lambert eaton myasthenic syndrome

Summary

Summary: An autoimmune disease characterized by weakness and fatigability of proximal muscles, particularly of the pelvic girdle, lower extremities, trunk, and shoulder girdle. There is relative sparing of extraocular and bulbar muscles. CARCINOMA, SMALL CELL of the lung is a frequently associated condition, although other malignancies and autoimmune diseases may be associated. Muscular weakness results from impaired impulse transmission at the NEUROMUSCULAR JUNCTION. Presynaptic calcium channel dysfunction leads to a reduced amount of acetylcholine being released in response to stimulation of the nerve. (From Adams et al., Principles of Neurology, 6th ed, pp 1471)

Top Publications

  1. Payne M, Bradbury P, Lang B, Vincent A, Han C, Newsom Davis J, et al. Prospective study into the incidence of Lambert Eaton myasthenic syndrome in small cell lung cancer. J Thorac Oncol. 2010;5:34-8 pubmed publisher
    The Lambert Eaton myasthenic syndrome (LEMS) is a paraneoplastic disorder associated with raised serum voltage-gated calcium channel (VGCC) antibodies in patients with small cell lung cancer (SCLC)...
  2. Tim R, Massey J, Sanders D. Lambert-Eaton myasthenic syndrome: electrodiagnostic findings and response to treatment. Neurology. 2000;54:2176-8 pubmed
    ..Treatment with 3, 4-diaminopyridine produced moderate to marked self-reported functional improvement in 79% of the 53 treated patients...
  3. Graus F, Lang B, Pozo Rosich P, Saiz A, Casamitjana R, Vincent A. P/Q type calcium-channel antibodies in paraneoplastic cerebellar degeneration with lung cancer. Neurology. 2002;59:764-6 pubmed
    ..Seven of 15 CSF samples had VGCC antibodies, with evidence of intrathecal synthesis in four. VGCC antibodies should be looked for in PCD, even if there are no symptoms of LEMS, and may be related to the cerebellar dysfunction...
  4. Fukuda T, Motomura M, Nakao Y, Shiraishi H, Yoshimura T, Iwanaga K, et al. Reduction of P/Q-type calcium channels in the postmortem cerebellum of paraneoplastic cerebellar degeneration with Lambert-Eaton myasthenic syndrome. Ann Neurol. 2003;53:21-8 pubmed
    ..This suggests that P/Q-type VGCCs of the cerebellar molecular layer is the immunological target in developing PCD-LEMS...
  5. Newsom Davis J. Therapy in myasthenia gravis and Lambert-Eaton myasthenic syndrome. Semin Neurol. 2003;23:191-8 pubmed
    ..In both forms, prednisone alone is an option or combined with azathioprine in NP-LEMS. In both MG and LEMS, where weakness is severe, plasma exchange or intravenous immunoglobulin treatment may provide short-term benefit...
  6. Petty R. Lambert Eaton myasthenic syndrome. Pract Neurol. 2007;7:265-7 pubmed
  7. Newsom Davis J. Lambert-Eaton myasthenic syndrome. Rev Neurol (Paris). 2004;160:177-80 pubmed
    ..In those with severe weakness, IVIg or plasmapheresis confers short-term benefits. Prednisone alone or combined with azathioprine or cyclosporin can achieve long-term control of the disorder...
  8. Oh S, Claussen G, Hatanaka Y, Morgan M. 3,4-Diaminopyridine is more effective than placebo in a randomized, double-blind, cross-over drug study in LEMS. Muscle Nerve. 2009;40:795-800 pubmed publisher
    ..A randomized, double-blind, cross-over drug trial of 3,4-DAP showed significant efficacy over placebo in patients with LEMS. As a long-term treatment, however, not all patients preferred this drug...
  9. Baslo M, Deymeer F, Serdaroglu P, Parman Y, Ozdemir C, Cuttini M. Decrement pattern in Lambert-Eaton myasthenic syndrome is different from myasthenia gravis. Neuromuscul Disord. 2006;16:454-8 pubmed

More Information

Publications62

  1. Iwanami M, Odaka M, Nakamura T, Hirata K. [Paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome associated with anti P/Q-type voltage-gated calcium channel antibody in a patient with primary double lung cancer]. Brain Nerve. 2009;61:1083-7 pubmed
    ..Physicians need to be aware that patients may develop PCD and LEMS associated with anti-VGCC antibody caused by small cell lung cancer, and a mass survey should be conducted and careful examinations performed...
