Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases

Lambert-Eaton myasthenic syndrome (LEMS) is an immune-mediated disorder of the presynaptic neuromuscular transmission, which more frequently occurs as the remote effect of a neoplasm, in the paraneoplastic form (P-LEMS), or in a non-paraneoplastic form (NP-LEMS); but few studies describe the clinical features of NP-LEMS. We analyzed the clinical manifestations, laboratory findings, electrophysiological studies, and treatment responses in ten Brazilian patients suffering from NP-LEMS. The mean age was 41.5 years. More often neurological findings were hyporeflexia or areflexia with a post-exercise improvement. Treatment response occurred with pyridostigmine, guanidine, prednisone, azathioprine, and cyclosporine; but not response was observed after intravenous immunoglobulin and plasma exchange. Age at onset, clinical manifestations, and electrophysiological abnormalities can help more in the diagnosis than serum antibodies; the symptomatic treatment with pyridostigmine was effective; and the immunosuppressive treatment with prednisone, azathioprine, or cyclosporine was more beneficial than plasma exchange or intravenous immunoglobulin treatment.

Bibliographic Details

Main Authors:	Lorenzoni,Paulo J., Scola,Rosana H., Kay,Cláudia S. Kamoi, Parolin,Sérgio F., Werneck,Lineu C.
Format:	Digital revista
Language:	English
Published:	Academia Brasileira de Neurologia - ABNEURO 2010
Online Access:	http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2010000600004
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