PMID- 38494283
OWN - NLM
STAT- MEDLINE
DCOM- 20240319
LR  - 20240327
IS  - 0072-9752 (Print)
IS  - 0072-9752 (Linking)
VI  - 200
DP  - 2024
TI  - Myasthenia gravis-Pathophysiology, diagnosis, and treatment.
PG  - 283-305
LID - B978-0-12-823912-4.00026-8 [pii]
LID - 10.1016/B978-0-12-823912-4.00026-8 [doi]
AB  - Myasthenia gravis (MG) is an autoimmune disease characterized by dysfunction of 
      the neuromuscular junction resulting in skeletal muscle weakness. It is equally 
      prevalent in males and females, but debuts at a younger age in females and at an 
      older age in males. Ptosis, diplopia, facial bulbar weakness, and limb weakness 
      are the most common symptoms. MG can be classified based on the presence of serum 
      autoantibodies. Acetylcholine receptor (AChR) antibodies are found in 80%-85% of 
      patients, muscle-specific kinase (MuSK) antibodies in 5%-8%, and <1% may have 
      low-density lipoprotein receptor-related protein 4 (Lrp4) antibodies. 
      Approximately 10% of patients are seronegative for antibodies binding the known 
      disease-related antigens. In patients with AChR MG, 10%-20% have a thymoma, which 
      is usually detected at the onset of the disease. Important differences between 
      clinical presentation, treatment responsiveness, and disease mechanisms have been 
      observed between these different serologic MG classes. Besides the typical 
      clinical features and serologic testing, the diagnosis can be established with 
      additional tests, including repetitive nerve stimulation, single fiber EMG, and 
      the ice pack test. Treatment options for MG consist of symptomatic treatment 
      (such as pyridostigmine), immunosuppressive treatment, or thymectomy. Despite the 
      treatment with symptomatic drugs, steroid-sparing immunosuppressants, intravenous 
      immunoglobulins, plasmapheresis, and thymectomy, a large proportion of patients 
      remain chronically dependent on corticosteroids (CS). In the past decade, the 
      number of treatment options for MG has considerably increased. Advances in the 
      understanding of the pathophysiology have led to new treatment options targeting 
      B or T cells, the complement cascade, the neonatal Fc receptor or cytokines. In 
      the future, these new treatments are likely to reduce the chronic use of CS, 
      diminish side effects, and decrease the number of patients with refractory 
      disease.
CI  - Copyright (c) 2024 Elsevier B.V. All rights are reserved, including those for text 
      and data mining, AI training, and similar technologies.
FAU - Tannemaat, Martijn R
AU  - Tannemaat MR
AD  - Department of Neurology, Leiden University Medical Center, Leiden, The 
      Netherlands.
FAU - Huijbers, Maartje G
AU  - Huijbers MG
AD  - Department of Neurology, Leiden University Medical Center, Leiden, The 
      Netherlands; Department of Human Genetics, Leiden University Medical Center, 
      Leiden, The Netherlands.
FAU - Verschuuren, Jan J G M
AU  - Verschuuren JJGM
AD  - Department of Neurology, Leiden University Medical Center, Leiden, The 
      Netherlands. Electronic address: j.j.g.m.verschuuren@lumc.nl.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - Netherlands
TA  - Handb Clin Neurol
JT  - Handbook of clinical neurology
JID - 0166161
RN  - 0 (Autoantibodies)
RN  - 0 (Immunosuppressive Agents)
SB  - IM
MH  - Female
MH  - Humans
MH  - Male
MH  - Autoantibodies
MH  - Electromyography
MH  - Immunosuppressive Agents
MH  - *Myasthenia Gravis/diagnosis/therapy
MH  - Neuromuscular Junction/metabolism
OTO - NOTNLM
OT  - AChR
OT  - Diagnosis
OT  - Lrp4
OT  - MuSK
OT  - Myasthenia gravis
OT  - Neuroimmunology
OT  - Neuromuscular disorders
OT  - Neuromuscular junction
OT  - Pathophysiology
OT  - Treatment
EDAT- 2024/03/18 00:42
MHDA- 2024/03/19 06:43
CRDT- 2024/03/17 21:57
PHST- 2024/03/19 06:43 [medline]
PHST- 2024/03/18 00:42 [pubmed]
PHST- 2024/03/17 21:57 [entrez]
AID - B978-0-12-823912-4.00026-8 [pii]
AID - 10.1016/B978-0-12-823912-4.00026-8 [doi]
PST - ppublish
SO  - Handb Clin Neurol. 2024;200:283-305. doi: 10.1016/B978-0-12-823912-4.00026-8.