LEMS
MCID: LMB002
MIFTS: 54

Lambert-Eaton Myasthenic Syndrome (LEMS) malady

Neuronal diseases category

Summaries for Lambert-Eaton Myasthenic Syndrome

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8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 63Wikipedia, 32MalaCards
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NINDS:43 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 lung cancer and myasthenia gravis. 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:42 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:63 Lambert–Eaton myasthenic syndrome (LEMS, also Lambert–Eaton syndrome, or Eaton–Lambert syndrome)... more...

Aliases & Classifications for Lambert-Eaton Myasthenic Syndrome

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8Disease Ontology, 43NINDS, 10DISEASES, 44Novoseek, 48Orphanet, 60UMLS, 42NIH Rare Diseases, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet, 25ICD10
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Classifications:

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


Characteristics (Orphanet epidemiological data):

48
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 43 10 44 48 60
myasthenic-myopathic syndrome of lambert-eaton 42
myasthenic syndrome of lambert-eaton 42
lambert eaton myasthenic syndrome 42
eaton lambert syndrome 42
lambert eaton syndrome 42
lems 42


External Ids:

Disease Ontology8 DOID:0050214
MESH via Orphanet35 D015624
ICD10 via Orphanet26 G73.1
SNOMED-CT via Orphanet57 56989000
UMLS via Orphanet61 C0022972
ICD1025 G73.1

Related Diseases for Lambert-Eaton Myasthenic Syndrome

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17GeneCards, 18GeneDecks
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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

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Drugs & Therapeutics for Lambert-Eaton Myasthenic Syndrome

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Lambert-Eaton Myasthenic Syndrome

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Anatomical Context for Lambert-Eaton Myasthenic Syndrome

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32MalaCards
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MalaCards organs/tissues related to Lambert-Eaton Myasthenic Syndrome:

32
Lung, Cerebellum, Skeletal muscle, Breast, Prostate, Testes, T cells, B cells, Pituitary

Animal Models for Lambert-Eaton Myasthenic Syndrome or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Lambert-Eaton Myasthenic Syndrome:

36
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

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50PubMed
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Articles related to Lambert-Eaton Myasthenic Syndrome:

