LEMS
MCID: LMB002
MIFTS: 50

Lambert-Eaton Myasthenic Syndrome (LEMS) malady

Neuronal category

Summaries for Lambert-Eaton Myasthenic Syndrome

Sources:
8Disease Ontology, 43NIH Rare Diseases, 44NINDS, 64Wikipedia, 33MalaCards
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NINDS:44 Lambert-Eaton myasthenic syndrome (LEMS) is a disorder of the neuromuscular junction-the site where nerve cells meet muscle cells and help activate the muscles. It is caused by a disruption of electrical impulses between these nerve and muscle cells. LEMS is an autoimmune condition; in such disorders the immune system, which normally protects the body from foreign organisms, mistakenly attacks the body's own tissues. The disruption of electrical impulses is associated with antibodies produced as a consequence of this autoimmunity. Symptoms include muscle weakness, a tingling sensation in the affected areas, fatigue, and dry mouth. LEMS is closely associated with cancer, in particular small cell lung cancer. More than half the individuals diagnosed with LEMS also develop small cell lung cancer. LEMS may appear up to 3 years before cancer is diagnosed.

MalaCards: Lambert-Eaton Myasthenic Syndrome, also known as myasthenic-myopathic syndrome of lambert-eaton, is related to cerebellar degeneration and adenocarcinoma. An important gene associated with Lambert-Eaton Myasthenic Syndrome is CACNB2 (calcium channel, voltage-dependent, beta 2 subunit), and among its related pathways are Sympathetic Nerve Pathway (Pre- and Post- Ganglionic Junction) and Calcium channels. The compounds ω-conotoxin mviic and (+-)-bay k 8644 have been mentioned in the context of this disorder. Affiliated tissues include lung, cerebellum and skeletal muscle, and related mouse phenotypes are homeostasis/metabolism and behavior/neurological.

Disease Ontology:8 A neuromuscular junction disease that results from an abnormality of acetylcholine (ach) release at the neuromuscular junction. it results from an autoimmune attack against voltage-gated calcium channels (vgcc) on the presynaptic motor nerve terminal.

NIH Rare Diseases:43 Lambert eaton myasthenic syndrome (lems) is a disorder of the neuromuscular junction. the neuromuscular junction is the site where nerve cells meet muscle cells and help activate the muscles. this syndrome occurs when antibodies interfere with electrical impulses between the nerve and muscle cells. it may be associated with other autoimmune diseases, or more commonly coincide with or precede a diagnosis of cancer such as small cell lung cancer. symptoms may include muscle weakness, a tingling sensation in the affected areas, fatigue, and dry mouth. treatment of a underlying disorder or cancer is the first priority of treatment. last updated: 4/22/2011

Wikipedia:64 Lambert–Eaton myasthenic syndrome (LEMS Lambert-Eaton syndrome or Eaton–Lambert syndrome) is a rare... more...

Aliases & Classifications for Lambert-Eaton Myasthenic Syndrome

Sources:
8Disease Ontology, 44NINDS, 10DISEASES, 45Novoseek, 49Orphanet, 61UMLS, 43NIH Rare Diseases, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet, 25ICD10
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Neuronal


Characteristics (Orphanet epidemiological data):

49
lambert-eaton myasthenic syndrome:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Adolescence / Young adulthood; Age of death: Normal


Aliases & Descriptions:

lambert-eaton myasthenic syndrome 8 44 10 45 49 61
myasthenic-myopathic syndrome of lambert-eaton 43
myasthenic syndrome of lambert-eaton 43
lambert eaton myasthenic syndrome 43
eaton lambert syndrome 43
lambert eaton syndrome 43
lems 43


External Ids:

Disease Ontology8 DOID:0050214
MESH via Orphanet36 D015624
ICD10 via Orphanet26 G73.1
SNOMED-CT via Orphanet58 56989000
UMLS via Orphanet62 C0022972
ICD1025 G73.1

