LEMS
MCID: LMB002
MIFTS: 53

Lambert-Eaton Myasthenic Syndrome (LEMS) malady

Rare diseases, Neuronal diseases categories
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Summaries for Lambert-Eaton Myasthenic Syndrome

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8Disease Ontology, 43NIH Rare Diseases, 44NINDS, 65Wikipedia, 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 lambert syndrome and lung cancer. An important gene associated with Lambert-Eaton Myasthenic Syndrome is CACNB2 (calcium channel, voltage-dependent, beta 2 subunit), and among its related pathways are ADP signalling through P2Y purinoceptor 12 and Integration of energy metabolism. The compounds nimodipine and nitrendipine have been mentioned in the context of this disorder. Affiliated tissues include lung, cerebellum and skeletal muscle, and related mouse phenotypes are respiratory system and craniofacial.

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:65 Lambert?Eaton myasthenic syndrome (LEMS, also Lambert?Eaton syndrome, or Eaton?Lambert syndrome) is a... more...

Aliases & Classifications for Lambert-Eaton Myasthenic Syndrome

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8Disease Ontology, 44NINDS, 10DISEASES, 45Novoseek, 49Orphanet, 62UMLS, 43NIH Rare Diseases, 36MESH via Orphanet, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet, 25ICD10
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Classifications:

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


Characteristics (Orphanet epidemiological data):

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


Aliases & Descriptions:

lambert-eaton myasthenic syndrome 8 44 10 45 49 62
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 Orphanet59 56989000
UMLS via Orphanet63 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

Symptoms for Lambert-Eaton Myasthenic Syndrome

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Drugs & Therapeutics for Lambert-Eaton Myasthenic Syndrome

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42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Lambert-Eaton Myasthenic Syndrome

Search NIH Clinical Center for Lambert-Eaton Myasthenic Syndrome

Genetic Tests for Lambert-Eaton Myasthenic Syndrome

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Anatomical Context for Lambert-Eaton Myasthenic Syndrome

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

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

Animal Models for Lambert-Eaton Myasthenic Syndrome or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Lambert-Eaton Myasthenic Syndrome:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053888.8SOX2, MUSK, CACNA1A
2MP:00053828.7SOX3, CACNB2, SOX2
3MP:00107718.4CACNA1A, MUSK, SOX2, CACNA1B
4MP:00030128.2SOX2, CACNA1A, SOX3
5MP:00053847.9MUSK, SOX3, SOX2, CACNA1A
6MP:00053867.6SOX3, MUSK, CACNA1A, CACNA1B, SOX2
7MP:00036317.3MUSK, CACNA1B, CACNA1A, CACNB2, SOX2, SOX3
8MP:00053787.3CACNA1B, MUSK, SOX2, SOX3, CACNB2, CACNA1A
9MP:00053767.3CACNA1A, SOX3, MUSK, CACNB2, SOX2, CACNA1B
10MP:00107687.3CACNA1A, CACNA1B, CACNB2, MUSK, SOX2, SOX3

Publications for Lambert-Eaton Myasthenic Syndrome

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52PubMed
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Articles related to Lambert-Eaton Myasthenic Syndrome:

(show top 50)    (show all 335)
idTitleAuthorsYear
1
Limbic encephalitis and Lambert Eaton myasthenic syndrome - An immunological profile of a new syndrome. (24269050)
2014
2
Antibodies to active zone protein ERC1 in Lambert-Eaton myasthenic syndrome. (23583364)
2013
3
Large granular lymphocytic leukemia associated with Lambert-Eaton Myasthenic Syndrome: A case report. (24371773)
2013
4
Lambert-Eaton myasthenic syndrome in mixed small cell carcinoma and adenocarcinoma of extrapulmonary origin. (23298881)
2013
5
Lambert-Eaton myasthenic syndrome following H1N1-influenza vaccination: a case report. (22571392)
2012
6
A case of lambert-eaton myasthenic syndrome with small-cell lung cancer and transient increase in anti-acetylcholine-receptor-binding antibody titer. (23323140)
2012
7
Lambert-Eaton myasthenic syndrome in brief. (21678709)
2011
8
Lambert-Eaton myasthenic syndrome as a cause of persistent neuromuscular weakness after a mediastinoscopic biopsy -A case report-. (20651998)
2010
9
Neuromuscular paraneoplastic syndromes: the Lambert-Eaton myasthenic syndrome. (20836646)
2010
10
Prospective study into the incidence of Lambert Eaton myasthenic syndrome in small cell lung cancer. (19934775)
2010
11
Surgical correction of strabismus in Lambert-Eaton myasthenic syndrome: case reports. (19347132)
2009
12
Favorable response to rituximab in a patient with anti-VGCC-positive Lambert-Eaton myasthenic syndrome and cerebellar dysfunction. (19609921)
2009
13
Lambert-eaton myasthenic syndrome. (19210909)
2009
14
The potential use of ephedrine in Lambert-Eaton myasthenic syndrome : clinical and electrophysiological evaluation. (18535871)
2008
15
Cerebellar ataxia in non-paraneoplastic Lambert-Eaton myasthenic syndrome. (18374949)
2008
16
Lambert-Eaton myasthenic syndrome with pure ocular weakness. (17515551)
2007
17
Antibodies to acetylcholine receptors in patients with different clinical forms of myasthenia and Lambert-Eaton myasthenic syndrome. (17187204)
2007
18
Electrophysiological differences in seropositive and seronegative Lambert-Eaton myasthenic syndrome. (17058271)
2007
19
A case of small cell carcinoma of the lung associated with paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome]. (16482925)
2006
20
Associated autoimmune diseases in patients with the Lambert-Eaton myasthenic syndrome and their families. (15503107)
2004
21
Treatment for Lambert-Eaton myasthenic syndrome. (12804456)
2003
22
High innate production of interleukin-10 and tumor necrosis factor-alpha contributes to susceptibility for non-paraneoplastic Lambert-Eaton myasthenic syndrome. (12864989)
2003
23
Seronegative Lambert-Eaton myasthenic syndrome: study of 110 Japanese patients. (12473768)
2002
24
Lambert-Eaton Myasthenic Syndrome. (11180749)
2001
25
Tonic pupils in Lambert-Eaton myasthenic syndrome. (11353435)
2001
26
The Lambert-Eaton myasthenic syndrome. (11269225)
2001
27
A randomized trial of 3,4-diaminopyridine in Lambert-Eaton myasthenic syndrome. (10680790)
2000
28
Lambert-Eaton myasthenic syndrome. (10616683)
1999
29
Decay of postexercise augmentation in the Lambert-Eaton myasthenic syndrome: effect of cooling. (9566398)
1998
30
Passive transfer of Lambert-Eaton myasthenic syndrome induces dihydropyridine sensitivity of ICa in mouse motor nerve terminals. (9744921)
1998
31
Acetylcholine receptor antibodies in the Lambert-Eaton myasthenic syndrome. (9484374)
1998
32
Differential effect of Lambert-Eaton myasthenic syndrome immunoglobulin on cloned neuronal voltage-gated calcium channels. (9668315)
1998
33
The role of autoantibodies in Lambert-Eaton myasthenic syndrome. (9668304)
1998
34
Single-fiber electromyography improvement with 3,4-diaminopyridine in Lambert-Eaton myasthenic syndrome. (9655139)
1998
35
A case of Addison's disease associated with the Lambert-Eaton myasthenic syndrome. (9294781)
1997
36
Lambert-Eaton myasthenic syndrome: clinical diagnosis, immune-mediated mechanisms, and update on therapies. (8968218)
1995
37
Lambert-Eaton myasthenic syndrome (LEMS) in association with lymphoproliferative disorders. (7783761)
1995
38
Lambert-Eaton myasthenic syndrome: pathogenesis and treatment. (7984825)
1994
39
Lambert-Eaton myasthenic syndrome in small cell lung cancer: nursing implications. (8047466)
1994
40
A Lambert-Eaton myasthenic syndrome antigen associated with presynaptic calcium channels. (8395427)
1993
41
Neuronal calcium channels as target for Lambert-Eaton myasthenic syndrome autoantibodies. (8395151)
1993
42
Serum and plasma from patients with Lambert-Eaton Myasthenic Syndrome reduce depolarization-dependent uptake of 45Ca2+ into rat cortical synaptosomes. (1814548)
1991
43
Type 2 fiber predominance in Lambert-Eaton myasthenic syndrome. (1922168)
1991
44
Effects of botulinum neurotoxin and Lambert-Eaton myasthenic syndrome IgG at mouse nerve terminals. (2550027)
1989
45
The effect of Lambert-Eaton myasthenic syndrome antibody on slow action potentials in mouse cardiac ventricle. (2907138)
1988
46
Lambert-Eaton myasthenic syndrome: the lack of short-term in vitro effects of serum factors on neuromuscular transmission. (2848093)
1988
47
Lambert-Eaton myasthenic syndrome IgG depletes presynaptic membrane active zone particles by antigenic modulation. (2853605)
1988
48
Lambert-Eaton myasthenic syndrome: evidence for calcium channel blockade. (2446555)
1987
49
Immunoglobulins of Lambert-Eaton myasthenic syndrome inhibit rat pituitary hormone release. (3122645)
1987
50
Lambert-Eaton myasthenic syndrome IgG: early morphologic effects and immunolocalization at the motor endplate. (3479929)
1987

Variations for Lambert-Eaton Myasthenic Syndrome

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Expression for genes affiliated with Lambert-Eaton Myasthenic Syndrome

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2BioGPS, 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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50PathCards, 55Reactome, 60Thomson Reuters, 53QIAGEN, 51PharmGKB, 38NCBI BioSystems Database, 30KEGG, 12EMD Millipore
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Pathways related to Lambert-Eaton Myasthenic Syndrome according to GeneCards/GeneDecks:

(show all 33)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.6CACNB2, CACNA1B
2
Show member pathways
9.4CACNB2, CACNA1A
3
Show member pathways
9.4CACNA1A, CACNA1B
49.4CACNA1A, CACNA1B
59.4CACNA1B, CACNA1A
69.4CACNA1A, CACNA1B
79.4CACNA1B, CACNA1A
8
Show member pathways
9.4CACNA1A, CACNA1B
99.4CACNA1A, CACNA1B
109.4CACNA1B, CACNA1A
11
Show member pathways
Calcium Regulation in the Cardiac Cell38
9.4CACNA1A, CACNA1B
12
Show member pathways
Synaptic Vesicle Pathway38
9.4CACNA1B, CACNA1A
13
Show member pathways
9.0CACNA1B, CACNB2, CACNA1A
14
Show member pathways
9.0CACNA1B, CACNA1A, CACNB2
15
Show member pathways
9.0CACNA1B, CACNB2, CACNA1A
16
Show member pathways
9.0CACNA1B, CACNA1A, CACNB2
17
Show member pathways
9.0CACNB2, CACNA1A, CACNA1B
18
Show member pathways
9.0CACNA1B, CACNB2, CACNA1A
19
Show member pathways
9.0CACNA1A, CACNA1B, CACNB2
20
Show member pathways
Development Ligand independent activation of ESR1 and ESR260
9.0CACNB2, CACNA1B, CACNA1A
21
Show member pathways
9.0CACNA1B, CACNB2, CACNA1A
229.0CACNB2, CACNA1A, CACNA1B
239.0CACNA1A, CACNB2, CACNA1B
24
Show member pathways
MAPK signaling pathway38
9.0CACNA1B, CACNA1A, CACNB2
259.0CACNB2, CACNA1A, CACNA1B
26
Show member pathways
9.0CACNA1B, CACNA1A, CACNB2
27
Show member pathways
9.0CACNA1B, CACNA1A, CACNB2
289.0CACNB2, CACNA1A, CACNA1B
29
Show member pathways
9.0CACNB2, CACNA1A, CACNA1B
30
Show member pathways
8.5MUSK, CACNB2, CACNA1A, CACNA1B
31
Show member pathways
8.5MUSK, CACNA1B, CACNB2, CACNA1A
32
Show member pathways
8.5CACNB2, CACNA1A, CACNA1B, MUSK
33
Show member pathways
7.1SOX2, CACNB2, CACNA1A, CACNA1B, SOX3, MUSK

Compounds for genes affiliated with Lambert-Eaton Myasthenic Syndrome

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45Novoseek, 29IUPHAR, 11DrugBank, 51PharmGKB, 24HMDB, 61Tocris Bioscience
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Compounds related to Lambert-Eaton Myasthenic Syndrome according to GeneCards/GeneDecks:

(show all 18)
idCompoundScoreTop Affiliating Genes
1nimodipine45 29 1111.9CACNA1B, CACNB2
2nitrendipine45 51 29 1112.8CACNA1B, CACNB2
3mibefradil45 29 1111.8CACNB2, CACNA1B
4amlodipine45 51 24 1112.8CACNA1B, CACNB2
5nicardipine45 29 1111.7CACNA1B, CACNB2
6omega-conotoxin mviic299.7CACNA1B, CACNA1A
7(+-)-bay k 8644619.7CACNA1A, CACNA1B
8nnc 55-0396 dihydrochloride619.7CACNA1A, CACNA1B
9mibefradil dihydrochloride619.7CACNA1A, CACNA1B
10omega-conotoxin gvia61 2910.7CACNA1A, CACNA1B
11pregabalin61 45 1111.7CACNA1A, CACNA1B
12ryanodine45 29 6111.5CACNA1B, MUSK
13nicotine45 29 51 1112.5CACNA1B, MUSK
14nifedipine45 29 51 1112.3CACNA1B, CACNB2
15verapamil45 29 51 24 1113.3CACNA1B, CACNA1A, CACNB2
16acetylcholine45 51 29 24 1113.1CACNA1B, CACNA1A, MUSK
17potassium45 24 1110.9MUSK, CACNA1A, CACNA1B
18calcium45 51 24 1111.4MUSK, CACNB2, CACNA1A, CACNA1B

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
1voltage-gated calcium channel complexGO:0058919.0CACNA1B, CACNA1A, CACNB2

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

idNameGO IDScoreTop Affiliating Genes
1membrane depolarizationGO:0518999.5CACNA1B, CACNA1A
2neuromuscular junction developmentGO:0075289.4MUSK, CACNB2
3pituitary gland developmentGO:0219839.1SOX2, SOX3
4negative regulation of neuron differentiationGO:0456659.1SOX2, SOX3
5synaptic transmissionGO:0072688.7CACNA1B, CACNA1A, CACNB2

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

idNameGO IDScoreTop Affiliating Genes
1high voltage-gated calcium channel activityGO:0083319.1CACNA1A, CACNB2
2voltage-gated calcium channel activityGO:0052458.9CACNA1B, CACNA1A, CACNB2
3protein bindingGO:0055157.9MUSK, CACNB2, CACNA1A, CACNA1B, SOX2

Products for genes affiliated with Lambert-Eaton Myasthenic Syndrome

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Sources for Lambert-Eaton Myasthenic Syndrome

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4CDC
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
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet