LGS
MCID: LNN001
MIFTS: 53

Lennox-Gastaut Syndrome (LGS) malady

Genetic diseases, Rare diseases, Neuronal diseases categories

Summaries for Lennox-Gastaut Syndrome

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44NIH Rare Diseases, 45NINDS, 48OMIM, 34MalaCards
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NINDS:45 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 west 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 zonisamide have been mentioned in the context of this disorder. Affiliated tissues include brain, eye and cortex, and related mouse phenotypes are no phenotypic analysis and mortality/aging.

NIH Rare Diseases:44 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:48 606369

Aliases & Classifications for Lennox-Gastaut Syndrome

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9Disease Ontology, 44NIH Rare Diseases, 22Genetics Home Reference, 45NINDS, 11DISEASES, 46Novoseek, 50Orphanet, 63UMLS, 21GeneTests, 23GTR, 48OMIM, 37MESH via Orphanet, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet
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Classifications:

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


Characteristics (Orphanet epidemiological data):

50
lennox-gastaut syndrome:
Inheritance: Autosomal dominant,Multigenic/multifactorial,Sporadic; Prevalence: 1-5/10000; Age of onset: Childhood


Aliases & Descriptions:

lennox-gastaut syndrome 9 44 22 45 11 46 50 63
epileptic encephalopathy lennox-gastaut type 44 21 23
lennox syndrome 9 63
childhood epileptic encephalopathy with diffuse slow spikes and waves 22
encephalopathy of childhood 44
lgs 22


External Ids:

Disease Ontology9 DOID:0050561
OMIM48 606369
MESH via Orphanet37 C535500
ICD10 via Orphanet27 G40.4
SNOMED-CT via Orphanet60 230418006
UMLS via Orphanet64 C0238111

Related Diseases for Lennox-Gastaut Syndrome

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18GeneCards, 19GeneDecks
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Graphical network of the top 20 diseases related to Lennox-Gastaut Syndrome:



Diseases related to lennox-gastaut syndrome

Symptoms for Lennox-Gastaut Syndrome

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48OMIM
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Clinical features from OMIM:

606369

Drugs & Therapeutics for Lennox-Gastaut Syndrome

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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-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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21GeneTests, 23GTR
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Genetic tests related to Lennox-Gastaut Syndrome:

id Genetic test Affiliating Genes
1 Epileptic Encephalopathy, Lennox-Gastaut Type21 MAPK10
2 Epileptic Encephalopathy Lennox-Gastaut Type23

Anatomical Context for Lennox-Gastaut Syndrome

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34MalaCards
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MalaCards organs/tissues related to Lennox-Gastaut Syndrome:

34
Brain, Eye, Cortex, Liver, Heart, Thalamus

Animal Models for Lennox-Gastaut Syndrome or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Lennox-Gastaut Syndrome:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00030128.5MAPK10, POMC, DCX, AVIL
2MP:00107688.2AVIL, SCN1A, DCX, POMC, CHD2
3MP:00107718.1CHD2, POMC, GPR56, AVIL
4MP:00053847.9MAPK10, CHD2, POMC, GPR56, AVIL
5MP:00036317.7DCX, SCN1A, MAPK10, GPR56, AVIL, POMC
6MP:00053867.3CHD2, MAPK10, DCX, SCN1A, GPR56, AVIL

Publications for Lennox-Gastaut Syndrome

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53PubMed
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Articles related to Lennox-Gastaut Syndrome:

(show top 50)    (show all 240)
idTitleAuthorsYear
1
Glycemic index treatment using Japanese foods in a girl with Lennox-Gastaut syndrome. (23583057)
2013
2
Tonic seizures of Lennox-Gastaut syndrome: Periictal single-photon emission computed tomography suggests a corticopontine network. (24117046)
2013
3
Whole-exome sequencing identifies a variant of the mitochondrial MT-ND1 gene associated with epileptic encephalopathy: west syndrome evolving to Lennox-Gastaut syndrome. (24105702)
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
Update on the management of Lennox-Gastaut syndrome. (22883278)
2012
7
Rufinamide as an adjuvant treatment in children with Lennox-Gastaut syndrome. (22421185)
2012
8
Clobazam : in patients with Lennox-Gastaut syndrome. (23034582)
2012
9
Post-section recruitment of epileptiform discharges in electrocorticography during callosotomy in 48 patients with Lennox-Gastaut syndrome. (22281384)
2012
10
Long-term safety and efficacy of clobazam for Lennox-Gastaut syndrome: interim results of an open-label extension study. (23141144)
2012
11
Localization of ictal onset zones in Lennox-Gastaut syndrome (LGS) based on information theoretical time delay analysis of intracranial electroencephalography (iEEG). (22071552)
2012
12
Surgical treatment of patients with Lennox-Gastaut syndrome phenotype. (22629163)
2012
13
Management of seizures in Lennox-Gastaut syndrome. (21351810)
2011
14
Localization of ictal onset zones in Lennox-Gastaut syndrome using directed transfer function method. (22255466)
2011
15
Increased risk of death among children with Lennox-Gastaut syndrome and infantile spasms. (20023065)
2010
16
Medical management of Lennox-Gastaut syndrome. (20158289)
2010
17
Does lacosamide aggravate Lennox-Gastaut syndrome? Report on three consecutive cases. (20970386)
2010
18
Supporting the recommended paediatric dosing regimen for rufinamide in Lennox-Gastaut syndrome using clinical trial simulation. (20084538)
2010
19
SCN1A mutation screening in adult patients with Lennox-Gastaut syndrome features. (19782004)
2009
20
Treatment of Lennox-Gastaut Syndrome (LGS). (19211283)
2009
21
Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. (19081517)
2009
22
Radiosurgical posterior corpus callosotomy in a child with Lennox-Gastaut syndrome. Case report. (17465368)
2007
23
Delayed response of seizures with vagus nerve stimulation in Lennox-Gastaut syndrome. (15505192)
2004
24
Nitrazepam for the treatment of Lennox-Gastaut syndrome. (12657414)
2003
25
Correction to infantile spasms and Lennox-Gastaut syndrome. (12822828)
2003
26
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
27
Drug therapy in Lennox-Gastaut syndrome. (11993742)
2002
28
Infantile spasms and Lennox-Gastaut syndrome. (11952036)
2002
29
Eating seizures in Lennox-Gastaut syndrome. (11244278)
2001
30
Cutis verticis gyrata, mental retardation and Lennox-Gastaut syndrome: a case report. (11731879)
2001
31
Long-term seizure outcome in 74 patients with Lennox-Gastaut syndrome: effects of incorporating MRI head imaging in defining the cryptogenic subgroup. (10756403)
2000
32
Vagus nerve stimulation treatment for Lennox-Gastaut syndrome. (10961788)
2000
33
Can eliminating monosodium glutamate from the diet affect Lennox-Gastaut syndrome? (9710653)
1998
34
Need for electroencephalogram video confirmation of atypical absence seizures in children with Lennox-Gastaut syndrome. (9796756)
1998
35
Lennox-Gastaut syndrome. (9390695)
1997
36
Temporo-spacial correlations between cortical and subcortical EEG spike-wave complexes of the Idiopathic Lennox-Gastaut syndrome. (9711757)
1997
37
Lamotrigine for generalized seizures associated with the Lennox-Gastaut syndrome. Lamictal Lennox-Gastaut Study Group. (9400037)
1997
38
Case study. A child with Lennox-Gastaut syndrome. (8788700)
1996
39
Evolution of Lennox-Gastaut syndrome: a long-term longitudinal study. (8681913)
1996
40
Multifocal independent Spike syndrome: relationship to hypsarrhythmia and the slow spike-wave (Lennox-Gastaut) syndrome. (7882540)
1995
41
Efficacy of felbamate in childhood epileptic encephalopathy (Lennox-Gastaut syndrome). The Felbamate Study Group in Lennox-Gastaut Syndrome. (8347179)
1993
42
Lennox-Gastaut syndrome--clinical seizure outcome and social prognosis. (2124295)
1990
43
Results of corpus callosum section in two patients with Lennox-Gastaut syndrome. (2516562)
1989
44
Depth EEG studies in the Lennox-Gastaut syndrome. (3117442)
1987
45
Lennox-gastaut syndrome with and without Dandy-Walker malformation. (3504401)
1987
46
Status epilepticus within the Lennox-Gastaut syndrome: clinical characteristics and management. (3109788)
1987
47
West syndrome evolving into the Lennox-Gastaut syndrome. (6697547)
1984
48
Secondary late-onset Lennox-Gastaut syndrome: a critical view. (6466153)
1984
49
The Lennox-Gastaut syndrome: comments on the syndrome's terminology and nosological position amongst the secondary generalized epilepsies of childhood. (6811252)
1982
50
Positron emission tomography in two cases of childhood epileptic encephalopathy (Lennox-Gastaut syndrome). (6981784)
1982

Variations for Lennox-Gastaut Syndrome

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Expression for genes affiliated with Lennox-Gastaut Syndrome

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2BioGPS, 16Gene 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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51PathCards, 31KEGG, 54QIAGEN, 39NCBI BioSystems Database
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Compounds for genes affiliated with Lennox-Gastaut Syndrome

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46Novoseek, 52PharmGKB, 25HMDB, 12DrugBank, 30IUPHAR, 62Tocris Bioscience
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Compounds related to Lennox-Gastaut Syndrome according to GeneCards/GeneDecks:

(show all 21)
idCompoundScoreTop Affiliating Genes
1topiramate46 52 25 1212.8POMC, SCN1A
2zonisamide46 52 1211.8SCN1A, POMC
3etorphine46 30 1211.8POMC, MAPK10
4dfmo46 30 6211.7MAPK10, POMC
5furosemide46 62 30 52 25 1214.7MAPK10, POMC
6lamotrigine46 52 1211.6SCN1A, ABAT
72-deoxyglucose46 1210.6MAPK10, POMC
8phenytoin46 52 30 1212.5ABAT, SCN1A
9vigabatrin30 46 1211.4POMC, ABAT
10levodopa46 1210.4ABAT, MAPK10
11heroin46 52 1211.3ABAT, POMC
12pyridoxal 5-phosphate469.3ABAT, POMC
13diazepam46 30 52 1212.3POMC, ABAT
14formaldehyde46 2510.1POMC, MAPK10
15valproic acid46 52 25 1212.1POMC, ABAT
16carbamazepine46 52 1211.0POMC, ABAT, SCN1A
17vegf469.0MAPK10, POMC, DCX
18cocaine46 129.9POMC, ABAT
19glutamine468.8ABAT, POMC, MAPK10
20alanine468.6MAPK10, ABAT, POMC
21serine468.3MAPK10, POMC, DCX, ABAT

GO Terms for genes affiliated with Lennox-Gastaut Syndrome

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17Gene Ontology
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Cellular components related to Lennox-Gastaut Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1neuron projectionGO:0430058.7DCX, ABAT, AVIL

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

idNameGO IDScoreTop Affiliating Genes
1neuropeptide signaling pathwayGO:0072189.3POMC, GPR56

Products for genes affiliated with Lennox-Gastaut Syndrome

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Sources for Lennox-Gastaut Syndrome

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet