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Eaton-Lambert syndrome(LEMS)

MedGen UID:
6005
•Concept ID:
C0022972
•
Disease or Syndrome
Synonyms: Lambert-Eaton Myasthenic Syndrome; LEMS; Myasthenic syndrome of Lambert-Eaton; Myasthenic-Myopathic syndrome of Lambert-Eaton
SNOMED CT: Lambert-Eaton myasthenic syndrome (56989000); LEMS - Lambert-Eaton myasthenic syndrome (56989000); Eaton-Lambert syndrome (56989000); Myasthenic syndrome (56989000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018556
Orphanet: ORPHA43393

Definition

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune, presynaptic disorder of neuromuscular transmission characterized by fluctuating muscle weakness and autonomic dysfunction frequently associated with small-cell lung cancer (SCLC). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEaton-Lambert syndrome
Follow this link to review classifications for Eaton-Lambert syndrome in Orphanet.

Professional guidelines

PubMed

Foodborne Botulism: Clinical Diagnosis and Medical Treatment.
Lonati D, Schicchi A, Crevani M, Buscaglia E, Scaravaggi G, Maida F, Cirronis M, Petrolini VM, Locatelli CA
Toxins (Basel) 2020 Aug 7;12(8) doi: 10.3390/toxins12080509. PMID: 32784744Free PMC Article
Consensus statement: the use of intravenous immunoglobulin in the treatment of neuromuscular conditions report of the AANEM ad hoc committee.
Donofrio PD, Berger A, Brannagan TH 3rd, Bromberg MB, Howard JF, Latov N, Quick A, Tandan R
Muscle Nerve 2009 Nov;40(5):890-900. doi: 10.1002/mus.21433. PMID: 19768755
Pearls and pitfalls in the diagnosis and management of neuromuscular junction disorders.
Pascuzzi RM
Semin Neurol 2001 Dec;21(4):425-40. doi: 10.1055/s-2001-19414. PMID: 11774058

Recent clinical studies

Etiology

3,4-diaminopyridine base effectively treats the weakness of Lambert-Eaton myasthenia.
Sanders DB, Juel VC, Harati Y, Smith AG, Peltier AC, Marburger T, Lou JS, Pascuzzi RM, Richman DP, Xie T, Demmel V, Jacobus LR, Aleš KL, Jacobus DP; Dapper Study Team
Muscle Nerve 2018 Apr;57(4):561-568. Epub 2018 Feb 2 doi: 10.1002/mus.26052. PMID: 29280483Free PMC Article
Trovafloxacin-induced weakness due to a demyelinating polyneuropathy.
Murray CK, Wortmann GW
South Med J 2000 May;93(5):514-5. PMID: 10832955
Neurologic disorders in patients with small cell lung cancer.
Sculier JP, Feld R, Evans WK, DeBoer G, Shepherd FA, Payne DG, Pringle JF, Yeoh JL, Quirt IC, Curtis JE
Cancer 1987 Nov 1;60(9):2275-83. doi: 10.1002/1097-0142(19871101)60:9<2275::aid-cncr2820600929>3.0.co;2-3. PMID: 2830955
Small cell carcinoma of lung: a prospective clinical study.
Chee YC
Ann Acad Med Singap 1985 Jul;14(3):450-6. PMID: 3000269
Edrophonium test in Eaton-Lambert syndrome: quantitative oculography.
Dell'Osso LF, Ayyar DR, Daroff RB, Abel LA
Neurology 1983 Sep;33(9):1157-63. doi: 10.1212/wnl.33.9.1157. PMID: 6684251

Diagnosis

Foodborne Botulism: Clinical Diagnosis and Medical Treatment.
Lonati D, Schicchi A, Crevani M, Buscaglia E, Scaravaggi G, Maida F, Cirronis M, Petrolini VM, Locatelli CA
Toxins (Basel) 2020 Aug 7;12(8) doi: 10.3390/toxins12080509. PMID: 32784744Free PMC Article
A malignancy work-up in patients with cancer-associated (paraneoplastic) autoimmune diseases: pemphigus and myasthenic syndromes as cases in point (review).
Sherer Y, Shoenfeld Y
Oncol Rep 1999 May-Jun;6(3):665-8. doi: 10.3892/or.6.3.665. PMID: 10203612
Paraneoplastic syndromes: a challenge for neuroscience nursing.
Alexander J, Foltz A, Parylovich K
J Neurosci Nurs 1993 Aug;25(4):228-32. doi: 10.1097/01376517-199308000-00006. PMID: 8228394
Small cell carcinoma of lung: a prospective clinical study.
Chee YC
Ann Acad Med Singap 1985 Jul;14(3):450-6. PMID: 3000269
Unusual tumours of the lung.
Wright ES, Pike E, Couves CM
J Surg Oncol 1983 Sep;24(1):23-9. doi: 10.1002/jso.2930240106. PMID: 6310266

Therapy

3,4-diaminopyridine base effectively treats the weakness of Lambert-Eaton myasthenia.
Sanders DB, Juel VC, Harati Y, Smith AG, Peltier AC, Marburger T, Lou JS, Pascuzzi RM, Richman DP, Xie T, Demmel V, Jacobus LR, Aleš KL, Jacobus DP; Dapper Study Team
Muscle Nerve 2018 Apr;57(4):561-568. Epub 2018 Feb 2 doi: 10.1002/mus.26052. PMID: 29280483Free PMC Article
Plasmapheresis: first year of experience at Humana Hospital-Alaska.
Neubauer RL, Morris GG
Alaska Med 1990 Oct-Dec;32(4):138-40. PMID: 2080799
Small cell carcinoma of lung: a prospective clinical study.
Chee YC
Ann Acad Med Singap 1985 Jul;14(3):450-6. PMID: 3000269
4-aminopyridine-a review.
Soni N, Kam P
Anaesth Intensive Care 1982 May;10(2):120-6. doi: 10.1177/0310057X8201000205. PMID: 6285756
Treatment with oral 4-aminopyridine in disorders of neuromuscular transmission.
Murray NM, Newsom-Davis J
Neurology 1981 Mar;31(3):265-71. doi: 10.1212/wnl.31.3.265. PMID: 6259555

Prognosis

Trovafloxacin-induced weakness due to a demyelinating polyneuropathy.
Murray CK, Wortmann GW
South Med J 2000 May;93(5):514-5. PMID: 10832955
Small cell carcinoma of lung: a prospective clinical study.
Chee YC
Ann Acad Med Singap 1985 Jul;14(3):450-6. PMID: 3000269
Unusual tumours of the lung.
Wright ES, Pike E, Couves CM
J Surg Oncol 1983 Sep;24(1):23-9. doi: 10.1002/jso.2930240106. PMID: 6310266
Clinical and subclinical oculomotor findings in the Eaton-Lambert syndrome.
Cruciger MP, Brown B, Denys EH, Magoon E, Wasson PJ, Newman NM, Scott AB
J Clin Neuroophthalmol 1983 Mar;3(1):19-22. PMID: 6222072
Neuromuscular transmission in thyrotoxicosis.
Puvanendran K, Cheah JS, Naganathan N, Yeo PP, Wong PK
J Neurol Sci 1979 Sep;43(1):47-57. doi: 10.1016/0022-510x(79)90072-8. PMID: 230318

Clinical prediction guides

False positive acetylcholine receptor antibodies in a case of unilateral chronic progressive external ophthalmoplegia: case report and review of literature.
Rajput R, Sachdev A, Din N, Damato EM, Murray A
Orbit 2018 Oct;37(5):385-388. Epub 2018 Jan 15 doi: 10.1080/01676830.2017.1423350. PMID: 29333908
Interferon-gamma, interleukin-4 and transforming growth factor-beta mRNA expression in multiple sclerosis and myasthenia gravis.
Link J
Acta Neurol Scand Suppl 1994;158:1-58. PMID: 7732782
Epidural analgesia in Eaton-Lambert myasthenic syndrome. Effects on respiratory function.
Sakura S, Saito Y, Maeda M, Kosaka Y
Anaesthesia 1991 Jul;46(7):560-2. doi: 10.1111/j.1365-2044.1991.tb09656.x. PMID: 1862896
The Eaton-Lambert syndrome.
Sandyk R
S Afr Med J 1983 Feb 26;63(9):323-5. PMID: 6298957
Autoimmune aetiology for myasthenic (Eaton-Lambert) syndrome.
Lang B, Newsom-Davis J, Wray D, Vincent A, Murray N
Lancet 1981 Aug 1;2(8240):224-6. doi: 10.1016/s0140-6736(81)90474-8. PMID: 6114283

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