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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Sources:
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, Liver, Heart, 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
Anesthetic management of a patient with Lennox-Gastaut syndrome with intractable epilepsy -a case report-. (24228152)
2013
2
Lennox-Gastaut syndrome and epilepsy with myoclonic-astatic seizures. (23622212)
2013
3
Efficacy of the ketogenic diet in Lennox-Gastaut syndrome: a retrospective review of one institution's experience and summary of the literature. (22443637)
2012
4
Clobazam : in patients with Lennox-Gastaut syndrome. (23034582)
2012
5
lennox-Gastaut syndrome: an updateon treatment. (23227575)
2012
6
Lacosamide in Lennox-Gastaut syndrome: case report. (22592511)
2012
7
Introduction: Transitioning care for adults with Lennox-Gastaut syndrome: challenges and promises. (21790559)
2011
8
Does lacosamide aggravate Lennox-Gastaut syndrome? Report on three consecutive cases. (20970386)
2010
9
Lennox-Gastaut syndrome (LGS): development of conceptual models of health-related quality of life (HRQL) for caregivers and children. (19948417)
2010
10
Lennox-Gastaut syndrome and idiopathic intracranial hypertension. (20542434)
2010
11
Rufinamide in children and adults with Lennox-Gastaut syndrome: first Italian multicenter experience. (20888268)
2010
12
Cost-utility analysis of rufinamide versus topiramate and lamotrigine for the treatment of children with Lennox-Gastaut Syndrome in the United Kingdom. (19942457)
2010
13
SCN1A mutation screening in adult patients with Lennox-Gastaut syndrome features. (19782004)
2009
14
Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. (19081517)
2009
15
Treatment of Lennox-Gastaut syndrome. (19588340)
2009
16
Adopting an orphan drug: rufinamide for Lennox-Gastaut syndrome. (19471615)
2009
17
Comparison of corpus callosotomy and vagus nerve stimulation in children with Lennox-Gastaut syndrome. (17825516)
2008
18
Corpus callosotomy in a patient of hemimegalencephaly and Lennox-Gastaut syndrome. (18439776)
2008
19
Radiosurgical posterior corpus callosotomy in a child with Lennox-Gastaut syndrome. Case report. (17465368)
2007
20
Localized retroauricular morphea in a boy with Lennox-Gastaut syndrome and hypospadias. (17461825)
2007
21
A common variable immunodeficient patient who developed acute disseminated encephalomyelitis followed by the Lennox-Gastaut syndrome. (15943601)
2005
22
Lennox-Gastaut syndrome (childhood epileptic encephalopathy). (14734932)
2003
23
Infantile spasms and Lennox-Gastaut syndrome. (11952036)
2002
24
Pathogenesis of Lennox-Gastaut syndrome: considerations and hypotheses. (11844713)
2001
25
Vagus nerve stimulation and Lennox-Gastaut syndrome: a review of the literature and data from the VNS patient registry. (15489825)
2001
26
Effects of 6 months of treatment with vagus nerve stimulation on behavior in children with lennox-gastaut syndrome in an open clinical and nonrandomized study. (12609211)
2001
27
Long-term follow-up study of Lennox-Gastaut syndrome in patients with severe motor and intellectual disabilities: with special reference to the problem of dysphagia. (11437619)
2001
28
Occurrence, outcome, and prognostic factors of infantile spasms and Lennox-Gastaut syndrome. (10080506)
1999
29
Lennox-Gastaut syndrome after a further attenuated live measles vaccination. (10598060)
1999
30
A double-blind, randomized trial of topiramate in Lennox-Gastaut syndrome. Topiramate YL Study Group. (10371538)
1999
31
Immunohistochemical analysis in a case of idiopathic Lennox-Gastaut syndrome. (10580554)
1999
32
Can eliminating monosodium glutamate from the diet affect Lennox-Gastaut syndrome? (9710653)
1998
33
Lennox-Gastaut syndrome. (9390695)
1997
34
Evolution of Lennox-Gastaut syndrome: a long-term longitudinal study. (8681913)
1996
35
High-dose intravenous immunoglobulin treatment in cryptogenic West and Lennox-Gastaut syndrome; an add-on study. (7813537)
1994
36
Efficacy of felbamate in childhood epileptic encephalopathy (Lennox-Gastaut syndrome). The Felbamate Study Group in Lennox-Gastaut Syndrome. (8347179)
1993
37
Felbamate therapy in the Lennox-Gastaut syndrome. (8487814)
1993
38
Electroencephalographic and magnetic resonance correlations in children with intractable seizures of Lennox-Gastaut syndrome and epilepsia partialis continua. (8453934)
1993
39
Lamotrigine as an add-on drug in the management of Lennox-Gastaut syndrome. (1490494)
1992
40
Double cortex. A neuronal migration anomaly as a possible cause of Lennox-Gastaut syndrome. (1728265)
1992
41
Laminar heterotopic grey matter (double cortex) in a patient with late onset Lennox-Gastaut syndrome. (1441556)
1992
42
The Lennox-Gastaut syndrome: electroencephalographic characteristics, clinical correlates, and follow-up studies. (1395056)
1992
43
Depth EEG studies in the Lennox-Gastaut syndrome. (3117442)
1987
44
Lennox-Gastaut syndrome and positron emission tomography. (3103527)
1987
45
The Lennox-Gastaut syndrome and its frontiers. (3742825)
1986
46
Adult Lennox Gastaut syndrome: patients with large focal structural lesions. (3843211)
1985
47
Adult Lennox Gastaut syndrome: features and diagnostic problems. (3843228)
1985
48
The Lennox-Gastaut syndrome: a personal study. (6811253)
1982
49
A case of Lennox-Gastaut syndrome successfully treated by removal of a parietotemporal astrocytoma. (115678)
1979
50
The electroencephalographic study on adult-type Lennox-Gastaut syndrome. (992514)
1976

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