Myasthenia gravis crisis.
Myasthenia gravis (MG) is an autoimmune disorder resulting from the production of antibodies against acetylcholine receptors leading to the destruction of the postsynaptic membrane at the neuromuscular junction. In the US there are about 18,000 people with MG. Myasthenia gravis crisis (MGC) is defined as any MG exacerbation necessitating mechanical ventilation. Most patients presenting with MGC have an identifiable risk factor. The diagnosis of MGC should be suspected in all patients with respiratory failure, particularly those with unclear etiology. Acute management of MGC requires supportive general and ventilatory therapy and institution of measures to improve the neuromuscular blockade. The latter includes plasma exchange or i.v. immunoglobulin, and removal of the offending trigger. The outcome of patients with MGC has improved significantly and the current mortality rate is about 4 to 8%.
Bershad
Eric M
EM
Division of Neurocritical Care, The Neurological Institute, University Hospitals Case Medical Center, Department of Neurology, Case Western Reserve University, Cleveland, Ohio, USA.
Feen
Eliahu S
ES
Suarez
Jose I
JI
D001784:Blood Gas Analysis; D002800:Cholinesterase Inhibitors / Q000627:therapeutic use; D003937:Diagnosis, Differential; D018450:Disease Progression; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D018482:Muscle, Skeletal / Q000276:immunology; D009157:Myasthenia Gravis / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D010951:Plasma Exchange; D011729:Pyridostigmine Bromide / Q000627:therapeutic use; D011950:Receptors, Cholinergic / Q000276:immunology; D012121:Respiration, Artificial; D012131:Respiratory Insufficiency / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D012307:Risk Factors
D002800:Cholinesterase Inhibitors; D016756:Immunoglobulins, Intravenous; D011950:Receptors, Cholinergic; D011729:Pyridostigmine Bromide