PMID- 31048702
OWN - NLM
STAT- MEDLINE
DCOM- 20190916
LR  - 20220409
IS  - 2056-676X (Electronic)
IS  - 2056-676X (Linking)
VI  - 5
IP  - 1
DP  - 2019 May 2
TI  - Myasthenia gravis.
PG  - 30
LID - 10.1038/s41572-019-0079-y [doi]
AB  - 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.
FAU - Gilhus, Nils Erik
AU  - Gilhus NE
AD  - Department of Clinical Medicine, University of Bergen, Bergen, Norway. 
      nils.gilhus@uib.no.
AD  - Department of Neurology, Haukeland University Hospital, Bergen, Norway. 
      nils.gilhus@uib.no.
FAU - Tzartos, Socrates
AU  - Tzartos S
AD  - Department of Neurobiology, Hellenic Pasteur Institute and Tzartos 
      NeuroDiagnostics, Athens, Greece.
FAU - Evoli, Amelia
AU  - Evoli A
AD  - Istituto di Neurologia, Fondazione Policlinico A. Gemelli, IRCCS, Rome, Italy.
AD  - Universita Cattolica del Sacro Cuore, Rome, Italy.
FAU - Palace, Jacqueline
AU  - Palace J
AD  - Nuffield Department of Clinical Neurosciences, University of Oxford, Hospitals 
      Trust, Oxford, UK.
FAU - Burns, Ted M
AU  - Burns TM
AD  - Department of Neurology, University of Virginia, Charlottesville, VA, USA.
FAU - Verschuuren, Jan J G M
AU  - Verschuuren JJGM
AD  - Department of Neurology, Leiden University Medical Centre, Leiden, Netherlands.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20190502
PL  - England
TA  - Nat Rev Dis Primers
JT  - Nature reviews. Disease primers
JID - 101672103
RN  - 0 (Adrenal Cortex Hormones)
RN  - 0 (Agrin)
RN  - 0 (Anti-Inflammatory Agents, Non-Steroidal)
RN  - 0 (Autoantibodies)
RN  - 0 (Biomarkers)
RN  - 0 (Cortactin)
RN  - 0 (KCNA4 protein, human)
RN  - 0 (Kv1.4 Potassium Channel)
RN  - 0 (LDL-Receptor Related Proteins)
RN  - 0 (LRP4 protein, human)
RN  - 0 (Muscle Proteins)
RN  - 0 (Receptors, Cholinergic)
RN  - 0 (Receptors, Nicotinic)
RN  - 0 (Ryanodine Receptor Calcium Release Channel)
RN  - 9007-34-5 (Collagen)
RN  - EC 2.7.10.1 (MUSK protein, human)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
RN  - EC 3.1.1.7 (Acetylcholinesterase)
RN  - EC 3.1.1.7 (COLQ protein, human)
SB  - IM
MH  - Acetylcholinesterase/genetics/physiology
MH  - Adrenal Cortex Hormones/therapeutic use
MH  - Agrin/genetics/physiology
MH  - Anti-Inflammatory Agents, Non-Steroidal/therapeutic use
MH  - Autoantibodies/analysis/blood
MH  - Biomarkers/analysis/blood
MH  - Blepharoptosis/etiology
MH  - Collagen/genetics/physiology
MH  - Cortactin/genetics/physiology
MH  - Electromyography/methods
MH  - Humans
MH  - Kv1.4 Potassium Channel/genetics/physiology
MH  - LDL-Receptor Related Proteins/genetics/physiology
MH  - Muscle Proteins/genetics/physiology
MH  - Myasthenia Gravis/*diagnosis/physiopathology/*therapy
MH  - Receptor Protein-Tyrosine Kinases/genetics/physiology
MH  - Receptors, Cholinergic/genetics/physiology
MH  - Receptors, Nicotinic/genetics
MH  - Risk Factors
MH  - Ryanodine Receptor Calcium Release Channel/genetics/physiology
EDAT- 2019/05/03 06:00
MHDA- 2019/09/17 06:00
CRDT- 2019/05/04 06:00
PHST- 2019/05/04 06:00 [entrez]
PHST- 2019/05/03 06:00 [pubmed]
PHST- 2019/09/17 06:00 [medline]
AID - 10.1038/s41572-019-0079-y [pii]
AID - 10.1038/s41572-019-0079-y [doi]
PST - epublish
SO  - Nat Rev Dis Primers. 2019 May 2;5(1):30. doi: 10.1038/s41572-019-0079-y.