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.

• Authors: Nils Erik Gilhus, Socrates Tzartos, Amelia Evoli, Jacqueline Palace, Ted M Burns, Jan J G M Verschuuren
• Journal: Nature reviews. Disease primers (Nat Rev Dis Primers) Vol. 5 Issue 1 Pg. 30 (05 02 2019) ISSN: 2056-676X [Electronic] England
• PMID: 31048702 (Publication Type: Journal Article, Review)

Chemical References

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

Topics

• Acetylcholinesterase (genetics, physiology)
• Adrenal Cortex Hormones (therapeutic use)
• Agrin (genetics, physiology)
• Anti-Inflammatory Agents, Non-Steroidal (therapeutic use)
• Autoantibodies (analysis, blood)
• Biomarkers (analysis, blood)
• Blepharoptosis (etiology)
• Collagen (genetics, physiology)
• Cortactin (genetics, physiology)
• Electromyography (methods)
• Humans
• Kv1.4 Potassium Channel (genetics, physiology)
• LDL-Receptor Related Proteins (genetics, physiology)
• Muscle Proteins (genetics, physiology)
• Myasthenia Gravis (diagnosis, physiopathology, therapy)
• Receptor Protein-Tyrosine Kinases (genetics, physiology)
• Receptors, Cholinergic (genetics, physiology)
• Receptors, Nicotinic (genetics)
• Risk Factors
• Ryanodine Receptor Calcium Release Channel (genetics, physiology)