Open AccessLambert-Eaton Myasthenic Syndrome in Brazil: a single center experience
Author(s) -
Icaro França Navarro Pinto,
Wladimir Bocca Vieira de Rezende Pinto,
Igor Braga Farias,
Bruno de Mattos Lombardi Badia,
Gustavo Carvalho Costa,
Carolina Maria Marin,
Ana Carolina Souza Jorge,
Emília Correa Souto,
Paulo de Lima Serrano,
Roberta Ismael Lacerda Machado,
Márcio Luiz Escórcio Bezerra,
Acary Souza Bullé Oliveira,
Paulo Victor Sgobbi de Souza
Publication year - 2021
Language(s) - English
Resource type - Conference proceedings
DOI - 10.5327/1516-3180.120
Subject(s) - lambert eaton myasthenic syndrome , medicine , muscle weakness , orthostatic vital signs , weakness , myasthenia gravis , cohort , autoantibody , cardiology , surgery , immunology , blood pressure , antibody
Lambert-Eaton Myasthenic Syndrome (LEMS) is an ultrarare autoimmune disorder of neuromuscular junction characterized by proximal muscle weakness, arreflexia and autonomic dysfunction due to presynaptic dysfunction caused by autoantibodies against the P/Q-type voltagegated calcium channel with diminished release of acetylcholine. LEMS can occurs as a primary autoimmune disorder or as paraneoplastic disorder with more than half of LEMS cases associated with small cell lung cancer. Objectives: The main objective of this study is described clinical, epidemiological, serological, and neurophysiological findings of a Brazilian cohort with definitive diagnosis of Lambert-Eaton Myasthenic Syndrome (LEMS). Results We identified eight patients with definitive LEMS with a 2:1 male/ female prevalence, all present with proximal muscle weakness with lower limb predominance and the most common autonomic dysfunction were xeropthalmia in 100% of patients, orthostatic hypotension presented in 6 of 9 patients and erectile dysfunction in all male patients. Conclusions: LEMS should always be suspected in patients with proximal muscle weakness associated with autonomic dysfunction and in this Brazilian cohort most cases were seronegative and do not have correlation with small-cell lung cancer in contrast with the current knowledge of disease.