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Myasthenia gravis

delete2019-05-02
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PRE
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N
Nils Erik Gilhus *
S
Socrates J. Tzartos
A
Amelia Evoli
J
Jacqueline Palace
T
Ted M. Burns
J
Jan J.G.M. Verschuuren
DOI:10.1038/s41572-019-0079-ydelete
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摘要

摘要

En 中文
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.
Keyword:
QUALITY-OF-LIFE
NICOTINIC ACETYLCHOLINE-RECEPTOR
LATE-ONSET MYASTHENIA
CLINICAL CHARACTERISTICS
MYCOPHENOLATE-MOFETIL
PROTEIN 4
INTRAVENOUS IMMUNOGLOBULIN
IGG4 AUTOANTIBODIES
RANDOMIZED-TRIAL
STRUCTURAL BASIS
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期刊

N
Nature Reviews Disease Primers
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60.6
论文数:
650
被引数:
3.8W

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Haukeland University Hospital
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university of bergen
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irccs policlinico gemelli
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University of Virginia
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university of oxford
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9.8W
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