Myasthenia Gravis

Research output: Contribution to journalArticle

Abstract

The basic abnormality in myasthenia gravis (MG) is a reduction in acetylcholine receptors (AChRs) at neuromuscular junctions due to the effects of autoantibodies that are directed against the AChRs in most patients, or against neighboring proteins involved in the clustering of AChRs (MuSK, LRP-4, or agrin). Clinically, MG is characterized by muscle weakness and fatigue, often in typical patterns. The diagnosis may be missed early, and depends on the recognition of clinical manifestations, the measurement of autoantibodies, and/or electrophysiological features. The treatment of MG involves the enhancement of neuromuscular transmission by anticholinesterase drugs (pyridostigmine), and by immunotherapy. Therapy should be designed to improve the clinical features quickly, and keep the symptoms in abeyance over the long term. Rapid improvement can be achieved when necessary by the administration of intravenous immunoglobulin or plasma exchange. Intermediate rates of improvement over months involve the use of adrenal corticosteroids, the calcineurin inhibitors cyclosporine or tacrolimus, and in some patients, the B-cell inhibitor rituximab. For long-term treatment, mycophenolate and azathioprine are the most effective agents. A thymectomy may also have long-term beneficial effects. The majority of MG patients can live normal lives, but most patients require lifelong treatment. The physician's skill in managing the immunotherapeutic agents and avoiding adverse side effects is of paramount importance in the treatment of MG.

Original languageEnglish (US)
Pages (from-to)419-424
Number of pages6
JournalSeminars in Neurology
Volume36
Issue number5
DOIs
StatePublished - Oct 1 2016

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Myasthenia Gravis
Cholinergic Receptors
Autoantibodies
Agrin
Pyridostigmine Bromide
Therapeutics
Thymectomy
Muscle Fatigue
Plasma Exchange
Neuromuscular Junction
Intravenous Immunoglobulins
Cholinesterase Inhibitors
Muscle Weakness
Azathioprine
Tacrolimus
Immunotherapy
Cyclosporine
Cluster Analysis
Adrenal Cortex Hormones
B-Lymphocytes

Keywords

  • acetylcholine receptors
  • antibodies
  • autoimmunity
  • immunotherapy
  • myasthenia gravis

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Myasthenia Gravis. / Drachman, Daniel B.

In: Seminars in Neurology, Vol. 36, No. 5, 01.10.2016, p. 419-424.

Research output: Contribution to journalArticle

Drachman, Daniel B. / Myasthenia Gravis. In: Seminars in Neurology. 2016 ; Vol. 36, No. 5. pp. 419-424.
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