  2. Roohi F, Smith P, Bergman M, Baig M, Sclar G. A diagnostic and management dilemma: combined paraneoplastic myasthenia gravis and Lambert-Eaton myasthenic syndrome presenting as acute respiratory failure. Neurologist. 2006;12:322-6 pubmed
    ..These major disorders of neuromuscular transmission are relatively common and distinctly recognized, but co-occurrence of these disorders (overlap myasthenic syndrome) is rare and has so far attracted little attention...
  3. Kanamori K, Yahata T, Otsuka K, Imanaka M, Yokota I. [A case of small cell lung carcinoma without apparent primary lesion accompanying Lambert-Eaton myasthenic syndrome]. Nihon Kokyuki Gakkai Zasshi. 2009;47:1151-5 pubmed
    ..For assessment of indistinguishable neuropathic symptoms, the possible diagnosis of paraneoplastic syndrome, such as LEMS, and the fact that early treatment for primary disease was effective, should be considered...
  4. Wirtz P, Willcox N, van der Slik A, Lang B, Maddison P, Koeleman B, et al. HLA and smoking in prediction and prognosis of small cell lung cancer in autoimmune Lambert-Eaton myasthenic syndrome. J Neuroimmunol. 2005;159:230-7 pubmed
    ..Moreover, negativity for HLA-B8 combined with smoking behavior points more strongly to an underlying SCLC and predicts a worse prognosis in SCLC-LEMS patients...
  5. Maddison P, Newsom Davis J, Mills K. Distribution of electrophysiological abnormality in Lambert-Eaton myasthenic syndrome. J Neurol Neurosurg Psychiatry. 1998;65:213-7 pubmed
    ..To assess the distribution of electrophysiological abnormality in Lambert-Eaton myasthenic syndrome (LEMS) to identify the most sensitive muscle to use in routine examination...
  6. Tschernatsch M, Gross O, Kneifel N, Kaps M, Blaes F. SOX-1 autoantibodies in patients with paraneoplastic neurological syndromes. Autoimmun Rev. 2009;8:549-51 pubmed publisher
    ..However, increasing evidence exists that they may also be associated with other neuroimmunological disorders without an underlying tumour...
  7. Fleury M, Tranchant C. [Myasthenia gravis]. Rev Prat. 2008;58:2217-24 pubmed
    ..During myasthenia crisis, intraveinous immune globulines or plasma exchanges can be used. Thymectomy is proposed in case of thymus abnormality...
  8. Nishimura T, Tasaka S, Yamada W, Hasegawa N, Soejima K, Sayama K, et al. [Small cell lung cancer with Sjögren's syndrome and Lambert-Eaton myasthenic syndrome]. Nihon Kokyuki Gakkai Zasshi. 2006;44:775-8 pubmed
    ..LEMS is frequently associated with a malignant tumor and an autoimmune disorder. We thought that in this patient, the presentation of LEMS was apparent because he had both Sjögren's syndrome and small cell lung cancer...
  9. Takamori M. Lambert-Eaton myasthenic syndrome: search for alternative autoimmune targets and possible compensatory mechanisms based on presynaptic calcium homeostasis. J Neuroimmunol. 2008;201-202:145-52 pubmed publisher
    ..We hypothesize that these signaling cascades help to compensate for the immune-mediated defects in calcium entry in LEMS, compensation that may frequently be restricted by the coincident anti-M1 mAChR antibodies in this disease...
  10. Payne S, Wilkins D, Howard R. An unusual cause of dysphagia. J Neurol Neurosurg Psychiatry. 2005;76:146 pubmed
  11. Illa I. IVIg in myasthenia gravis, Lambert Eaton myasthenic syndrome and inflammatory myopathies: current status. J Neurol. 2005;252 Suppl 1:I14-8 pubmed
    ..the current knowledge of the benefits of treating with IVIg patients with myasthenia gravis (MG), Lambert Eaton myasthenic syndrome (LEMS), dermatomyositis (DM), polymyositis (PM) and inclusion body myositis (IBM)...
  12. Skeie G, Apostolski S, Evoli A, Gilhus N, Hart I, Harms L, et al. Guidelines for the treatment of autoimmune neuromuscular transmission disorders. Eur J Neurol. 2006;13:691-9 pubmed
    ..xi) For immunosuppressive treatment of LEMS and NMT it is reasonable to adopt treatment procedures by analogy with MG (good practice point)...
