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

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.

• Authors: E Boutin, C Rey, M Romeu, J Pouget, J Franques
• Journal: La Revue de medecine interne (Rev Med Interne) Vol. 34 Issue 8 Pg. 493-6 (Aug 2013) ISSN: 1768-3122 [Electronic] France
• Vernacular Title: Efficacité du rituximab dans un cas de syndrome de Lambert-Eaton non paranéoplasique séronégatif.
• PMID: 23759215 (Publication Type: Case Reports, English Abstract, Journal Article)
• Copyright: Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Chemical References

• Antibodies, Monoclonal, Murine-Derived
• Immunosuppressive Agents
• Rituximab

Topics

• Adult
• Antibodies, Monoclonal, Murine-Derived (therapeutic use)
• Humans
• Immunosuppressive Agents (therapeutic use)
• Lambert-Eaton Myasthenic Syndrome (drug therapy)
• Male
• Rituximab
• Treatment Outcome