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

A. Evoli, R. Liguori, A. Romani, R. Mantegazza, A. Di Muzio, B. Giometto, E. Pegoraro, C. Rodolico, M. C. Vigliani

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)515-520
Number of pages6
JournalNeurological Sciences
Volume35
Issue number4
DOIs
Publication statusPublished - 2014

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Keywords

  • 3,4-Diaminopyridine
  • Lambert-Eaton myasthenic syndrome
  • Small-cell lung cancer
  • Voltage-gated calcium channel antibodies

ASJC Scopus subject areas

  • Clinical Neurology
  • Psychiatry and Mental health
  • Dermatology

Cite this

Evoli, A., Liguori, R., Romani, A., Mantegazza, R., Di Muzio, A., Giometto, B., Pegoraro, E., Rodolico, C., & Vigliani, M. C. (2014). Italian recommendations for Lambert-Eaton myasthenic syndrome (LEMS) management. Neurological Sciences, 35(4), 515-520. https://doi.org/10.1007/s10072-014-1637-4