[Myasthenia gravis].
Myasthenia gravis is a rare, auto-immune neuromuscular junction disorder. Prevalence rates is about 50/1,000000. The disease results from circulating auto-antibody attacks against post-synaptic targets (acetylcholine receptor [AChR] in 80% cases) on the endplate region of the postsynaptic membrane. The diagnosis is supported clinically by transient weakness, increased by activity that can affect eye movements, swallowing, speech, upper and lower limbs, and trunk. There are generalized or focalized forms (as ocular myasthenia). The course is variable and evolved either with attacks or more chronically. Helpful tests for diagnosis are serologic antibodies detection against AChR, decrement of muscle action potential after repetitive nerve stimulations, identification of thymus gland abnormality (frequently associated with myasthenia) by chest computed tomography. Myasthenia gravis treatment is based on oral form of cholinesterase inhibitors, corticosteroids and other immunosuppressive drugs in severe forms. During myasthenia crisis, intraveinous immune globulines or plasma exchanges can be used. Thymectomy is proposed in case of thymus abnormality.
Fleury
Marie-Céline
MC
Service de neurologie, Hôpitaux universitaires, 67091 Strasbourg Cedex, France.
Tranchant
Christine
C
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use; D000328:Adult; D000911:Antibodies, Monoclonal / Q000008:administration & dosage / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001379:Azathioprine / Q000008:administration & dosage / Q000627:therapeutic use; D002648:Child; D002800:Cholinesterase Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use; D007231:Infant, Newborn; D015624:Lambert-Eaton Myasthenic Syndrome / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000601:surgery / Q000628:therapy; D010951:Plasma Exchange; D011729:Pyridostigmine Bromide / Q000008:administration & dosage / Q000627:therapeutic use; D013902:Radiography, Thoracic; D000069283:Rituximab; D013934:Thymectomy; D013952:Thymus Hyperplasia / Q000175:diagnosis / Q000601:surgery; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D002800:Cholinesterase Inhibitors; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D000069283:Rituximab; D011729:Pyridostigmine Bromide; D001379:Azathioprine