Article thumbnail

Lambert-Eaton Myasthenic Syndrome; Pathogenesis, Diagnosis, and Therapy

By Nils Erik Gilhus

Abstract

Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare disease with a well-characterized pathogenesis. In 50% of the patients, LEMS is a paraneoplastic manifestation and caused by a small cell lung carcinoma (SCLC). Both LEMS patients with SCLC and those without this tumour have in 85% of cases pathogenetic antibodies of very high LEMS specificity against voltage-gated calcium channels (VGCCs) in the cell membrane of the presynaptic motor nerve terminal. Better understanding of LEMS pathogenesis has lead to targeted symptomatic therapy aimed at the neuromuscular junction and to semispecific immuno-suppression. For SCLC LEMS, tumour therapy is essential

Topics: Review Article
Publisher: SAGE-Hindawi Access to Research
OAI identifier: oai:pubmedcentral.nih.gov:3182560
Provided by: PubMed Central

To submit an update or takedown request for this paper, please submit an Update/Correction/Removal Request.

Suggested articles

Citations

  1. (1995). An improved diagnostic assay for LambertEaton myasthenic syndrome,”
  2. (1998). Antibodies against the calcium channel beta-subunit in Lambert-Eaton myasthenic syndrome,”
  3. Calcium channels, neuromuscular synaptic transmission and neurological diseases,”
  4. (2008). Cerebellar ataxia in non-paraneoplastic Lambert-Eaton myasthenic syndrome,”
  5. (2010). Current therapy for Lambert-Eaton myasthenic syndrome: development of 3,4-diaminopyridine phosphate salt as first-line symptomatic treatment,”
  6. (1956). E.H.Lambert,L.M.Eaton,andE.D.Rooke,“Defectofneuromuscular conduction associated with malignant neoplasms,”
  7. (1997). Effect of Lambert-Eaton myasthenic syndrome antibodies on autonomic neurons in the mouse,”
  8. (2008). EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases,”
  9. (2010). Guidelines for treatment of autoimmune neuromuscular transmission disorders,”
  10. (2008). H.L.Pellkofer,L.Armbruster,M.Krumbholzetal.,“LambertEaton myasthenic syndrome differential reactivity of tumor versus non-tumor patients to subunits of the voltage-gated calcium channel,”
  11. (2010). L o r e n z o n i ,R .H .S c o l a ,C .S .K .K a y ,S .F .P a r o l i n ,a n dL .
  12. (2009). Lambert-Eaton myastenic syndrome,”
  13. (2008). Lambert-Eaton myasthenic syndrome: search for alternative autoimmune targets and possible compensatory mechanisms based on presynaptic calcium homeostasis,”
  14. (2009). Management of myasthenic conditions: nonimmune issues,” Current Opinion
  15. (2009). Managing non-paraneoplastic Lambert-Eaton myasthenic syndrome: clinical characteristics in 25 German patients,”
  16. (2010). Myasthenia and related disorders of the neuromuscular junction,”
  17. (1991). Neurological paraneoplastic syndromes in patients with small cell lung cancer,”
  18. (1992). NewsomDavis,“Immunocytochemicalcharacteristicsofsmallcelllung carcinoma associated with the Lambert-Eaton myasthenic syndrome,”
  19. (1985). Paraneoplastic myasthenic syndrome IgG inhibits 45Ca2+ flux in a human small cell carcinoma line,”
  20. (2008). Regulatory T cells in paraneoplastic neurological syndromes,”
  21. (2011). Screening for tumours in paraneoplastic syndromes: report of an EFNS task force,” E u r o pe a nJ o u rn a lo fN eu r o l o gy ,
  22. (1997). Small-cell lung cancer, paraneoplastic cerebellar degeneration and the LambertEaton myasthenic syndrome,”
  23. (2011). Sont et al., “Clinical DutchEnglishLambert-Eatonmyasthenicsyndrome(LEMS)tumorassociationpredictionscoreaccurately predictssmall-celllung cancer in
  24. (2009). SOX antibodies in small-cell lung cancer and Lambert-Eaton myasthenic syndrome: frequency and relation with survival,”
  25. (2009). St˚ alberg, “Monozygous twins with neuromuscular transmission defects at opposite sides of the motor endplate,”
  26. (2003). The epidemiology of myasthenia gravis, Lambert-Eaton myasthenic syndrome and their associated tumours in the northern part of the province of South Holland,”
  27. (2008). The LambertEaton myasthenic syndrome 1988–2008: a clinical picture in 97 patients,”
  28. (2011). The use of rituximab
  29. (2008). Transient neonatal Lambert-Eaton syndrome,”
  30. (2011). Treatment for LambertEaton myasthenic syndrome,”
  31. (2011). Unusual paraneoplastic syndromes of breast carcinoma: a combination of cerebellar degenerationandLambert-Eatonmyasthenicsyndrome,”Irish
  32. (2001). W i r t z ,B .O .R o e p ,G .M .T .S c h r e u d e re ta l .