  13. Bischoff P. [Which diagnostics are essential in patients with neuromuscular diseases?]. Anasthesiol Intensivmed Notfallmed Schmerzther. 2003;38:488-91 pubmed
  14. Bhatt J, Pascuzzi R. Neuromuscular disorders in clinical practice: case studies. Neurol Clin. 2006;24:233-65 pubmed
    ..These 16 brief case overviews challenge and refresh diagnostic skills and provide the framework for selected comments regarding management options...
  15. Zivaljevic M, Popovic S, Vujkov T. [Lambert-Eaton myasthenic syndrome--a rare manifestation of paraneoplastic syndrome in ovarian cancer--case report]. Med Pregl. 2005;58:495-7 pubmed
    ..Symptomatic treatment of the junctional disorder is based on cholinergic drugs, immunosuppression, immunomodulation and physical therapy useful in case of unsuccessful antineoplastic therapy...
  16. Evoli A. Clinical aspects of neuromuscular transmission disorders. Acta Neurol Scand Suppl. 2006;183:8-11 pubmed
    ..In neuromyotonia and cramp-fasciculation syndrome, that are thought to be due to anti-voltage-gated potassium channel abs, signs of peripheral nerve hyperexcitability can be associated with CNS features...
  17. D Amour M, Gariepy G, Braidy J. Lambert-Eaton myasthenic syndrome. CMAJ. 2007;176:37 pubmed
  18. Lang B, Vincent A. Autoimmune disorders of the neuromuscular junction. Curr Opin Pharmacol. 2009;9:336-40 pubmed publisher
    ..General immunosuppression is still the main treatment, but novel treatments that reduce complement-mediated damage or inhibit the binding of pathogenic antibodies are beginning to look promising. ..
  19. Pinto A, Iwasa K, Newland C, Newsom Davis J, Lang B. The action of Lambert-Eaton myasthenic syndrome immunoglobulin G on cloned human voltage-gated calcium channels. Muscle Nerve. 2002;25:715-24 pubmed
    ..Although several LEMS IgGs can be shown to bind to the alpha(1B) (C2D7) cell line, no functional effects were seen on this channel...
  20. Motomura M, Hamasaki S, Nakane S, Fukuda T, Nakao Y. Apheresis treatment in Lambert-Eaton myasthenic syndrome. Ther Apher. 2000;4:287-90 pubmed
    ..Considering our experiences and other literature, we discuss the indication of apheresis treatment of LEMS...
  21. Bayrak A, Sandikci U, Yon S, Onar M. Manifestation of Lambert-Eaton myasthenic syndrome during last trimester of pregnancy. Int J Neurosci. 2010;120:439-41 pubmed publisher
    ..With this case we wanted to emphasize the effects of pregnancy on the course of patients with LEMS...
  22. Rudnicki S. Lambert-Eaton myasthenic syndrome with pure ocular weakness. Neurology. 2007;68:1863-4 pubmed
  23. Farrugia M, Vincent A. Autoimmune mediated neuromuscular junction defects. Curr Opin Neurol. 2010;23:489-95 pubmed publisher
    ..This review summarizes the recent advances on pathogenesis of antibody-mediated disorders of the neuromuscular junction, and results of studies on clinical assessment and treatments...
  24. Takamori M. [Recent advance in research for myasthenia gravis, in relation to various antibodies affecting synaptic structure and function]. Rinsho Shinkeigaku. 2009;49:789-93 pubmed
    ..4) When one faces "seronegative" MG, one should be cautious to conformation-specific antibodies and also congenital myasthenic syndromes...
  25. Meriggioli M, Sanders D. Advances in the diagnosis of neuromuscular junction disorders. Am J Phys Med Rehabil. 2005;84:627-38 pubmed
    ..However, knowledge of the sensitivity and specificity of each test can help to focus the diagnostic evaluation and maximize the diagnostic yield of each test...
  26. Alexopoulou A, Dourakis S, Louka O, Marinaki O, Kalmantis T. Hairy cell leukemia and Lambert-Eaton myasthenic syndrome. Leukemia. 2003;17:655-6 pubmed
  27. Hoffman W, Helman S, Sekul E, Carroll J, Vega R. Lambert-Eaton Myasthenic syndrome in a child with an autoimmune phenotype. Am J Med Genet A. 2003;119A:77-80 pubmed
    ..HLA associations were present for Graves disease, vitiligo, psoriasis, and IgA deficiency. There was also evidence of autoimmunity involving the pancreatic islet cells and gastric parietal cells...
  28. Fong C. Recent advance in immunological tests in paraneoplastic neurological syndrome. Acta Neurol Taiwan. 2005;14:28-35 pubmed
    ..The recent advances in understanding of the autoimmune pathology of these disorders should lead to more effective treatment options...
  29. Vincent A, Lily O, Palace J. Pathogenic autoantibodies to neuronal proteins in neurological disorders. J Neuroimmunol. 1999;100:169-80 pubmed
  30. Tsujihata M, Ito H, Satoh A, Yoshimura T, Motomura M, Nakamura T. Semiquantitative measurement of acetylcholine receptor at the motor end-plate in myasthenia gravis. Intern Med. 2001;40:376-81 pubmed
    ..The purpose of this study was to investigate whether this semiquantitative measurement of the motor end-plate acetylcholine receptors (AChRs) can be used to confirm the diagnosis of myasthenia gravis (MG), and in particular ocular MG...
  31. Martin Moutot N, Haro L, Santos R, Mori Y, Seagar M. Phoneutria nigriventer omega-Phonetoxin IIA: a new tool for anti-calcium channel autoantibody assays in Lambert-Eaton myasthenic syndrome. Neurobiol Dis. 2006;22:57-63 pubmed
    ..1 and/or Cav2.2 antibodies detected using two different omega-conotoxins. Thus, the 125I-omegaPtxIIA assay detects a broader spectrum of autoantibody specificities than current omega-conotoxin-based assays...
  32. Mercelis R. Abnormal single-fiber electromyography in patients not having myasthenia: risk for diagnostic confusion?. Ann N Y Acad Sci. 2003;998:509-11 pubmed
  33. Isobe Z, Suga T, Katou T, Hara K, Aoki F, Aoyagi K, et al. [A case of small cell lung carcinoma complicated by Lambert-Eaton myasthenic syndrome]. Nihon Kokyuki Gakkai Zasshi. 2008;46:226-31 pubmed
    ..We believe that it is important to perform electomyogram and nerve conduction studies, not only radiographic findings, to detect the "hidden" symptoms of LEMS...
  34. Delahunt B, Abernethy D, Johnson C, Nacey J. Prostate carcinoma and the Lambert-Eaton myasthenic syndrome. J Urol. 2003;169:278-9 pubmed
  35. Titulaer M, Klooster R, Potman M, Sabater L, Graus F, Hegeman I, et al. SOX antibodies in small-cell lung cancer and Lambert-Eaton myasthenic syndrome: frequency and relation with survival. J Clin Oncol. 2009;27:4260-7 pubmed publisher
    ..Clinical outcome with respect to SOX antibodies was evaluated, as the SOX-related antitumor immune response might help to control the tumor growth...
  36. Inuzuka T. [Molecular mechanism of paraneoplastic neurological syndrome]. Nihon Ronen Igakkai Zasshi. 2003;40:204-9 pubmed
  37. Baggi F, Ubiali F, Nava S, Nessi V, Andreetta F, Rigamonti A, et al. Effect of IgG immunoadsorption on serum cytokines in MG and LEMS patients. J Neuroimmunol. 2008;201-202:104-10 pubmed publisher
    ..Our observation suggests that the massive removal of antibodies might induce modifications of the cytokine balance linked to T and B cells mediated autoimmunity...
  38. Sutton I, Winer J. The immunopathogenesis of paraneoplastic neurological syndromes. Clin Sci (Lond). 2002;102:475-86 pubmed
    ..This review article examines current concepts in the immunopathogenesis of paraneoplastic neurological syndromes...
  39. Sidnev D, Karganov M, Shcherbakova N, Alchinova I, Sanadze A. Antibodies to acetylcholine receptors in patients with different clinical forms of myasthenia and Lambert-Eaton myasthenic syndrome. Neurosci Behav Physiol. 2007;37:129-31 pubmed
  40. Rosenfeld M, Wong E, Dalmau J, Manley G, Egan D, Posner J, et al. Sera from patients with Lambert-Eaton myasthenic syndrome recognize the beta-subunit of Ca2+ channel complexes. Ann N Y Acad Sci. 1993;681:408-11 pubmed
  41. Takamori M. What's in the serum of seronegative MG and LEMS?. Neurology. 2003;61:277; author reply 277-8 pubmed
  42. Ferrer I. [Pathology of paraneoplastic syndromes of the central and peripheral nervous systems and muscle ]. Rev Neurol. 2000;31:1228-36 pubmed
    ..In this paper we describe the main paraneoplastic neurological pictures and known antibodies, including the molecular aspects and the neoplasias most frequently related to them...
