PMID- 34064035
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20210615
IS  - 2077-0383 (Print)
IS  - 2077-0383 (Electronic)
IS  - 2077-0383 (Linking)
VI  - 10
IP  - 11
DP  - 2021 May 21
TI  - Myasthenia Gravis: Epidemiology, Pathophysiology and Clinical Manifestations.
LID - 10.3390/jcm10112235 [doi]
LID - 2235
AB  - Myasthenia gravis (MG) is an autoimmune neurological disorder characterized by 
      defective transmission at the neuromuscular junction. The incidence of the 
      disease is 4.1 to 30 cases per million person-years, and the prevalence rate 
      ranges from 150 to 200 cases per million. MG is considered a classic example of 
      antibody-mediated autoimmune disease. Most patients with MG have autoantibodies 
      against the acetylcholine receptors (AChRs). Less commonly identified 
      autoantibodies include those targeted to muscle-specific kinase (MuSK), 
      low-density lipoprotein receptor-related protein 4 (Lrp4), and agrin. These 
      autoantibodies disrupt cholinergic transmission between nerve terminals and 
      muscle fibers by causing downregulation, destruction, functional blocking of 
      AChRs, or disrupting the clustering of AChRs in the postsynaptic membrane. The 
      core clinical manifestation of MG is fatigable muscle weakness, which may affect 
      ocular, bulbar, respiratory and limb muscles. Clinical manifestations vary 
      according to the type of autoantibody, and whether a thymoma is present.
FAU - Dresser, Laura
AU  - Dresser L
AUID- ORCID: 0000-0002-6347-0873
AD  - Department of Neurology, University of Chicago, Chicago, IL 60637, USA.
FAU - Wlodarski, Richard
AU  - Wlodarski R
AD  - Department of Neurology, University of Chicago, Chicago, IL 60637, USA.
FAU - Rezania, Kourosh
AU  - Rezania K
AUID- ORCID: 0000-0003-3986-5709
AD  - Department of Neurology, University of Chicago, Chicago, IL 60637, USA.
FAU - Soliven, Betty
AU  - Soliven B
AD  - Department of Neurology, University of Chicago, Chicago, IL 60637, USA.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20210521
PL  - Switzerland
TA  - J Clin Med
JT  - Journal of clinical medicine
JID - 101606588
PMC - PMC8196750
OTO - NOTNLM
OT  - B cells
OT  - T cells
OT  - acetylcholine receptor
OT  - autoantibodies
OT  - cytokines
OT  - myasthenia gravis
COIS- K.R. has received honoraria for consultations and serving on advisory boards for 
      Alexion, Kabafusion and Grifols. B.S. and K.R. have received funding from Alexion 
      for conducting clinical trials on MG and ALS. L.D. and R.W. declare no conflict 
      of interest.
EDAT- 2021/06/03 06:00
MHDA- 2021/06/03 06:01
PMCR- 2021/05/21
CRDT- 2021/06/02 01:08
PHST- 2021/04/27 00:00 [received]
PHST- 2021/05/17 00:00 [revised]
PHST- 2021/05/17 00:00 [accepted]
PHST- 2021/06/02 01:08 [entrez]
PHST- 2021/06/03 06:00 [pubmed]
PHST- 2021/06/03 06:01 [medline]
PHST- 2021/05/21 00:00 [pmc-release]
AID - jcm10112235 [pii]
AID - jcm-10-02235 [pii]
AID - 10.3390/jcm10112235 [doi]
PST - epublish
SO  - J Clin Med. 2021 May 21;10(11):2235. doi: 10.3390/jcm10112235.