TY - JOUR
T1 - Myasthenia Gravis
AU - Drachman, Daniel B.
N1 - Publisher Copyright:
© Thieme Medical Publishers333 Seventh Avenue, New York, NY 10001, USA.
PY - 2016/10/1
Y1 - 2016/10/1
N2 - 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.
AB - 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.
KW - acetylcholine receptors
KW - antibodies
KW - autoimmunity
KW - immunotherapy
KW - myasthenia gravis
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U2 - 10.1055/s-0036-1586265
DO - 10.1055/s-0036-1586265
M3 - Article
C2 - 27704496
AN - SCOPUS:84990840610
SN - 0271-8235
VL - 36
SP - 419
EP - 424
JO - Seminars in neurology
JF - Seminars in neurology
IS - 5
ER -