LGS
MCID: LNN001
MIFTS: 54

Lennox-Gastaut Syndrome (LGS) malady

Genetic diseases, Rare diseases, Neuronal diseases categories
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Summaries for Lennox-Gastaut Syndrome

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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 based summary: Lennox-Gastaut Syndrome, also known as epileptic encephalopathy lennox-gastaut type, is related to west syndrome and blindness. 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.

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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Sources:
8Disease Ontology, 42NIH Rare Diseases, 21Genetics Home Reference, 43NINDS, 10DISEASES, 44Novoseek, 48Orphanet, 62UMLS, 20GeneTests, 22GTR, 46OMIM, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Lennox-Gastaut Syndrome, Aliases & Descriptions:

Name: Lennox-Gastaut Syndrome 8 42 21 43 10 44 48 62
Epileptic Encephalopathy Lennox-Gastaut Type 42 20 22
Encephalopathy of Childhood 42 62
 
Lennox Syndrome 8 62
Childhood Epileptic Encephalopathy with Diffuse Slow Spikes and Waves 21
Lgs 21


Classifications:

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


Characteristics (Orphanet epidemiological data):

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


External Ids:

Disease Ontology8 DOID:0050561
OMIM46 606369
MESH via Orphanet35 C535500
ICD10 via Orphanet26 G40.4
UMLS via Orphanet63 C0238111

Related Diseases for Lennox-Gastaut Syndrome

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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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Clinical features from OMIM:

606369

Drugs & Therapeutics for Lennox-Gastaut Syndrome

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Drug clinical trials:

Search ClinicalTrials for Lennox-Gastaut Syndrome

Search NIH Clinical Center for Lennox-Gastaut Syndrome

Genetic Tests for Lennox-Gastaut Syndrome

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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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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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MGI Mouse Phenotypes related to Lennox-Gastaut Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00030128.5AVIL, DCX, POMC, MAPK10
2MP:00107688.2CHD2, POMC, DCX, SCN1A, AVIL
3MP:00107718.1AVIL, GPR56, POMC, CHD2
4MP:00053847.9MAPK10, CHD2, POMC, GPR56, AVIL
5MP:00036317.7AVIL, GPR56, SCN1A, DCX, POMC, MAPK10
6MP:00053867.3MAPK10, AVIL, GPR56, SCN1A, DCX, POMC

Publications for Lennox-Gastaut Syndrome

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Articles related to Lennox-Gastaut Syndrome:

(show top 50)    (show all 262)
idTitleAuthorsYear
1
Lennox-Gastaut syndrome: impact on the caregivers and families of patients. (25336963)
2014
2
Anterior corpus callosotomy in school-aged children with Lennox-Gastaut syndrome: A prospective study. (24912732)
2014
3
Adjunctive levetiracetam treatment in pediatric Lennox-Gastaut syndrome. (25266616)
2014
4
Conceptualizing lennox-gastaut syndrome as a secondary network epilepsy. (25400619)
2014
5
Efficacy and tolerability of add-on Lacosamide in children with Lennox-Gastaut syndrome. (25052288)
2014
6
Withdrawal-related adverse events from clinical trials of clobazam in Lennox-Gastaut syndrome. (24949576)
2014
7
Glycemic index treatment using Japanese foods in a girl with Lennox-Gastaut syndrome. (23583057)
2013
8
Tonic seizures of Lennox-Gastaut syndrome: Periictal single-photon emission computed tomography suggests a corticopontine network. (24117046)
2013
9
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
10
Lennox-Gastaut syndrome in south Iran: electro-clinical manifestations. (22921514)
2012
11
Efficacy of the ketogenic diet in Lennox-Gastaut syndrome: a retrospective review of one institution's experience and summary of the literature. (22443637)
2012
12
Update on the management of Lennox-Gastaut syndrome. (22883278)
2012
13
Rufinamide as an adjuvant treatment in children with Lennox-Gastaut syndrome. (22421185)
2012
14
Clobazam : in patients with Lennox-Gastaut syndrome. (23034582)
2012
15
Post-section recruitment of epileptiform discharges in electrocorticography during callosotomy in 48 patients with Lennox-Gastaut syndrome. (22281384)
2012
16
Long-term safety and efficacy of clobazam for Lennox-Gastaut syndrome: interim results of an open-label extension study. (23141144)
2012
17
Localization of ictal onset zones in Lennox-Gastaut syndrome (LGS) based on information theoretical time delay analysis of intracranial electroencephalography (iEEG). (22071552)
2012
18
Management of seizures in Lennox-Gastaut syndrome. (21351810)
2011
19
Localization of ictal onset zones in Lennox-Gastaut syndrome using directed transfer function method. (22255466)
2011
20
Increased risk of death among children with Lennox-Gastaut syndrome and infantile spasms. (20023065)
2010
21
Medical management of Lennox-Gastaut syndrome. (20158289)
2010
22
Does lacosamide aggravate Lennox-Gastaut syndrome? Report on three consecutive cases. (20970386)
2010
23
Supporting the recommended paediatric dosing regimen for rufinamide in Lennox-Gastaut syndrome using clinical trial simulation. (20084538)
2010
24
SCN1A mutation screening in adult patients with Lennox-Gastaut syndrome features. (19782004)
2009
25
Treatment of Lennox-Gastaut Syndrome (LGS). (19211283)
2009
26
Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. (19081517)
2009
27
Radiosurgical posterior corpus callosotomy in a child with Lennox-Gastaut syndrome. Case report. (17465368)
2007
28
Delayed response of seizures with vagus nerve stimulation in Lennox-Gastaut syndrome. (15505192)
2004
29
Nitrazepam for the treatment of Lennox-Gastaut syndrome. (12657414)
2003
30
Correction to infantile spasms and Lennox-Gastaut syndrome. (12822828)
2003
31
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
32
Drug therapy in Lennox-Gastaut syndrome. (11993742)
2002
33
Infantile spasms and Lennox-Gastaut syndrome. (11952036)
2002
34
Eating seizures in Lennox-Gastaut syndrome. (11244278)
2001
35
Cutis verticis gyrata, mental retardation and Lennox-Gastaut syndrome: a case report. (11731879)
2001
36
Long-term seizure outcome in 74 patients with Lennox-Gastaut syndrome: effects of incorporating MRI head imaging in defining the cryptogenic subgroup. (10756403)
2000
37
Vagus nerve stimulation treatment for Lennox-Gastaut syndrome. (10961788)
2000
38
Can eliminating monosodium glutamate from the diet affect Lennox-Gastaut syndrome? (9710653)
1998
39
Lennox-Gastaut syndrome. (9390695)
1997
40
Temporo-spacial correlations between cortical and subcortical EEG spike-wave complexes of the Idiopathic Lennox-Gastaut syndrome. (9711757)
1997
41
Case study. A child with Lennox-Gastaut syndrome. (8788700)
1996
42
Evolution of Lennox-Gastaut syndrome: a long-term longitudinal study. (8681913)
1996
43
Multifocal independent Spike syndrome: relationship to hypsarrhythmia and the slow spike-wave (Lennox-Gastaut) syndrome. (7882540)
1995
44
Efficacy of felbamate in childhood epileptic encephalopathy (Lennox-Gastaut syndrome). The Felbamate Study Group in Lennox-Gastaut Syndrome. (8347179)
1993
45
Lennox-Gastaut syndrome--clinical seizure outcome and social prognosis. (2124295)
1990
46
Results of corpus callosum section in two patients with Lennox-Gastaut syndrome. (2516562)
1989
47
Depth EEG studies in the Lennox-Gastaut syndrome. (3117442)
1987
48
Lennox-gastaut syndrome with and without Dandy-Walker malformation. (3504401)
1987
49
West syndrome evolving into the Lennox-Gastaut syndrome. (6697547)
1984
50
Secondary late-onset Lennox-Gastaut syndrome: a critical view. (6466153)
1984

Variations for Lennox-Gastaut Syndrome

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Expression for genes affiliated with Lennox-Gastaut Syndrome

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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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Compounds for genes affiliated with Lennox-Gastaut Syndrome

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

(show all 21)
idCompoundScoreTop Affiliating Genes
1topiramate44 50 24 1112.8POMC, SCN1A
2zonisamide44 50 1111.8POMC, SCN1A
3etorphine44 28 1111.8POMC, MAPK10
4dfmo44 28 6111.7MAPK10, POMC
5furosemide44 61 28 50 24 1114.7POMC, MAPK10
6lamotrigine44 50 1111.6ABAT, SCN1A
72-deoxyglucose44 1110.6MAPK10, POMC
8phenytoin44 50 28 1112.5SCN1A, ABAT
9vigabatrin28 44 1111.4POMC, ABAT
10levodopa44 1110.4MAPK10, ABAT
11heroin44 50 1111.3ABAT, POMC
12pyridoxal 5-phosphate449.3POMC, ABAT
13diazepam44 28 50 1112.3POMC, ABAT
14formaldehyde44 2410.1MAPK10, POMC
15valproic acid44 50 24 1112.1ABAT, POMC
16carbamazepine44 50 1111.0POMC, SCN1A, ABAT
17vegf449.0MAPK10, POMC, DCX
18cocaine44 119.9ABAT, POMC
19glutamine448.8ABAT, POMC, MAPK10
20alanine448.6MAPK10, POMC, ABAT
21serine448.3ABAT, DCX, POMC, MAPK10

GO Terms for genes affiliated with Lennox-Gastaut Syndrome

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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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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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet