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Rev Med Interne. 2013 Aug;34(8):493-6. doi: 10.1016/j.revmed.2013.04.007. Epub 2013 Jun 4.

[Favourable outcome after treatment with rituximab in a case of seronegative non-paraneoplastic Lambert-Eaton myasthenic syndrome].

[Article in French]

Author information

  • 1Centre de référence des maladies neuromusculaires et de la SLA, centre hospitalier universitaire de La Timone, 264 rue Saint-Pierre, Marseille cedex 05, France. emmanuelle.boutin@ap-hm.fr

Abstract

INTRODUCTION:

Lambert-Eaton myasthenic syndrome is a rare and autoimmune presynaptic disorder of the neuromuscular junction, due in 85% of cases to autoantibodies directed against voltage-gated calcium channels. It is a paraneoplastic disorder in 50 to 60% of cases. Diagnosis involves a proximal muscle weakness and areflexia, associated with a significant increment after post-exercise stimulation in electrophysiological study. Symptomatic treatment is based on 3,4-diaminopyridine. No etiological treatment has proven its efficacy in both paraneoplastic and non-paraneoplastic Lambert-Eaton myasthenic syndrome.

CASE REPORT:

We report a 41-year-old man who presented with a seronegative non-paraneoplastic Lambert-Eaton myasthenic syndrome in whom conventional immunosuppressive treatments (corticosteroids, azathioprine) failed, and who eventually improved after treatment with rituximab.

CONCLUSION:

Rituximab was an effective and well-tolerated treatment in this case of seronegative non-paraneoplastic Lambert-Eaton myasthenic syndrome. Its indication should be discussed when conventional immunosuppressive therapy fails in both seropositive and seronegative patients.

Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

KEYWORDS:

Lambert-Eaton myasthenic syndrome; Rituximab; Syndrome de Lambert-Eaton

PMID:
23759215
[PubMed - indexed for MEDLINE]
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