Related Diseases for Lambert-Eaton Myasthenic Syndrome

Sources:
17GeneCards, 18GeneDecks
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Diseases related to Lambert-Eaton Myasthenic Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 68)
idRelated DiseaseScoreTop Affiliating Genes
1cerebellar degeneration31.3CACNA1A
2adenocarcinoma30.9ENO2, NMT1, GRP
3congenital myasthenic syndrome30.8RAPSN, MUSK
4thymoma30.6MUSK
5lambert syndrome11.7
6n syndrome11.2
7small cell carcinoma11.0
8paraneoplastic cerebellar degeneration10.8
9char syndrome10.7
10paraneoplastic syndromes10.7
11cerebellar degeneration, subacute10.7
12systemic lupus erythematosus10.6
13arthritis10.6
14autonomic dysfunction10.6
15transitional cell carcinoma10.5
16german syndrome10.5
17thymic hyperplasia10.5
18ataxia10.5
19bronchogenic carcinoma10.5
20neurogenic bladder10.4
21psoriatic arthritis10.4
22intracranial aneurysm10.4
23lung occult small cell carcinoma10.4
24adult syndrome10.4
25conn's syndrome10.4
26insulinoma10.4
27addison's disease10.4
28autonomic neuropathy10.4
29bladder carcinoma10.4
30bladder transitional cell carcinoma10.4
31botulism10.4
32strabismus10.4
33micro syndrome10.4
34wilms tumor10.4
35short syndrome10.4
36c-like syndrome10.4
37carcinoid syndrome10.4
38diffuse panbronchiolitis10.4
39limbic encephalitis10.4
40paraneoplastic neurologic disorders10.4
41back pain10.4
42myoclonus10.4
43undifferentiated connective tissue syndrome10.4
44discoid lupus erythematosus10.4
45neuroendocrine carcinoma10.2
46prostate small cell carcinoma10.2
47pituitary apoplexy10.2
48cervix small cell carcinoma10.2
49large cell carcinoma10.2
50acute leukemia10.2

Graphical network of the top 20 diseases related to Lambert-Eaton Myasthenic Syndrome:



Diseases related to lambert-eaton myasthenic syndrome

Clinical Features for Lambert-Eaton Myasthenic Syndrome

Drugs & Therapeutics for Lambert-Eaton Myasthenic Syndrome

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
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Approved drugs:

Search CenterWatch for Lambert-Eaton Myasthenic Syndrome

Drug clinical trials:

Search ClinicalTrials for Lambert-Eaton Myasthenic Syndrome

Search NIH Clinical Center for Lambert-Eaton Myasthenic Syndrome

Search CenterWatch for Lambert-Eaton Myasthenic Syndrome

Genetic Tests for Lambert-Eaton Myasthenic Syndrome

Anatomical Context for Lambert-Eaton Myasthenic Syndrome

Sources:
33MalaCards
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MalaCards organs/tissues related to Lambert-Eaton Myasthenic Syndrome:

33
Lung, Cerebellum, Skeletal muscle, Breast, Prostate, T cells, B cells, Pituitary

Animal Models for Lambert-Eaton Myasthenic Syndrome or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Lambert-Eaton Myasthenic Syndrome:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053768.8CACNA1A, CACNA1B, CACNB3, SOX3, SOX2, TRPC3
2MP:00053868.2SOX2, SOX3, CACNB3, CACNA1B, CACNA1A, SOX1
3MP:00036318.0ELAVL4, CACNB2, CACNA1A, CACNA1B, CACNB3, SOX3
4MP:00107687.9SOX2, SOX3, CACNA1B, CACNA1A, CACNB2, SOX1

Publications for Lambert-Eaton Myasthenic Syndrome

Sources:
51PubMed
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Articles related to Lambert-Eaton Myasthenic Syndrome:

(show top 50)    (show all 350)
idTitleAuthorsYear
1
Lambert-Eaton myasthenic syndrome in mixed small cell carcinoma and adenocarcinoma of extrapulmonary origin. (23298881)
2013
2
Pseudomyopathic changes in needle electromyography in lambert-eaton myasthenic syndrome. (23970984)
2013
3
Lambert-Eaton Myasthenic Syndrome presenting with a "dropped head syndrome" and associated with antibodies against N-type calcium channels. (23053834)
2013
4
Single-fiber EMG and clinical correlation in Lambert-Eaton myasthenic syndrome. (23505075)
2013
5
Lambert-Eaton myasthenic syndrome following H1N1-influenza vaccination: a case report. (22571392)
2012
6
A patient with coexisting myasthenia gravis and lambert-eaton myasthenic syndrome. (23091535)
2012
7
Lambert-Eaton myasthenic syndrome in brief. (21678709)
2011
8
Unusual paraneoplastic syndromes of breast carcinoma: a combination of cerebellar degeneration and Lambert-Eaton Myasthenic Syndrome. (19005633)
2011
9
Lambert-Eaton myasthenic syndrome as a cause of persistent neuromuscular weakness after a mediastinoscopic biopsy -A case report-. (20651998)
2010
10
Prospective study into the incidence of Lambert Eaton myasthenic syndrome in small cell lung cancer. (19934775)
2010
11
Ten-second exercise is superior to 30-second exercise for post-exercise facilitation in diagnosing Lambert-Eaton myasthenic syndrome. (18288711)
2008
12
SOX1 antibodies are markers of paraneoplastic Lambert-Eaton myasthenic syndrome. (18032743)
2008
13
The Lambert-Eaton myasthenic syndrome 1988-2008: a clinical picture in 97 patients. (18644631)
2008
14
Paraneoplastic chronic demyelinating neuropathy and Lambert-Eaton myasthenic syndrome associated with multiple anti-neural antibodies and small-cell lung cancer. (18841652)
2008
15
Antibodies to acetylcholine receptors in patients with different clinical forms of myasthenia and Lambert-Eaton myasthenic syndrome. (17187204)
2007
16
HLA and smoking in prediction and prognosis of small cell lung cancer in autoimmune Lambert-Eaton myasthenic syndrome. (15652424)
2005
17
Treatment for Lambert-Eaton myasthenic syndrome. (12804456)
2003
18
Therapy in myasthenia gravis and Lambert-Eaton myasthenic syndrome. (12894384)
2003
19
HLA-B8 in patients with the Lambert-Eaton myasthenic syndrome reduces likelihood of associated small cell lung carcinoma. (14592876)
2003
20
Prostate carcinoma and the Lambert-Eaton myasthenic syndrome. (12478159)
2003
21
Neurogenic bladder in Lambert-Eaton myasthenic syndrome and its response to 3,4-diaminopyridine. (11166786)
2001
22
HLA class I and II in Lambert-Eaton myasthenic syndrome without associated tumor. (11476904)
2001
23
Apheresis treatment in Lambert-Eaton myasthenic syndrome. (10975475)
2000
24
Antibodies to calcium channel and synaptotagmin in Lambert-Eaton myasthenic syndrome. (10768604)
2000
25
Favourable prognosis in Lambert-Eaton myasthenic syndrome and small-cell lung carcinoma. (10023900)
1999
26
Single-fiber electromyography improvement with 3,4-diaminopyridine in Lambert-Eaton myasthenic syndrome. (9655139)
1998
27
3,4-Diaminopyridine (DAP) in the treatment of Lambert-Eaton myasthenic syndrome (LEMS). (9668334)
1998
28
A treatment algorithm for Lambert-Eaton myasthenic syndrome. (9668335)
1998
29
A case of Addison's disease associated with the Lambert-Eaton myasthenic syndrome. (9294781)
1997
30
Down-regulation of non-L-, non-N-type (Q-like) Ca2+ channels by Lambert-Eaton myasthenic syndrome (LEMS) antibodies in rat insulinoma RINm5F cells. (8654565)
1996
31
Lambert-Eaton myasthenic syndrome: clinical diagnosis, immune-mediated mechanisms, and update on therapies. (8968218)
1995
32
Antigens associated with N- and L-type calcium channels in Lambert-Eaton myasthenic syndrome. (7891097)
1995
33
Cardiac arrest following an iatrogenic 3,4-diaminopyridine intoxication in a patient with Lambert-Eaton myasthenic syndrome. (7760450)
1995
34
Repetitive nerve stimulation studies in the Lambert-Eaton myasthenic syndrome. (8065402)
1994
35
Heterogeneity of calcium channel autoantibodies detected using a small-cell lung cancer line derived from a Lambert-Eaton myasthenic syndrome patient. (8309586)
1994
36
Atropine modulates presynaptic function in mice with passively transferred Lambert-Eaton myasthenic syndrome. (8423826)
1993
37
Interaction of synaptotagmin with voltage gated calcium channels: a role in Lambert-Eaton myasthenic syndrome? (8186692)
1993
38
Synaptotagmin associates with presynaptic calcium channels and is a Lambert-Eaton myasthenic syndrome antigen. (9137575)
1993
39
Systemic effect of local botulinum toxin injections unmasks subclinical Lambert-Eaton myasthenic syndrome. (8229041)
1993
40
Sera from patients with Lambert-Eaton myasthenic syndrome recognize the beta-subunit of Ca2+ channel complexes. (8395157)
1993
41
Immunocytochemical characteristics of small cell lung carcinoma associated with the Lambert-Eaton myasthenic syndrome. (1314026)
1992
42
Calcium channel autoantibodies in the Lambert-Eaton myasthenic syndrome. (1645944)
1991
43
Lambert-Eaton myasthenic syndrome: a clinical contribution. (2058358)
1991
44
Successful alternate day guanidine therapy following guanidine-induced neutropenia in the Lambert-Eaton myasthenic syndrome. (2355949)
1990
45
The Lambert-Eaton myasthenic syndrome in association with rheumatoid arthritis. (2306579)
1990
46
Transient Lambert-Eaton myasthenic syndrome associated with systemic lupus erythematosus. (2747733)
1989
47
Voltage-dependent Ca2+ channels in small cell carcinomas are blocked by autoantibodies from patients with Lambert-Eaton myasthenic syndrome. (2849894)
1988
48
Overlap myasthenic syndrome: combined myasthenia gravis and Eaton-Lambert syndrome. (3614671)
1987
49
Autoimmunity in the Lambert-Eaton myasthenic syndrome. (6302494)
1982
50
Autoimmune aetiology for myasthenic (Eaton-Lambert) syndrome. (6114283)
1981

