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Italian recommendations for Lambert–Eaton myasthenic syndrome (LEMS) management

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Abstract

Lambert–Eaton myasthenic syndrome (LEMS) is a pre-synaptic disorder of the neuromuscular and autonomic transmission mediated by antibodies to voltage-gated calcium channels at the motor nerve terminal. LEMS is a quite rare and probably under-diagnosed disease: the onset may be slow and clinical signs are typically fluctuating, thus adding to the delay in diagnosis. LEMS weakness typically involves lower and upper limbs and the proximal muscles are predominantly affected. A significant proportion of patients also have dysfunction of the autonomic nervous system that may include dry mouth, constipation, blurred vision, impaired sweating, and orthostatic hypotension. LEMS recognition is based on clinical, electrophysiological and immunological criteria. Nearly 50–60 % of patients with LEMS have an underlying tumour that, in almost all cases, is a small-cell lung cancer; the onset of neurological symptoms generally precedes tumour detection. A careful screening for the early detection of the possible associated cancer is a crucial step for optimal disease management. The Italian Working Group on Myasthenic Syndromes developed diagnostic and therapeutic algorithms that could serve in routine clinical practice as tools for a patient-tailored approach.

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Acknowledgments

There were no funding sources for this work. The authors have no conflict of interest to disclose.

Author information

Authors and Affiliations

  1. Istituto di Neurologia, Università Cattolica, Roma, Italy

    A. Evoli

  2. IRCCS Istituto delle Scienze Neurologiche di Bologna e Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Bologna, Italy

    R. Liguori

  3. IRCCS Istituto Neurologico Nazionale ‘C.Mondino’, Pavia, Italy

    A. Romani

  4. Divisione Neuroimmunologia e Malattie Neuromuscolari, Fondazione IRCCS, Istituto Neurologico Carlo Besta, Via Celoria 11, 20133, Milan, Italy

    R. Mantegazza

  5. Centro di Riferimento Regionale Malattie Neuromuscolari, Ospedale Clinicizzato “SS Annunziata”, Chieti, Italy

    A. Di Muzio

  6. UOC di Neurologia, Ospedale Ca’ Foncello, Treviso, Italy

    B. Giometto

  7. Dipartimento di Neuroscienze, NPSRR, Università di Padova, Padua, Italy

    E. Pegoraro

  8. Dipartimento di Neuroscienze, Università di Messina, Messina, Italy

    C. Rodolico

  9. Dipartimento di Neuroscienze, Ospedale San Giovanni Battista, Torino, Italy

    M. C. Vigliani

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  1. A. Evoli
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  2. R. Liguori
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  3. A. Romani
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  5. A. Di Muzio
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  6. B. Giometto
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  7. E. Pegoraro
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  9. M. C. Vigliani
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Corresponding author

Correspondence to A. Evoli.

Additional information

For the GISMIA (Gruppo Italiano sulle Sindromi MIAsteniche) Working Group.

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Evoli, A., Liguori, R., Romani, A. et al. Italian recommendations for Lambert–Eaton myasthenic syndrome (LEMS) management. Neurol Sci 35, 515–520 (2014). https://doi.org/10.1007/s10072-014-1637-4

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  • DOI: https://doi.org/10.1007/s10072-014-1637-4

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