(show top 50)    (show all 341)
idTitleAuthorsYear
1
The exhaustibility of Lambert-Eaton myasthenic syndrome. (24125855)
2014
2
Single-fiber EMG and clinical correlation in Lambert-Eaton myasthenic syndrome. (23505075)
2013
3
Lung adenocarcinoma with Lambert-Eaton myasthenic syndrome indicated by voltage-gated calcium channel: a case report. (22950670)
2012
4
Lambert-eaton myasthenic syndrome; pathogenesis, diagnosis, and therapy. (21969911)
2011
5
Current therapy for Lambert-Eaton myasthenic syndrome: development of 3,4-diaminopyridine phosphate salt as first-line symptomatic treatment. (20377318)
2010
6
A case of small cell lung carcinoma without apparent primary lesion accompanying Lambert-Eaton myasthenic syndrome]. (20058696)
2009
7
Lambert-Eaton myasthenic syndrome. (21686564)
2009
8
Mild clinical expression of Lambert-Eaton myasthenic syndrome in a patient with HIV infection. (21686701)
2009
9
Cerebellar ataxia in non-paraneoplastic Lambert-Eaton myasthenic syndrome. (18374949)
2008
10
Lambert-Eaton myasthenic syndrome: search for alternative autoimmune targets and possible compensatory mechanisms based on presynaptic calcium homeostasis. (18653248)
2008
11
Lambert-Eaton myasthenic syndrome with pure ocular weakness. (17515551)
2007
12
Electrophysiological differences in seropositive and seronegative Lambert-Eaton myasthenic syndrome. (17058271)
2007
13
Pregnancy and delivery of a healthy baby in autoimmune Lambert-Eaton myasthenic syndrome. (16598612)
2006
14
A diagnostic and management dilemma: combined paraneoplastic myasthenia gravis and Lambert-Eaton myasthenic syndrome presenting as acute respiratory failure. (17122730)
2006
15
Electrophysiological diagnostic criteria of Lambert-Eaton myasthenic syndrome. (16003742)
2005
16
Lambert-Eaton myasthenic syndrome. Clinical and electrophysiological findings in seven cases. (15378868)
2004
17
Lambert-Eaton myasthenic syndrome. (15257511)
2004
18
Reduction of P/Q-type calcium channels in the postmortem cerebellum of paraneoplastic cerebellar degeneration with Lambert-Eaton myasthenic syndrome. (12509844)
2003
19
Differences in clinical features between the Lambert-Eaton myasthenic syndrome with and without cancer: an analysis of 227 published cases. (12140105)
2002
20
The Lambert-Eaton myasthenic syndrome. (11908225)
2002
21
Lambert-Eaton myasthenic syndrome associated with idiopathic thrombocytopenic purpura and diffuse panbronchiolitis: long-term remission after a course of intravenous immunoglobulin combined with low-dose prednisolone. (10555101)
1999
22
Endocrine function in Lambert-Eaton myasthenic syndrome. (10072063)
1999
23
Differential effect of Lambert-Eaton myasthenic syndrome immunoglobulin on cloned neuronal voltage-gated calcium channels. (9668315)
1998
24
The role of autoantibodies in Lambert-Eaton myasthenic syndrome. (9668304)
1998
25
Autonomic dysfunction in the Lambert-Eaton myasthenic syndrome: serologic and clinical correlates. (9443463)
1998
26
Lambert-Eaton myasthenic syndrome. Antigenicity of recombinant human P/Q-type calcium channel alpha 1 subunit putative ion pore region (domain IV, S5-S6). (9668316)
1998
27
Incidence of serum anti-P/O-type and anti-N-type calcium channel autoantibodies in the Lambert-Eaton myasthenic syndrome. (9094058)
1997
28
Combined low-rate nerve stimulation and maximal voluntary contraction in the detection of compound muscle action potential facilitation in Lambert-Eaton myasthenic syndrome. (9270686)
1997
29
Specificity of autoantibodies react with omega-conotoxin MVIIC-sensitive calcium channel in Lambert-Eaton myasthenic syndrome. (8761275)
1996
30
Lambert-Eaton myasthenic syndrome immunoglobulins react with multiple types of calcium channels in small-cell lung carcinoma. (8957015)
1996
31
The proteins synaptotagmin and syntaxin are not general targets of Lambert-Eaton myasthenic syndrome autoantibody. (7861157)
1995
32
Binding of Lambert-Eaton myasthenic syndrome IgG to synaptosomal proteins does not correlate with an inhibition of calcium uptake. (7783957)
1995
33
Lambert-Eaton myasthenic syndrome: pathogenesis and treatment. (7984825)
1994
34
Lambert-Eaton myasthenic syndrome in small cell lung cancer: nursing implications. (8047466)
1994
35
Effect of intravenous immunoglobulin in Lambert-Eaton myasthenic syndrome with small-cell lung cancer: correlation with the titer of anti-voltage-gated calcium channel antibody. (8065398)
1994
36
Neuronal calcium channels as target for Lambert-Eaton myasthenic syndrome autoantibodies. (8395151)
1993
37
Primary respiratory failure as the presenting symptom in Lambert-Eaton myasthenic syndrome. (8505927)
1993
38
Cloning and characterization of a Lambert-Eaton myasthenic syndrome antigen. (8494331)
1993
39
Synaptotagmin: a Lambert-Eaton myasthenic syndrome antigen that associates with presynaptic calcium channels. (8305896)
1993
40
Practical aspects of 3,4-diaminopyridine treatment of the Lambert-Eaton myasthenic syndrome. (8213058)
1993
41
Systemic effect of local botulinum toxin injections unmasks subclinical Lambert-Eaton myasthenic syndrome. (8229041)
1993
42
Sera from patients with Lambert-Eaton myasthenic syndrome recognize the beta-subunit of Ca2+ channel complexes. (8395157)
1993
43
The synaptic vesicle protein synaptotagmin associates with calcium channels and is a putative Lambert-Eaton myasthenic syndrome antigen. (1314395)
1992
44
Specificity of Lambert-Eaton myasthenic syndrome immunoglobulin for nerve terminal calcium channels. (1363289)
1992
45
The Lambert-Eaton myasthenic syndrome in association with rheumatoid arthritis. (2306579)
1990
46
Increased amplitude of F-response in Lambert-Eaton myasthenic syndrome. (2313309)
1990
47
The effect of Lambert-Eaton myasthenic syndrome antibody on slow action potentials in mouse cardiac ventricle. (2907138)
1988
48
Voltage-dependent Ca2+ channels in small cell carcinomas are blocked by autoantibodies from patients with Lambert-Eaton myasthenic syndrome. (2849894)
1988
49
Ultrastructural study of the motor end-plate in botulism and Lambert-Eaton myasthenic syndrome. (3694228)
1987
50
Interference with calcium channels by Lambert Eaton myasthenic syndrome antibody. (2446554)
1987

Genetic Variations for Lambert-Eaton Myasthenic Syndrome

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Expression for genes affiliated with Lambert-Eaton Myasthenic Syndrome

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1BioGPS, 15Gene Expression Omnibus DataSets
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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

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49PharmGKB, 12EMD Millipore, 53Reactome, 51QIAGEN, 37NCBI BioSystems Database, 29KEGG
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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
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10.0CACNB2, CACNA1A, CACNB3
49.9CACNB3, CACNA1B, CACNA1A, CACNB2
59.9CACNB3, CACNA1B, CACNA1A, CACNB2
6
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9.9CACNB3, CACNA1B, CACNA1A, CACNB2
79.9CACNB3, CACNA1B, CACNA1A, CACNB2
8
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9.9CACNB3, CACNA1B, CACNA1A, CACNB2
99.9CACNB2, CACNA1A, CACNA1B, CACNB3
10
Hide members
9.9CACNB2, CACNA1A, CACNA1B, CACNB3
11
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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
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9.7CACNB2, CACNA1A, CACNA1B, CACNB3, MUSK
19
Hide members
9.7CACNA1A, CACNA1B, CACNB3, MUSK, CACNB2
20
Development Ligand-independent activation of ESR1 and ESR2
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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

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28IUPHAR, 59Tocris Bioscience, 44Novoseek, 11DrugBank, 49PharmGKB, 24HMDB
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Compounds related to Lambert-Eaton Myasthenic Syndrome according to GeneCards/GeneDecks:

(show all 16)
idCompoundScoreTop Affiliating Genes
1ω-conotoxin mviic2810.5CACNA1A, CACNA1B
2(+-)-bay k 86445910.4CACNA1A, CACNA1B
3nnc 55-0396 dihydrochloride5910.4CACNA1A, CACNA1B
4mibefradil dihydrochloride5910.3CACNA1A, CACNA1B
5nimodipine44 28 1112.3CACNB2, CACNA1B, CACNB3
6mibefradil44 28 1112.3CACNB3, CACNA1B, CACNB2
7omega-conotoxin gvia5910.3CACNA1A, CACNA1B
8ryanodine44 28 5912.2CACNA1B, TRPC3, MUSK
9pregabalin59 44 1112.2CACNA1B, CACNA1A
10Dronedarone1110.2CACNB3, CACNB2
11verapamil44 49 28 11 2414.1CACNB3, CACNA1B, CACNA1A, CACNB2
12mibg4410.0CACNA1B, ENO2
13potassium44 11 2411.9CACNA1A, CACNA1B, MUSK, RAPSN, ENO2
14acetylcholine44 49 28 11 2413.8ENO2, RAPSN, MUSK, GRP, CACNA1B, CACNA1A
15nifedipine44 49 28 1112.7GRP, CACNA1B, CACNB2
16calcium44 49 11 2412.2CACNB2, CACNA1A, CACNA1B, CACNB3, GRP, TRPC3

GO Terms for genes affiliated with Lambert-Eaton Myasthenic Syndrome

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16Gene Ontology
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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

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet