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, 42NIH Rare Diseases, 43NINDS, 10DISEASES, 44Novoseek, 48Orphanet, 60UMLS, 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
Large granular lymphocytic leukemia associated with Lambert-Eaton Myasthenic Syndrome: A case report. (24371773)
2013
2
Clinical presentation and differential diagnosis of Lambert-Eaton myasthenic syndrome. (23545618)
2013
3
Long-Term Follow-Up in Infantile-Onset Lambert-Eaton Myasthenic Syndrome. (24114606)
2013
4
Paraneoplastic syndromes of the neuromuscular junction: therapeutic options in myasthenia gravis, lambert-eaton myasthenic syndrome, and neuromyotonia. (23263888)
2013
5
Treatment in Lambert-Eaton myasthenic syndrome. (23278581)
2012
6
SOX1 antibodies in Lambert-Eaton myasthenic syndrome and screening for small cell lung carcinoma. (23278580)
2012
7
Lambert-Eaton myasthenic syndrome: from clinical characteristics to therapeutic strategies. (22094130)
2011
8
Sequential fluctuating paraneoplastic ocular flutter-opsoclonus-myoclonus syndrome and Lambert-Eaton myasthenic syndrome in small-cell lung cancer. (20392978)
2011
9
Case report: a case review of Lambert-Eaton myasthenic syndrome and low back pain. (21475559)
2011
10
Neuromuscular paraneoplastic syndromes: the Lambert-Eaton myasthenic syndrome. (20836646)
2010
11
Lambert-Eaton myasthenic syndrome revealing an abdominal neuroblastoma. (19635290)
2009
12
Lambert-Eaton myasthenic syndrome in childhood. (18585938)
2009
13
Lambert Eaton myasthenic syndrome. (17636143)
2007
14
Lambert-Eaton myasthenic syndrome associated with thymitis. (15642933)
2005
15
IVIg in myasthenia gravis, Lambert Eaton myasthenic syndrome and inflammatory myopathies: current status. (15959667)
2005
16
Treatment for Lambert-Eaton myasthenic syndrome. (15846654)
2005
17
Oculobulbar involvement is typical with Lambert-Eaton myasthenic syndrome. (12557298)
2003
18
Seronegative Lambert-Eaton myasthenic syndrome: study of 110 Japanese patients. (12473768)
2002
19
Postexercise facilitation of reflexes is not common in Lambert-Eaton myasthenic syndrome. (12370470)
2002
20
Respiratory failure in lambert-eaton myasthenic syndrome precipitated by calcium-channel blockers: report of a case and literature review. (19078691)
2002
21
Lambert-eaton myasthenic syndrome: a case report and review of the literature. (12626085)
2002
22
Tonic pupils in Lambert-Eaton myasthenic syndrome. (11353435)
2001
23
Long term outcome in Lambert-Eaton myasthenic syndrome without lung cancer. (11160470)
2001
24
Lambert-Eaton myasthenic syndrome as an autoimmune calcium-channelopathy. (10683522)
2000
25
Antibodies against the beta subunit of voltage-dependent calcium channels in Lambert-Eaton myasthenic syndrome. (10188953)
1999
26
Decay of postexercise augmentation in the Lambert-Eaton myasthenic syndrome: effect of cooling. (9566398)
1998
27
Functional evaluation of inhibition of autonomic transmitter release by autoantibody from Lambert-Eaton myasthenic syndrome. (9585366)
1998
28
Antibodies to synthetic peptides of the alpha1A subunit of the voltage-gated calcium channel in Lambert-Eaton myasthenic syndrome. (9153453)
1997
29
Small-cell lung cancer, paraneoplastic cerebellar degeneration and the Lambert-Eaton myasthenic syndrome. (9278623)
1997
30
Immunopathogenesis of myasthenia gravis and the Lambert-Eaton myasthenic syndrome]. (9264830)
1997
31
Lambert-Eaton (myasthenic) syndrome: pre-anaesthetic treatment with intravenous immunoglobulins. (8795338)
1996
32
Lambert-Eaton myasthenic syndrome presenting with severe respiratory failure. (8808659)
1996
33
Lambert-Eaton myasthenic syndrome (LEMS) in association with lymphoproliferative disorders. (7783761)
1995
34
Coexistence of paraneoplastic sensory neuronopathy and Lambert-Eaton myasthenic syndrome in a small cell lung cancer patient. (8072202)
1994
35
Inhibition of low- and high-threshold Ca2+ channels of human neuroblastoma IMR32 cells by Lambert-Eaton myasthenic syndrome (LEMS) IgGs. (7898770)
1994
36
Synaptotagmin can cause an immune-mediated model of Lambert-Eaton myasthenic syndrome in rats. (8285596)
1994
37
Lambert-Eaton myasthenic syndrome and polyneuropathy in a patient with epidermoid carcinoma of the lung. (8385613)
1993
38
Anesthesia for an unsuspected Lambert-Eaton myasthenic syndrome with autoantibodies and occult small cell lung carcinoma. (1309626)
1992
39
Type 2 fiber predominance in Lambert-Eaton myasthenic syndrome. (1922168)
1991
40
Immunological evidence for the co-existence of the Lambert-Eaton myasthenic syndrome and myasthenia gravis in two patients. (1650816)
1991
41
Therapeutic approaches to Lambert-Eaton myasthenic syndrome in the intra-individual comparison. (1763511)
1991
42
Neuronal antinuclear antibodies in a patient with Lambert-Eaton myasthenic syndrome and small-cell lung carcinoma. (2536905)
1989
43
Respiratory muscle weakness in the Lambert-Eaton myasthenic syndrome. (2595631)
1989
44
Lambert-Eaton myasthenic syndrome IgG depletes presynaptic membrane active zone particles by antigenic modulation. (2853605)
1988
45
Immunoglobulins of Lambert-Eaton myasthenic syndrome inhibit rat pituitary hormone release. (3122645)
1987
46
Lambert-Eaton myasthenic syndrome: I. Early morphological effects of IgG on the presynaptic membrane active zones. (3662451)
1987
47
Congenital Lambert-Eaton myasthenic syndrome. (3035103)
1987
48
Passive transfer of the Lambert-Eaton myasthenic syndrome: neuromuscular transmission in mice injected with plasma. (2997605)
1985
49
Lambert-Eaton myasthenic syndrome. (2988146)
1985
50
Increased frequency of IgG heavy chain marker Glm(2) and of HLA-B8 in Lambert-Eaton myasthenic syndrome with and without associated lung carcinoma. (2993207)
1985

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

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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
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
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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