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Lambert–Eaton myasthenic syndrome (LEMS): a rare autoimmune presynaptic disorder often associated with cancer

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An Erratum to this article was published on 10 July 2017

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Abstract

Lambert–Eaton myasthenic syndrome (LEMS) is a rare autoimmune neuromuscular junction disorder that is related to the loss of functional P/Q-type voltage-gated calcium channels (VGCCs) on presynaptic nerve terminals. Up to 60% of cases occur as a paraneoplastic disorder (SCLC-LEMS), most commonly in association with small cell lung cancer. The remaining cases have an idiopathic non-tumor etiology but are associated with underlying autoimmune disease (NT-LEMS). Patients with LEMS invariably experience progressive proximal muscle weakness, often accompanied by general fatigue and autonomic symptoms. Some LEMS clinical symptoms overlap with those of other myasthenic syndromes, most commonly myasthenia gravis, which can contribute to misdiagnosis or delayed diagnosis. Prognosis is related to the presence of cancer or autoimmune disease and the severity/distribution of muscle weakness. Cause of death in patients with SCLC-LEMS is typically tumor progression, whereas NT-LEMS does not reduce life expectancy. LEMS diagnosis is supported by a threefold approach: clinical features, electromyography, and anti-VGCC antibody serology. LEMS is a clinically important early indicator of possible cancer; therefore, a LEMS diagnosis should immediately prompt rigorous oncological screening and surveillance. Symptomatic treatment of LEMS typically involves medications that improve neurotransmission (e.g., the potassium channel blocker amifampridine [3,4-diaminopyridine]), with addition of immunosuppressants/modulators (e.g., prednisone plus azathioprine) in individuals with persistent symptoms. Where a tumor is identified, oncological treatment should take priority. It should be remembered, however, that LEMS has a significant impact on a patient’s quality of life and ability to perform daily activities, and therefore warrants timely diagnosis and appropriate treatment in and of itself.

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  • 10 July 2017

    An erratum to this article has been published.

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Acknowledgements

Funding for the manuscript was provided by BioMarin Europe Ltd and editorial support was provided by Virgo Health.

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Authors and Affiliations

  1. Friedrich-Baur-Institute, Department of Neurology Klinikum München Ludwig-Maximilians-University Munich, Munich, Germany

    Benedikt Schoser

  2. Institute de Myologie, 47 Boulevard de l’Hôpital, 75013, Paris, France

    Bruno Eymard

  3. BioMarin Europe Ltd, 10 Bloomsbury Way, London, WC1A 2SL, UK

    Joe Datt

  4. Fondazione IRCCS Istituto Neurologico, Via Giovanni Celoria, 11, 20133, Milan, Italy

    Renato Mantegazza

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BS designed and initiated this review, selected the incorporated papers, supervised the writing with input from all coauthors and approved the final version of this manuscript. BE, JD and RM contributed to the concept of this review, provided feedback at all stages of development and approved the final version of this manuscript.

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Correspondence to Benedikt Schoser.

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Conflicts of interest

BS has received speaker honoraria from BioMarin, Sanofi Genzyme, CSL Behring, and Amicus Therapeutics. He is a member of the neuromuscular advisory board of Audentes Therapeutics and has received an unrestricted research grant from Sanofi Genzyme. BE has received honoraria from BioMarin Europe Ltd, Novartis and the LFB Group. JD is a EUMEA Medical Affairs Senior Director for BioMarin Europe Ltd. RM has received honoraria for participation in clinical trials, advisory boards, congresses and travel from Alexion Pharmaceuticals, Biomarin Europe Ltd. and Catalyst.

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An erratum to this article is available at https://doi.org/10.1007/s00415-017-8556-2.

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Schoser, B., Eymard, B., Datt, J. et al. Lambert–Eaton myasthenic syndrome (LEMS): a rare autoimmune presynaptic disorder often associated with cancer. J Neurol 264, 1854–1863 (2017). https://doi.org/10.1007/s00415-017-8541-9

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