title: Lambert-Eaton myasthenic syndrome presenting with severe respiratory failure.
abstract: Two cases of Lambert-Eaton myasthenic syndrome (LEMS) who presented with primary respiratory failure are reported. In each case, although not initially suspected clinically, the electrophysiological findings, which included reduced compound muscle action potential amplitudes, decrement to 3-Hz stimulation, and potentiation after 40-Hz stimulation, led to the diagnosis in the critical care unit. Electrophysiological studies of the respiratory system, including repetitive nerve stimulation of the phrenic nerve, were extremely valuable in management. As shown by these cases, the severe respiratory failure in LEMS is reversible with treatment. Thus, LEMS should be considered in cases of unexplained respiratory failure, other clinical features of the disorder sought, and the electrophysiological hallmarks looked for including studies of the respiratory system.
authors.affiliation: Department of Clinical Neurological Sciences, Victoria Hospital, University of Western Ontario, London, Canada.
authors.forename: M W
authors.initials: MW
authors.lastname: Nicolle
authors.forename: D J
authors.initials: DJ
authors.lastname: Stewart
authors.forename: H
authors.initials: H
authors.lastname: Remtulla
authors.forename: R
authors.initials: R
authors.lastname: Chen
authors.forename: C F
authors.initials: CF
authors.lastname: Bolton
mesh_terms: D000200:Action Potentials; D000368:Aged; D003422:Critical Care; D004558:Electric Stimulation; D004568:Electrodiagnosis; D005260:Female; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D008297:Male; D009132:Muscles / Q000503:physiopathology; D010791:Phrenic Nerve / Q000503:physiopathology; D012131:Respiratory Insufficiency / Q000209:etiology* / Q000628:therapy