MCID: LNN001
MIFTS: 46

Lennox-Gastaut Syndrome malady

Genetic diseases, Rare diseases, Neuronal diseases categories

Aliases & Classifications for Lennox-Gastaut Syndrome

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Sources:
9Disease Ontology, 41NIH Rare Diseases, 20GeneTests, 21Genetics Home Reference, 42NINDS, 11DISEASES, 43Novoseek, 47Orphanet, 22GTR, 60UMLS, 34MESH via Orphanet, 26ICD10 via Orphanet, 61UMLS via Orphanet
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Lennox-Gastaut Syndrome, Aliases & Descriptions:

Name: Lennox-Gastaut Syndrome 9 41 21 42 11 43 47 60
Epileptic Encephalopathy Lennox-Gastaut Type 41 20 22
Encephalopathy of Childhood 41 60
 
Lennox Syndrome 9 60
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):

47
lennox-gastaut syndrome:
Inheritance: Autosomal dominant,Multigenic/multifactorial,Not applicable; Prevalence: 1-9/1000000 (Europe),1-5/10000 (Europe); Age of onset: Childhood


External Ids:

Disease Ontology9 DOID:0050561
Orphanet47 2382
MESH via Orphanet34 C535500
ICD10 via Orphanet26 G40.4
UMLS via Orphanet61 C0238111

Summaries for Lennox-Gastaut Syndrome

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NINDS:42 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.

NIH Rare Diseases:41 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.

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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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:

31
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:

35
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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Articles related to Lennox-Gastaut Syndrome:

(show top 50)    (show all 266)
idTitleAuthorsYear
1
In response: Lennox-Gastaut syndrome may be a curable, reversible epileptic encephalopathy. (25778756)
2015
2
Lennox-Gastaut syndrome: impact on the caregivers and families of patients. (25336963)
2014
3
Anterior corpus callosotomy in school-aged children with Lennox-Gastaut syndrome: A prospective study. (24912732)
2014
4
Adjunctive levetiracetam treatment in pediatric Lennox-Gastaut syndrome. (25266616)
2014
5
Conceptualizing lennox-gastaut syndrome as a secondary network epilepsy. (25400619)
2014
6
Efficacy and tolerability of add-on Lacosamide in children with Lennox-Gastaut syndrome. (25052288)
2014
7
Withdrawal-related adverse events from clinical trials of clobazam in Lennox-Gastaut syndrome. (24949576)
2014
8
Glycemic index treatment using Japanese foods in a girl with Lennox-Gastaut syndrome. (23583057)
2013
9
Tonic seizures of Lennox-Gastaut syndrome: Periictal single-photon emission computed tomography suggests a corticopontine network. (24117046)
2013
10
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
11
Lennox-Gastaut syndrome in south Iran: electro-clinical manifestations. (22921514)
2012
12
Efficacy of the ketogenic diet in Lennox-Gastaut syndrome: a retrospective review of one institution's experience and summary of the literature. (22443637)
2012
13
Update on the management of Lennox-Gastaut syndrome. (22883278)
2012
14
Rufinamide as an adjuvant treatment in children with Lennox-Gastaut syndrome. (22421185)
2012
15
Clobazam : in patients with Lennox-Gastaut syndrome. (23034582)
2012
16
Post-section recruitment of epileptiform discharges in electrocorticography during callosotomy in 48 patients with Lennox-Gastaut syndrome. (22281384)
2012
17
Long-term safety and efficacy of clobazam for Lennox-Gastaut syndrome: interim results of an open-label extension study. (23141144)
2012
18
Localization of ictal onset zones in Lennox-Gastaut syndrome (LGS) based on information theoretical time delay analysis of intracranial electroencephalography (iEEG). (22071552)
2012
19
Management of seizures in Lennox-Gastaut syndrome. (21351810)
2011
20
Localization of ictal onset zones in Lennox-Gastaut syndrome using directed transfer function method. (22255466)
2011
21
Increased risk of death among children with Lennox-Gastaut syndrome and infantile spasms. (20023065)
2010
22
Medical management of Lennox-Gastaut syndrome. (20158289)
2010
23
Does lacosamide aggravate Lennox-Gastaut syndrome? Report on three consecutive cases. (20970386)
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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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 Suite gene sharing:

(show all 21)
idCompoundScoreTop Affiliating Genes
1topiramate43 49 24 1212.8POMC, SCN1A
2zonisamide43 49 1211.8POMC, SCN1A
3etorphine43 28 1211.8POMC, MAPK10
4dfmo43 28 5911.7MAPK10, POMC
5furosemide43 59 28 49 24 1214.7POMC, MAPK10
6lamotrigine43 49 1211.6ABAT, SCN1A
72-deoxyglucose43 1210.6MAPK10, POMC
8phenytoin43 49 28 1212.5SCN1A, ABAT
9vigabatrin28 43 1211.4POMC, ABAT
10levodopa43 1210.4MAPK10, ABAT
11heroin43 49 1211.3ABAT, POMC
12pyridoxal 5-phosphate439.3POMC, ABAT
13diazepam43 28 49 1212.3POMC, ABAT
14formaldehyde43 2410.1MAPK10, POMC
15valproic acid43 49 24 1212.1ABAT, POMC
16carbamazepine43 49 1211.0POMC, SCN1A, ABAT
17vegf439.0MAPK10, POMC, DCX
18cocaine43 129.9ABAT, POMC
19glutamine438.8ABAT, POMC, MAPK10
20alanine438.6MAPK10, POMC, ABAT
21serine438.3ABAT, 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 Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1neuron projectionGO:00430058.7DCX, ABAT, AVIL

Biological processes related to Lennox-Gastaut Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1neuropeptide signaling pathwayGO:00072189.3POMC, 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
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet