LGS
MCID: LNN001
MIFTS: 46

Lennox-Gastaut Syndrome (LGS) malady

Neuronal category

Summaries for Lennox-Gastaut Syndrome

Sources:
43NIH Rare Diseases, 44NINDS, 21Genetics Home Reference, 47OMIM, 33MalaCards
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NINDS:44 Lennox-Gastaut syndrome is a severe form of epilepsy. Seizures usually begin before 4 years of age. Seizure types, which vary among patients, include tonic (stiffening of the body, upward deviation of the eyes, dilation of the pupils, and altered respiratory patterns), atonic (brief loss of muscle tone and consciousness, causing abrupt falls), atypical absence (staring spells), and myoclonic (sudden muscle jerks). There may be periods of frequent seizures mixed with brief, relatively seizure-free periods. Most children with Lennox-Gastaut syndrome experience some degree of impaired intellectual functioning or information processing, along with developmental delays, and behavioral disturbances. Lennox-Gastaut syndrome can be caused by brain malformations, perinatal asphyxia, severe head injury, central nervous system infection and inherited degenerative or metabolic conditions. In 30-35 percent of cases, no cause can be found.

MalaCards: Lennox-Gastaut Syndrome, also known as epileptic encephalopathy lennox-gastaut type, is related to status epilepticus and tuberous sclerosis. An important gene associated with Lennox-Gastaut Syndrome is SCN1A (sodium channel, voltage-gated, type I, alpha subunit), and among its related pathways are Adipocytokine signaling pathway and Melanocyte Development and Pigmentation. The compounds topiramate and etorphine have been mentioned in the context of this disorder. Affiliated tissues include brain, cortex and heart, and related mouse phenotypes are nervous system and behavior/neurological.

NIH Rare Diseases:43 Lennox-gastaut syndrome is a form of severe epilepsy that begins in childhood. it is characterized by multiple types of seizures and intellectual disability. this condition can be caused by brain malformations, perinatal asphyxia (lack of oxygen), severe head injury, central nervous system infection and inherited degenerative or metabolic conditions. in about one-third of cases, no cause can be found. treatment for lennox-gastaut syndrome includes anti-epileptic medications such as valproate, lamotrigine, felbamate, or topiramate. there is usually no single antiepileptic medication that will control seizures. children may improve initially, but many later show tolerance to a drug or develop uncontrollable seizures. last updated: 9/17/2013

Genetics Home Reference:21 Lennox-Gastaut syndrome is a form of severe epilepsy that begins in childhood. It is characterized by multiple types of seizures and intellectual disability.

Description from OMIM:47 606369

Aliases & Classifications for Lennox-Gastaut Syndrome

Sources:
8Disease Ontology, 43NIH Rare Diseases, 21Genetics Home Reference, 44NINDS, 10DISEASES, 45Novoseek, 49Orphanet, 61UMLS, 20GeneTests, 22GTR, 47OMIM, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
lennox-gastaut syndrome:
Inheritance: Autosomal dominant,Sporadic; Prevalence: 1-5/10000; Age of onset: Childhood


Aliases & Descriptions:

lennox-gastaut syndrome 8 43 21 44 10 45 49 61
epileptic encephalopathy lennox-gastaut type 43 20 22
lennox syndrome 8 61
childhood epileptic encephalopathy with diffuse slow spikes and waves 21
encephalopathy of childhood 43
lgs 21


External Ids:

Disease Ontology8 DOID:0050561
OMIM47 606369
MESH via Orphanet36 C535500
ICD10 via Orphanet26 G40.4
SNOMED-CT via Orphanet58 230418006
UMLS via Orphanet62 C0238111

Related Diseases for Lennox-Gastaut Syndrome

Sources:
17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Lennox-Gastaut Syndrome:



Diseases related to lennox-gastaut syndrome

Clinical Features for Lennox-Gastaut Syndrome

Sources:
47OMIM
See all sources

Clinical features from OMIM:

606369

Drugs & Therapeutics for Lennox-Gastaut Syndrome

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

Search CenterWatch for Lennox-Gastaut Syndrome

Drug clinical trials:

Search ClinicalTrials for Lennox-Gastaut Syndrome

Search NIH Clinical Center for Lennox-Gastaut Syndrome

Search CenterWatch for Lennox-Gastaut Syndrome

Genetic Tests for Lennox-Gastaut Syndrome

Sources:
20GeneTests, 22GTR
See all sources

Genetic tests related to Lennox-Gastaut Syndrome:

id Genetic test Affiliating Genes
1 Epileptic Encephalopathy, Lennox-gastaut Type20 MAPK10
2 Epileptic Encephalopathy Lennox-gastaut Type22

Anatomical Context for Lennox-Gastaut Syndrome

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Lennox-Gastaut Syndrome:

33
Brain, Cortex, Heart, Liver, Thalamus

Animal Models for Lennox-Gastaut Syndrome or affiliated genes

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

MGI Mouse Phenotypes related to Lennox-Gastaut Syndrome:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00036317.4GPR56, AVIL, MAPK10, POMC, DCX, SCN1A
2MP:00053866.7GPR56, SCN1A, DCX, CHD2, POMC, MAPK10

Publications for Lennox-Gastaut Syndrome

Sources:
51PubMed
See all sources

Articles related to Lennox-Gastaut Syndrome:

(show top 50)    (show all 243)
idTitleAuthorsYear
1
Glycemic index treatment using Japanese foods in a girl with Lennox-Gastaut syndrome. (23583057)
2013
2
Copy number variants in adult patients with Lennox-Gastaut syndrome features. (23415449)
2013
3
Treatment of Lennox-Gastaut syndrome. (23450537)
2013
4
Lennox-Gastaut syndrome in south Iran: electro-clinical manifestations. (22921514)
2012
5
Efficacy of the ketogenic diet in Lennox-Gastaut syndrome: a retrospective review of one institution's experience and summary of the literature. (22443637)
2012
6
Clobazam : in patients with Lennox-Gastaut syndrome. (23034582)
2012
7
ONe More Weapon in the FIght Against Drop Seizures in Lennox-Gastaut Syndrome. (22473540)
2012
8
Localization of ictal onset zones in Lennox-Gastaut syndrome using directed transfer function method. (22255466)
2011
9
Valproate treatment after liver transplant in a patient with Lennox-Gastaut syndrome. (21376637)
2011
10
Levetiracetam efficacy in patients with Lennox-Gastaut syndrome. Presentation of a case. (21353345)
2011
11
Strategies for transitioning to adult care for youth with Lennox-Gastaut syndrome and related disorders. (21790562)
2011
12
Rufinamide for pediatric patients with Lennox-Gastaut syndrome: a comprehensive overview. (21351809)
2011
13
Increased risk of death among children with Lennox-Gastaut syndrome and infantile spasms. (20023065)
2010
14
Medical management of Lennox-Gastaut syndrome. (20158289)
2010
15
Lennox-Gastaut syndrome in adulthood: clinical and EEG features. (20149600)
2010
16
The cost effectiveness of rufinamide in the treatment of Lennox-Gastaut syndrome in the UK. (20151724)
2010
17
Rufinamide: a new antiepileptic medication for the treatment of seizures associated with lennox-gastaut syndrome. (20233912)
2010
18
Long-term vagus nerve stimulation in the treatment of Lennox-Gastaut syndrome. (19699154)
2009
19
Comparison of corpus callosotomy and vagus nerve stimulation in children with Lennox-Gastaut syndrome. (17825516)
2008
20
Vagus nerve stimulation improves severely impaired heart rate variability in a patient with Lennox-Gastaut-Syndrome. (18096413)
2008
21
Lennox gastaut syndrome, review of the literature and a case report. (18541034)
2008
22
Neuromodulation of the centromedian thalamic nuclei in the treatment of generalized seizures and the improvement of the quality of life in patients with Lennox-Gastaut syndrome. (16886984)
2006
23
A comparison of seizure outcome after callosotomy in patients with Lennox-Gastaut syndrome and a positive or negative history for West syndrome. (16884930)
2006
24
Drug therapy in Lennox-Gastaut syndrome. (11993742)
2002
25
Lennox-Gastaut syndrome. (11918467)
2002
26
Topiramate as add-on drug in children, adolescents and young adults with Lennox-Gastaut syndrome: an Italian multicentric study. (12350390)
2002
27
Lennox-Gastaut syndrome. Clinical description and diagnosis. (11993741)
2002
28
Cutis verticis gyrata, mental retardation and Lennox-Gastaut syndrome: a case report. (11731879)
2001
29
Long-term seizure outcome in 74 patients with Lennox-Gastaut syndrome: effects of incorporating MRI head imaging in defining the cryptogenic subgroup. (10756403)
2000
30
Topiramate in Lennox-Gastaut syndrome: open-label treatment of patients completing a randomized controlled trial. Topiramate YL Study Group. (10768308)
2000
31
A pilot study of topiramate in children with Lennox-Gastaut syndrome. (10412513)
1999
32
The efficacy of felbamate as add-on therapy to valproic acid in the Lennox-Gastaut syndrome. (10210023)
1999
33
Can eliminating monosodium glutamate from the diet affect Lennox-Gastaut syndrome? (9710653)
1998
34
Treatment of absence status in the Lennox-Gastaut syndrome with propofol. (9674834)
1998
35
Community-based study of Lennox-Gastaut syndrome. (9184597)
1997
36
Prevalence and descriptive epidemiology of Lennox-Gastaut syndrome among Atlanta children. (9578523)
1997
37
Serologic HLA typing in cryptogenic Lennox-Gastaut syndrome. (8174524)
1994
38
Cerebrospinal fluid examinations in cryptogenic West and Lennox-Gastaut syndrome before and after intravenous immunoglobulin administration. (7957036)
1994
39
Estimation of electrical source generators of diffuse slow spike and wave complex in Lennox-Gastaut syndrome. (8271581)
1993
40
Chromosome fragility in the Lennox-Gastaut epilepsy syndrome. (1458000)
1992
41
Epileptiform EEG activity of the centromedian thalamic nuclei in children with intractable generalized seizures of the Lennox-Gastaut syndrome. (1904342)
1991
42
Multicystic encephalomalacia in an adult case of Lennox-Gastaut syndrome: a case report. (1762241)
1991
43
Lennox-Gastaut syndrome: a developmental study. (2516558)
1989
44
Lennox-Gastaut syndrome. Considerations in its concept and categorization. (3071644)
1988
45
Lennox-gastaut syndrome with and without Dandy-Walker malformation. (3504401)
1987
46
Subacute necrotizing encephalomyelopathy (Leigh disease): report of a case with Lennox-Gastaut syndrome. (4083386)
1985
47
Immunogenetics of the Lennox-Gastaut syndrome: frequency of HL-A antigens and haplotypes in patients and first-degree relatives. (1222746)
1975
48
Tonic status epilepticus precipitated by intravenous benzodiazepine in five patients with Lennox-Gastaut syndrome. (4626571)
1972
49
A special type of attack observed in the Lennox-Gastaut syndrome in adults. (4097478)
1970
50
The Lennox-Gastaut syndrome: a severe type of childhood epilepsy. (4979309)
1969

Genetic Variations for Lennox-Gastaut Syndrome

Expression for genes affiliated with Lennox-Gastaut Syndrome

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

Search GEO for disease gene expression data for Lennox-Gastaut Syndrome.

Pathways for genes affiliated with Lennox-Gastaut Syndrome

Sources:
30KEGG, 52QIAGEN, 38NCBI BioSystems Database
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Pathways related to Lennox-Gastaut Syndrome according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.6MAPK10, POMC
29.6MAPK10, POMC
39.6MAPK10, POMC
4
Hide members
9.1ABAT, MAPK10, SCN1A

Compounds for genes affiliated with Lennox-Gastaut Syndrome

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

Compounds related to Lennox-Gastaut Syndrome according to GeneCards/GeneDecks:

(show all 15)
idCompoundScoreTop Affiliating Genes
1topiramate45 11 2411.8POMC, SCN1A
2etorphine45 29 1111.8POMC, MAPK10
3zonisamide45 1110.8SCN1A, POMC
4dfmo45 60 2911.8MAPK10, POMC
5lamotrigine45 1110.8ABAT, SCN1A
6furosemide45 50 60 29 11 2414.7MAPK10, POMC
7vigabatrin45 29 1111.6ABAT, POMC
8heroin45 50 1111.6ABAT, POMC
9phenytoin45 50 29 1112.5SCN1A, ABAT
10levodopa45 1110.5ABAT, MAPK10
11pyridoxal 5-phosphate459.4ABAT, POMC
12diazepam45 50 29 1112.4ABAT, POMC
132-deoxyglucose45 1110.3MAPK10, POMC
14carbamazepine45 50 1111.3ABAT, POMC, SCN1A
15glutamine458.9ABAT, MAPK10, POMC

GO Terms for genes affiliated with Lennox-Gastaut Syndrome

Sources:
16Gene Ontology
See all sources

Cellular components related to Lennox-Gastaut Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1neuron projectionGO:0430058.9ABAT, AVIL, DCX

Biological processes related to Lennox-Gastaut Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1neuropeptide signaling pathwayGO:0072189.1GPR56, POMC

Products for genes affiliated with Lennox-Gastaut Syndrome

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Sources for Lennox-Gastaut 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