Myasthenia gravis.


Journal

Nature reviews. Disease primers
ISSN: 2056-676X
Titre abrégé: Nat Rev Dis Primers
Pays: England
ID NLM: 101672103

Informations de publication

Date de publication:
02 05 2019
Historique:
entrez: 4 5 2019
pubmed: 3 5 2019
medline: 17 9 2019
Statut: epublish

Résumé

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.

Identifiants

pubmed: 31048702
doi: 10.1038/s41572-019-0079-y
pii: 10.1038/s41572-019-0079-y
doi:

Substances chimiques

Adrenal Cortex Hormones 0
Agrin 0
Anti-Inflammatory Agents, Non-Steroidal 0
Autoantibodies 0
Biomarkers 0
Cortactin 0
KCNA4 protein, human 0
Kv1.4 Potassium Channel 0
LDL-Receptor Related Proteins 0
LRP4 protein, human 0
Muscle Proteins 0
Receptors, Cholinergic 0
Receptors, Nicotinic 0
Ryanodine Receptor Calcium Release Channel 0
Collagen 9007-34-5
MUSK protein, human EC 2.7.10.1
Receptor Protein-Tyrosine Kinases EC 2.7.10.1
Acetylcholinesterase EC 3.1.1.7
COLQ protein, human EC 3.1.1.7

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

30

Auteurs

Nils Erik Gilhus (NE)

Department of Clinical Medicine, University of Bergen, Bergen, Norway. nils.gilhus@uib.no.
Department of Neurology, Haukeland University Hospital, Bergen, Norway. nils.gilhus@uib.no.

Socrates Tzartos (S)

Department of Neurobiology, Hellenic Pasteur Institute and Tzartos NeuroDiagnostics, Athens, Greece.

Amelia Evoli (A)

Istituto di Neurologia, Fondazione Policlinico A. Gemelli, IRCCS, Rome, Italy.
Università Cattolica del Sacro Cuore, Rome, Italy.

Jacqueline Palace (J)

Nuffield Department of Clinical Neurosciences, University of Oxford, Hospitals Trust, Oxford, UK.

Ted M Burns (TM)

Department of Neurology, University of Virginia, Charlottesville, VA, USA.

Jan J G M Verschuuren (JJGM)

Department of Neurology, Leiden University Medical Centre, Leiden, Netherlands.

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Classifications MeSH