autoimmune disease caused by antibodies against the acetylcholine receptor (AChR), muscle-specific kinase (MuSK) or other AChR-related proteins in the postsynaptic muscle membrane. Localized or general muscle weakness is the predominant symptom and is induced by the antibodies. Patients are grouped according to the presence of antibodies, symptoms, age at onset and thymus pathology. Diagnosis is straightforward in most patients with typical symptoms and a positive antibody test, although a detailed clinical and neurophysiological examination is important in antibody-negative patients. MG therapy should be ambitious and aim for clinical remission or only mild symptoms with near-normal function and quality of life. Treatment should be based on MG subgroup and includes symptomatic treatment using acetylcholinesterase inhibitors, thynnectonny and innnnunotherapy. Intravenous immunoglobulin and plasma exchange are fast-acting treatments used for disease exacerbations, and intensive care is necessary during exacerbations with respiratory failure. Comorbidity is frequent, particularly in elderly patients. Active physical training should be encouraged.
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页数:19
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