Genetic Variations for Lambert-Eaton Myasthenic Syndrome

Expression for genes affiliated with Lambert-Eaton Myasthenic Syndrome

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Lambert-Eaton Myasthenic Syndrome

Search GEO for disease gene expression data for Lambert-Eaton Myasthenic Syndrome.

Pathways for genes affiliated with Lambert-Eaton Myasthenic Syndrome

Sources:
50PharmGKB, 12EMD Millipore, 54Reactome, 52QIAGEN, 38NCBI BioSystems Database, 30KEGG
See all sources

Pathways related to Lambert-Eaton Myasthenic Syndrome according to GeneCards/GeneDecks:

(show all 23)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.2CACNA1B, CACNA1A
210.0TRPC3, CACNA1B, CACNA1A
3
Hide members
10.0CACNB2, CACNA1A, CACNB3
49.9CACNB3, CACNA1B, CACNA1A, CACNB2
59.9CACNB3, CACNA1B, CACNA1A, CACNB2
6
Hide members
9.9CACNB3, CACNA1B, CACNA1A, CACNB2
79.9CACNB3, CACNA1B, CACNA1A, CACNB2
8
Hide members
9.9CACNB3, CACNA1B, CACNA1A, CACNB2
99.9CACNB2, CACNA1A, CACNA1B, CACNB3
10
Hide members
9.9CACNB2, CACNA1A, CACNA1B, CACNB3
11
Hide members
9.9CACNB2, CACNA1A, CACNA1B, CACNB3
12
Hide members
9.9CACNB2, CACNA1A, CACNA1B, CACNB3
139.9CACNB2, CACNA1A, CACNA1B, CACNB3
14
Hide members
9.9CACNB2, CACNA1A, CACNA1B, CACNB3
159.9CACNB2, CACNA1A, CACNA1B, CACNB3
16
Hide members
9.9CACNB2, CACNA1A, CACNA1B, CACNB3
17
Hide members
9.9CACNB2, CACNA1A, CACNA1B, CACNB3
18
Hide members
9.7CACNB2, CACNA1A, CACNA1B, CACNB3, MUSK
19
Hide members
9.7CACNA1A, CACNA1B, CACNB3, MUSK, CACNB2
20
Development Ligand-independent activation of ESR1 and ESR2
Hide members
9.7CACNB2, CACNA1A, CACNA1B, CACNB3, TRPC3
21
Hide members
9.6CACNB2, CACNA1A, CACNA1B, CACNB3, TRPC3, MUSK
22
Hide members
9.5CACNB2, CACNA1A, CACNA1B, CACNB3, HLA-DRB3, HLA-DQA1
23
Hide members
9.1CACNB2, CACNA1A, CACNA1B, CACNB3, SOX3, SOX21