  43. Titulaer M, Wirtz P, Willems L, van Kralingen K, Smitt P, Verschuuren J. Screening for small-cell lung cancer: a follow-up study of patients with Lambert-Eaton myasthenic syndrome. J Clin Oncol. 2008;26:4276-81 pubmed publisher
    ..We evaluated screening to optimize screening strategy for SCLC. It is important to detect these tumors early in newly diagnosed patients with LEMS to offer optimal patient treatment...
  44. Wirtz P, Lang B, Graus F, van den Maagdenberg A, Saiz A, de Koning Gans P, et al. P/Q-type calcium channel antibodies, Lambert-Eaton myasthenic syndrome and survival in small cell lung cancer. J Neuroimmunol. 2005;164:161-5 pubmed
    ..In both series, only patients with LEMS had a remarkably long survival, whereas presence of the antibodies without LEMS did not result in a better prognosis...
  45. Wirtz P, Kuks J, Wintzen A, Verschuuren J. [Proximal muscle weakness, depressed tendon reflexes and autonomic dysfunction: the Lambert-Eaton myasthenic syndrome]. Ned Tijdschr Geneeskd. 2001;145:57-61 pubmed
    ..In conclusion, in every patient presenting with unexplained proximal weakness, LEMS should be considered, especially if depressed tendon reflexes and autonomic dysfunction are found as well...
  46. Leger J, Bachoud Levi A, Eymard B, Theodore C, Bouche P, Pierrot Deseilligny C. [Paraneoplastic myasthenic syndrome]. Rev Neurol (Paris). 1993;149:485-8 pubmed
    ..The relationship between our case and recently reported cases of co-existence of the Lambert-Eaton myasthenic syndrome and myasthenia gravis is discussed...
  47. Yachachi Monfort A, Cortés Pastor F, de la Rubia Nieto A, Sánchez Garre M. [Treatment of Lambert-Eaton syndrome with 3,4- diaminopyridine and pyridostigmine]. Farm Hosp. 2007;31:254-6 pubmed
  48. Truffert A, Allali G, Vokatch N, Lalive P. Mild clinical expression of Lambert-Eaton myasthenic syndrome in a patient with HIV infection. J Neurol Neurosurg Psychiatry. 2007;78:910-1 pubmed
  49. Lee J, Shin J, Kim D, Jung D, Park K, Lee M, et al. A case of Lambert-Eaton myasthenic syndrome associated with atypical bronchopulmonary carcinoid tumor. J Korean Med Sci. 2004;19:753-5 pubmed
    ..We concluded that LEMS could be associated with rare pulmonary neuroendocrine tumor other than SCLC, which necessitates pathologic confirmation followed by aggressive treatment for optimal management in these rare cases...
  50. Pellkofer H, Armbruster L, Krumbholz M, Titulaer M, Verschuuren J, Schumm F, et al. Lambert-eaton myasthenic syndrome differential reactivity of tumor versus non-tumor patients to subunits of the voltage-gated calcium channel. J Neuroimmunol. 2008;204:136-9 pubmed publisher
    ..In most cases neurologic symptoms appear before tumor diagnosis, but there is as yet no clear specific serologic marker to distinguish between NT- and T-LEMS...
  51. Adams P, Snutch T. Calcium channelopathies: voltage-gated calcium channels. Subcell Biochem. 2007;45:215-51 pubmed
    ..At least in those instances wherein the channelopathies can be attributed to gain-of-function mechanisms, the data point towards new therapeutic strategies for developing highly selective calcium channel antagonists...
  52. Oh S, Hatanaka Y, Claussen G, Sher E. Electrophysiological differences in seropositive and seronegative Lambert-Eaton myasthenic syndrome. Muscle Nerve. 2007;35:178-83 pubmed
  53. Bosdure E, Attarian S, Mancini J, Mikaeloff Y, Chabrol B. [Lambert-Eaton myastenic syndrome revealing neuroblastoma in 2 children]. Arch Pediatr. 2006;13:1121-4 pubmed
    ..This disease is extremely rare in children. Only 10 cases have been reported in the last 35 years. We report 2 new observations occurring in very young patients, aged 2 and 3 years, with a ganglioneurobastoma as primitive tumor...