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

Abstract:

: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.

journal_name

Arq Neuropsiquiatr

authors

Lorenzoni PJ,Scola RH,Kay CS,Parolin SF,Werneck LC

doi

10.1590/s0004-282x2010000600004

subject

Has Abstract

pub_date

2010-12-01 00:00:00

pages

849-54

issue

6

eissn

0004-282X

issn

1678-4227

pii

S0004-282X2010000600004

journal_volume

68

pub_type

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