Compounds for genes affiliated with Lambert-Eaton Myasthenic Syndrome

Sources:
29IUPHAR, 60Tocris Bioscience, 45Novoseek, 11DrugBank, 50PharmGKB, 24HMDB
See all sources

Compounds related to Lambert-Eaton Myasthenic Syndrome according to GeneCards/GeneDecks:

(show all 16)
idCompoundScoreTop Affiliating Genes
1ω-conotoxin mviic2910.5CACNA1A, CACNA1B
2(+-)-bay k 86446010.4CACNA1A, CACNA1B
3nnc 55-0396 dihydrochloride6010.4CACNA1A, CACNA1B
4mibefradil dihydrochloride6010.3CACNA1A, CACNA1B
5nimodipine45 29 1112.3CACNB2, CACNA1B, CACNB3
6mibefradil45 29 1112.3CACNB3, CACNA1B, CACNB2
7omega-conotoxin gvia6010.3CACNA1A, CACNA1B
8ryanodine45 29 6012.2CACNA1B, TRPC3, MUSK
9pregabalin60 45 1112.2CACNA1B, CACNA1A
10Dronedarone1110.2CACNB3, CACNB2
11verapamil45 50 29 11 2414.1CACNB3, CACNA1B, CACNA1A, CACNB2
12mibg4510.0CACNA1B, ENO2
13potassium45 11 2411.9CACNA1A, CACNA1B, MUSK, RAPSN, ENO2
14acetylcholine45 50 29 11 2413.8ENO2, RAPSN, MUSK, GRP, CACNA1B, CACNA1A
15nifedipine45 50 29 1112.7GRP, CACNA1B, CACNB2
16calcium45 50 11 2412.2CACNB2, CACNA1A, CACNA1B, CACNB3, GRP, TRPC3

GO Terms for genes affiliated with Lambert-Eaton Myasthenic Syndrome

Sources:
16Gene Ontology
See all sources

Cellular components related to Lambert-Eaton Myasthenic Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1MHC class II protein complexGO:0426139.8HLA-DRB3, HLA-DQA1
2voltage-gated calcium channel complexGO:0058919.8CACNB3, CACNA1B, CACNA1A, CACNB2
3cell junctionGO:0300549.7RAPSN, NMT1, MUSK, BSN

Biological processes related to Lambert-Eaton Myasthenic Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1chromatin organizationGO:00632510.1SOX1, SOX2
2membrane depolarizationGO:05189910.0CACNA1A, CACNA1B, CACNB3
3calcium ion transportGO:0068169.9TRPC3, CACNB3, CACNB2
4T cell receptor signaling pathwayGO:0508529.6HLA-DQA1, HLA-DRB3, CACNB3
5synaptic transmissionGO:0072689.5RAPSN, BSN, CACNB3, CACNA1B, CACNA1A, CACNB2

Molecular functions related to Lambert-Eaton Myasthenic Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1high voltage-gated calcium channel activityGO:00833110.1CACNB2, CACNA1A
2AU-rich element bindingGO:01709110.0ELAVL4, ELAVL3
3voltage-gated calcium channel activityGO:0052459.9CACNB3, CACNA1B, CACNA1A, CACNB2
4MHC class II receptor activityGO:0323959.8HLA-DQA1, HLA-DRB3

Products for genes affiliated with Lambert-Eaton Myasthenic Syndrome

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Sources for Lambert-Eaton Myasthenic Syndrome

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet