LGS
MCID: LNN001
MIFTS: 49

Lennox-Gastaut Syndrome (LGS) malady

Neuronal diseases category

Summaries for Lennox-Gastaut Syndrome

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21Genetics Home Reference, 42NIH Rare Diseases, 43NINDS, 46OMIM, 32MalaCards
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NINDS:43 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 epilepsy syndrome and status epilepticus. 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, eye and cortex, and related mouse phenotypes are nervous system and behavior/neurological.

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.

NIH Rare Diseases:42 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

Description from OMIM:46 606369

Aliases & Classifications for Lennox-Gastaut Syndrome

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8Disease Ontology, 42NIH Rare Diseases, 20GeneTests, 22GTR, 21Genetics Home Reference, 43NINDS, 10DISEASES, 44Novoseek, 48Orphanet, 60UMLS, 46OMIM, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
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Classifications:

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


Characteristics (Orphanet epidemiological data):

48
lennox-gastaut syndrome:
Inheritance: Autosomal dominant,Sporadic; Prevalence: 1-5/10000; Age of onset: Childhood


Aliases & Descriptions:

lennox-gastaut syndrome 8 42 21 43 10 44 48 60
epileptic encephalopathy lennox-gastaut type 42 20 22
lennox syndrome 8 60
childhood epileptic encephalopathy with diffuse slow spikes and waves 21
encephalopathy of childhood 42
lgs 21


External Ids:

Disease Ontology8 DOID:0050561
OMIM46 606369
MESH via Orphanet35 C535500
ICD10 via Orphanet26 G40.4
SNOMED-CT via Orphanet57 230418006
UMLS via Orphanet61 C0238111

Related Diseases for Lennox-Gastaut Syndrome

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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

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46OMIM
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Clinical features from OMIM:

606369

Drugs & Therapeutics for Lennox-Gastaut Syndrome

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-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

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20GeneTests, 22GTR
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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

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32MalaCards
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MalaCards organs/tissues related to Lennox-Gastaut Syndrome:

32
Brain, Eye, Cortex, Heart, Liver, Thalamus

Animal Models for Lennox-Gastaut Syndrome or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Lennox-Gastaut Syndrome:

36
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

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50PubMed
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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
Long-term use of clobazam in Lennox-Gastaut syndrome: experience in a single tertiary epilepsy center. (23334068)
2013
3
Networks underlying paroxysmal fast activity and slow spike and wave in Lennox-Gastaut syndrome. (23864316)
2013
4
Cost-effectiveness analysis of antiepileptic drugs in the treatment of Lennox-Gastaut syndrome. (23973644)
2013
5
Localization of epileptogenic zones in Lennox-Gastaut syndrome using frequency domain source imaging of intracranial electroencephalography: a preliminary investigation. (23354062)
2013
6
Vagus nerve stimulation vs. corpus callosotomy in the treatment of Lennox-Gastaut syndrome: a meta-analysis. (23068970)
2013
7
Surgical treatment of patients with Lennox-Gastaut syndrome phenotype. (22629163)
2012
8
The ketogenic diet in the treatment of Lennox-Gastaut syndrome. (22443688)
2012
9
Clobazam as an adjunctive therapy in treating seizures associated with Lennox-Gastaut syndrome. (22128252)
2011
10
Evolution and management of Lennox-Gastaut syndrome through adolescence and into adulthood: are seizures always the primary issue? (21669559)
2011
11
Localization of ictal onset zones in Lennox-Gastaut syndrome using directional connectivity analysis of intracranial electroencephalography. (21515079)
2011
12
Adjunctive rufinamide in Lennox-Gastaut syndrome: a long-term, open-label extension study. (20199521)
2010
13
Treating Lennox-Gastaut syndrome in epileptic pediatric patients with third-generation rufinamide. (20957124)
2010
14
Late-onset Lennox-Gastaut syndrome in a patient with 15q11.2-q13.1 duplication. (19396834)
2009
15
Bilateral frontoparietal polymicrogyria, Lennox-Gastaut syndrome, and GPR56 gene mutations. (19016831)
2009
16
Clobazam in the treatment of Lennox-Gastaut syndrome. (19170737)
2009
17
Spectral analysis of EEG gamma rhythms associated with tonic seizures in Lennox-Gastaut syndrome. (19375286)
2009
18
Neuropsychological findings: myoclonic astatic epilepsy (MAE) and Lennox-Gastaut syndrome (LGS). (17105463)
2006
19
Toxic hepatitis in a case of Angelman syndrome associated with Lennox-Gastaut syndrome. (15517829)
2004
20
Nitrazepam for the treatment of Lennox-Gastaut syndrome. (12657414)
2003
21
Treatment of Lennox-Gastaut syndrome. (12917958)
2003
22
Temporo-spatial correlations between scalp and centromedian thalamic EEG activities of stage II slow wave sleep in patients with generalized seizures of the cryptogenic Lennox-Gastaut syndrome. (11801421)
2002
23
Long-term electro-clinical changes in the Lennox-Gastaut syndrome before, during, and after the slow spike-wave pattern. (11795207)
2002
24
Eating seizures in Lennox-Gastaut syndrome. (11244278)
2001
25
Cutis verticis gyrata, mental retardation and Lennox-Gastaut syndrome: a case report. (11731879)
2001
26
Sleep in Lennox-Gastaut syndrome: the role of the cyclic alternating pattern (CAP) in the gate control of clinical seizures and generalized polyspikes. (11518625)
2001
27
Long-term seizure outcome in 74 patients with Lennox-Gastaut syndrome: effects of incorporating MRI head imaging in defining the cryptogenic subgroup. (10756403)
2000
28
Vagus nerve stimulation treatment for Lennox-Gastaut syndrome. (10961788)
2000
29
A risk-benefit assessment of therapies for Lennox-Gastaut syndrome. (10877040)
2000
30
Nonconvulsive status epilepticus--a possible cause of mental retardation in patients with Lennox-Gastaut syndrome. (11071139)
2000
31
Temporo-spacial correlations between cortical and subcortical EEG spike-wave complexes of the Idiopathic Lennox-Gastaut syndrome. (9711757)
1997
32
Case study. A child with Lennox-Gastaut syndrome. (8788700)
1996
33
Felbamate in the treatment of Lennox-Gastaut syndrome. (8039473)
1994
34
Lennox-Gastaut syndrome in the elderly? (7813093)
1994
35
Rectal administration of felbamate in a child with Lennox-Gastaut syndrome. (7936261)
1994
36
Gamma-vinyl-GABA (vigabatrin) in the therapy of Lennox-Gastaut syndrome: an open study. (7925171)
1994
37
Electroclinical seizures in Lennox-Gastaut syndrome. (8422843)
1993
38
The Lennox-Gastaut syndrome. (8243377)
1993
39
Felbamate in the treatment of Lennox-Gastaut syndrome: results of a 12-month open-label study following a randomized clinical trial. (8243374)
1993
40
Partial corpus callosotomy beneficial for Lennox-Gastaut syndrome--report of two cases. (1720213)
1991
41
The Lennox-Gastaut syndrome. (2499428)
1989
42
Lennox-Gastaut syndrome in association with tuberous sclerosis. (2630483)
1989
43
Status epilepticus within the Lennox-Gastaut syndrome: clinical characteristics and management. (3109788)
1987
44
Overnight polygraphic study of Lennox-Gastaut syndrome. (3445924)
1987
45
The Lennox Gastaut syndrome. (3105523)
1986
46
West syndrome evolving into the Lennox-Gastaut syndrome. (6697547)
1984
47
Secondary late-onset Lennox-Gastaut syndrome: a critical view. (6466153)
1984
48
Positron emission tomography in two cases of childhood epileptic encephalopathy (Lennox-Gastaut syndrome). (6981784)
1982
49
Linear nevus sebaceus syndrome. Report of a case with Lennox-Gastaut syndrome following infantile spasms. (6972208)
1981
50
Lennox-Gastaut syndrome and computerized axial tomography findings. (303994)
1977

Genetic Variations for Lennox-Gastaut Syndrome

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Expression for genes affiliated with Lennox-Gastaut Syndrome

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1BioGPS, 15Gene Expression Omnibus DataSets
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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

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29KEGG, 51QIAGEN, 37NCBI BioSystems Database
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Pathways related to Lennox-Gastaut Syndrome according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5MAPK10, POMC
29.5MAPK10, POMC
39.5MAPK10, POMC
4
Hide members
9.1ABAT, MAPK10, SCN1A

Compounds for genes affiliated with Lennox-Gastaut Syndrome

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

(show all 15)
idCompoundScoreTop Affiliating Genes
1topiramate44 11 2411.8POMC, SCN1A
2etorphine44 28 1111.8POMC, MAPK10
3zonisamide44 1110.8SCN1A, POMC
4lamotrigine44 1110.8ABAT, SCN1A
5dfmo44 59 2811.7MAPK10, POMC
6furosemide44 49 59 28 11 2414.7MAPK10, POMC
7vigabatrin44 28 1111.6ABAT, POMC
8heroin44 49 1111.6ABAT, POMC
9phenytoin44 49 28 1112.5SCN1A, ABAT
10pyridoxal 5-phosphate449.4ABAT, POMC
11levodopa44 1110.4ABAT, MAPK10
12diazepam44 49 28 1112.4ABAT, POMC
13carbamazepine44 49 1111.3ABAT, POMC, SCN1A
142-deoxyglucose44 1110.2MAPK10, POMC
15glutamine448.8ABAT, MAPK10, POMC

GO Terms for genes affiliated with Lennox-Gastaut Syndrome

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16Gene Ontology
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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

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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