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

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

Risultato della ricerca: Contributo in rivistaArticolo in rivista

12 Citazioni (Scopus)

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.
Lingua originaleEnglish
pagine (da-a)515-520
Numero di pagine6
RivistaNeurological Sciences
Volume35
DOI
Stato di pubblicazionePubblicato - 2014

Keywords

  • Lambert-Eaton syndrome
  • Small-cell lung cancer

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