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Nature Reviews Disease Primers
Article . 2019 . Peer-reviewed
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Myasthenia gravis

Authors: Gilhus, Nils Erik; Tzartos, Socrates; Evoli, Amelia; Palace, Jacqueline; Burns, Ted M; Verschuuren, Jan J G M;

Myasthenia gravis

Abstract

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, thymectomy and immunotherapy. 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.

Keywords

Electromyography, Anti-Inflammatory Agents, Non-Steroidal, Muscle Proteins, Receptor Protein-Tyrosine Kinases, Ryanodine Receptor Calcium Release Channel, Receptors, Nicotinic, Adrenal Cortex Hormones, Risk Factors, Acetylcholinesterase; Adrenal Cortex Hormones; Agrin; Anti-Inflammatory Agents, Non-Steroidal; Autoantibodies; Biomarkers; Blepharoptosis; Collagen; Cortactin; Electromyography; Humans; Kv1.4 Potassium Channel; LDL-Receptor Related Proteins; Muscle Proteins; Myasthenia Gravis; Receptor Protein-Tyrosine Kinases; Receptors, Cholinergic; Receptors, Nicotinic; Risk Factors; Ryanodine Receptor Calcium Release Channel, Myasthenia Gravis, Acetylcholinesterase, Blepharoptosis, Humans, Kv1.4 Potassium Channel, Receptors, Cholinergic, Agrin, Collagen, Cortactin, Biomarkers, LDL-Receptor Related Proteins, Autoantibodies

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    citations
    This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
    651
    popularity
    This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
    Top 0.1%
    influence
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    Top 1%
    impulse
    This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
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citations
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
651
Top 0.1%
Top 1%
Top 0.1%
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