MCID: LNN001
MIFTS: 59

Lennox-Gastaut Syndrome

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Lennox-Gastaut Syndrome

MalaCards integrated aliases for Lennox-Gastaut Syndrome:

Name: Lennox-Gastaut Syndrome 12 49 24 50 55 36 51 14 69
Epileptic Encephalopathy Lennox-Gastaut Type 49 28
Lennox Syndrome 12 69
Childhood Epileptic Encephalopathy with Diffuse Slow Spikes and Waves 24
Epileptic Encephalopathy, Lennox-Gastaut Type 13
Encephalopathy of Childhood 49
Lgs 24

Characteristics:

Orphanet epidemiological data:

55
lennox-gastaut syndrome
Inheritance: Autosomal dominant,Multigenic/multifactorial,Not applicable; Prevalence: 1-9/1000000 (Europe),1-5/10000 (Europe); Age of onset: Childhood;

Classifications:

Orphanet: 55  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:0050561
Orphanet 55 ORPHA2382
MESH via Orphanet 42 C535500
UMLS via Orphanet 70 C0238111
ICD10 via Orphanet 33 G40.4
KEGG 36 H01813

Summaries for Lennox-Gastaut Syndrome

NINDS : 50 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 myoclonic astatic epilepsy and epileptic encephalopathy, early infantile, 15, and has symptoms including intellectual disability, encephalopathy and eeg with focal sharp slow waves. An important gene associated with Lennox-Gastaut Syndrome is SCN1A (Sodium Voltage-Gated Channel Alpha Subunit 1), and among its related pathways/superpathways are Neuroscience and GABAergic synapse. The drugs Melatonin and Antioxidants have been mentioned in the context of this disorder. Affiliated tissues include brain, eye and cortex, and related phenotypes are behavior/neurological and mortality/aging

Genetics Home Reference : 24 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 : 49 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

Related Diseases for Lennox-Gastaut Syndrome

Diseases related to Lennox-Gastaut Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 63)
# Related Disease Score Top Affiliating Genes
1 myoclonic astatic epilepsy 33.0 SCN1A SLC2A1
2 epileptic encephalopathy, early infantile, 15 31.5 AKT3 KCNQ2 SCN1A ST3GAL3 STXBP1
3 epilepsy 30.7 GABRA1 GABRB3 KCNQ2 SCN1A SLC2A1 TBC1D24
4 focal epilepsy 30.5 SCN1A SLC2A1 TBC1D24
5 west syndrome 29.8 ALG13 KCNQ2 MT-ND1 POMC SCN1A ST3GAL3
6 epileptic encephalopathy, early infantile, 6 29.4 CHD2 GABRA1 GABRB3 KCNQ2 SCN1A STXBP1
7 neurodegeneration with brain iron accumulation 5 11.8
8 trichorhinophalangeal syndrome, type ii 11.3
9 scn1a-related seizure disorders 11.3
10 cdkl5-related disorder 11.3
11 scn2a related disorders 11.3
12 encephalopathy, acute, infection-induced 4 11.3
13 exostoses, multiple, type i 10.9
14 lymphomatoid granulomatosis 10.9
15 encephalopathy 10.6
16 arachnoiditis 10.4 MT-ND1 POMC
17 status epilepticus 10.4
18 landau-kleffner syndrome 10.4 POMC TBC1D24
19 epileptic encephalopathy, early infantile, 1 10.3 KCNQ2 TBC1D24
20 aging 10.3
21 cerebritis 10.3
22 infantile epileptic encephalopathy 10.3 POMC SCN1A STXBP1
23 epilepsy with generalized tonic-clonic seizures 10.3 SCN1A TBC1D24
24 visual epilepsy 10.2 KCNQ2 SCN1A SLC2A1
25 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.2
26 hypothalamic hamartomas 10.2
27 tuberous sclerosis 10.2
28 seizure disorder 10.2 KCNQ2 SCN1A SLC2A1
29 malignant migrating partial seizures of infancy 10.2 SCN1A TBC1D24
30 angelman syndrome 10.1
31 cataract 32, multiple types 10.1
32 myoclonic epilepsy of unverricht and lundborg 10.1
33 polymicrogyria, bilateral frontoparietal 10.1
34 polymicrogyria, bilateral temporooccipital 10.1
35 alacrima, achalasia, and mental retardation syndrome 10.1
36 common variable immunodeficiency 10.1
37 hepatitis 10.1
38 hypospadias 10.1
39 encephalomalacia 10.1
40 astrocytoma 10.1
41 acute disseminated encephalomyelitis 10.1
42 neuronitis 10.1
43 measles 10.1
44 tyrosinemia 10.1
45 intracranial hypertension 10.1
46 polymicrogyria 10.1
47 cutis verticis gyrata 10.1
48 hemimegalencephaly 10.1
49 pachygyria 10.1
50 cerebral atrophy 10.1

Graphical network of the top 20 diseases related to Lennox-Gastaut Syndrome:



Diseases related to Lennox-Gastaut Syndrome

Symptoms & Phenotypes for Lennox-Gastaut Syndrome

Human phenotypes related to Lennox-Gastaut Syndrome:

55 31 (show all 19)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 intellectual disability 55 31 hallmark (90%) Very frequent (99-80%) HP:0001249
2 encephalopathy 55 31 hallmark (90%) Very frequent (99-80%) HP:0001298
3 eeg with focal sharp slow waves 55 31 hallmark (90%) Very frequent (99-80%) HP:0011195
4 aggressive behavior 55 31 frequent (33%) Frequent (79-30%) HP:0000718
5 autistic behavior 55 31 frequent (33%) Frequent (79-30%) HP:0000729
6 hyperactivity 55 31 frequent (33%) Frequent (79-30%) HP:0000752
7 mental deterioration 55 31 frequent (33%) Frequent (79-30%) HP:0001268
8 myoclonus 55 31 frequent (33%) Frequent (79-30%) HP:0001336
9 generalized tonic-clonic seizures 55 31 frequent (33%) Frequent (79-30%) HP:0002069
10 abnormality of brainstem morphology 55 31 frequent (33%) Frequent (79-30%) HP:0002363
11 falls 55 31 frequent (33%) Frequent (79-30%) HP:0002527
12 atypical absence seizures 55 31 frequent (33%) Frequent (79-30%) HP:0007270
13 generalized tonic seizures 55 31 frequent (33%) Frequent (79-30%) HP:0010818
14 atonic seizures 55 31 frequent (33%) Frequent (79-30%) HP:0010819
15 personality disorder 55 31 frequent (33%) Frequent (79-30%) HP:0012075
16 generalized myoclonic seizures 55 31 occasional (7.5%) Occasional (29-5%) HP:0002123
17 focal seizures 55 31 occasional (7.5%) Occasional (29-5%) HP:0007359
18 behavioral abnormality 55 Frequent (79-30%)
19 eeg abnormality 55 Frequent (79-30%)

UMLS symptoms related to Lennox-Gastaut Syndrome:


hemiplegia

MGI Mouse Phenotypes related to Lennox-Gastaut Syndrome:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.2 ADGRG1 AKT3 CHD2 DCX DNM1 GABRA1
2 mortality/aging MP:0010768 10.07 AKT3 CHD2 DCX DNM1 GABRA1 GABRB3
3 nervous system MP:0003631 9.97 ADGRG1 AKT3 DCX DNM1 GABRA1 GABRB2
4 no phenotypic analysis MP:0003012 9.56 GABRA1 GABRB3 MAPK10 POMC SCNM1 SLC2A1
5 reproductive system MP:0005389 9.28 SCN1A SCNM1 AKT3 CHD2 DCX GABRA1

Drugs & Therapeutics for Lennox-Gastaut Syndrome

Drugs for Lennox-Gastaut Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 33)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Melatonin Approved, Nutraceutical, Vet_approved Phase 4 73-31-4 896
2 Antioxidants Phase 4
3 Central Nervous System Depressants Phase 4
4 Protective Agents Phase 4,Phase 3,Phase 1
5
Ethanol Approved Phase 3 64-17-5 702
6
Benzocaine Approved, Investigational Phase 3 1994-09-7, 94-09-7 2337
7
Clobazam Approved, Illicit Phase 3,Phase 2 22316-47-8 2789
8 sodium fluoride Approved Phase 3 7681-49-4
9
Topiramate Approved Phase 3,Phase 1 97240-79-4 5284627
10
Ezogabine Approved, Investigational Phase 3,Phase 2 150812-12-7 121892
11 Strawberry Approved, Nutraceutical Phase 3
12 tannic acid Approved, Nutraceutical Phase 3
13 Anticonvulsants Phase 3,Phase 2,Phase 1
14 Pharmaceutical Solutions Phase 3
15 Listerine Phase 3
16 Anti-Obesity Agents Phase 3,Phase 1
17 Neuroprotective Agents Phase 3,Phase 1
18 Neurotransmitter Agents Phase 3,Phase 2
19 Neurotransmitter Uptake Inhibitors Phase 3,Phase 2
20
Serotonin Phase 3,Phase 2 50-67-9 5202
21 Serotonin Agents Phase 3,Phase 2
22 Serotonin Uptake Inhibitors Phase 3,Phase 2
23
Dronabinol Approved, Illicit Phase 1 1972-08-3 16078 2978
24
Lamotrigine Approved, Investigational 84057-84-1 3878
25 calcium channel blockers
26 Calcium, Dietary
27 Diuretics, Potassium Sparing
28 Excitatory Amino Acid Antagonists
29 Excitatory Amino Acids
30 Sodium Channel Blockers
31 Fluorodeoxyglucose F18
32 Turmeric extract
33 Turmeric Nutraceutical

Interventional clinical trials:

(show all 34)

# Name Status NCT ID Phase Drugs
1 Melatonin Versus Placebo in the Lennox-Gastaut Syndrome: Neurophysiological and Neuropsychological Effects Unknown status NCT01370486 Phase 4 melatonin;placebo
2 Transcranial Direct Current Stimulation, Treatment of Childhood Drug-Resistant Lennox-Gastaut Syndrome, A Pilot Study Completed NCT02731300 Phase 4
3 A Placebo-Controlled, Double-Blind Comparative Study of E2080 in Lennox-Gastaut Syndrome Patients (Study E2080-J081-304) Completed NCT01146951 Phase 3 Rufinamide (E2080);Placebo
4 Study of Rufinamide in Pediatric Subjects 1 to Less Than 4 Years of Age With Lennox-Gastaut Syndrome Inadequately Controlled With Other Anti-epileptic Drugs Completed NCT01405053 Phase 3 Rufinamide;Any other approved AED
5 A Study to Investigate the Efficacy and Safety of Cannabidiol (GWP42003-P; CBD) as Adjunctive Treatment for Seizures Associated With Lennox-Gastaut Syndrome in Children and Adults Completed NCT02224690 Phase 3 GWP42003-P;Placebo Control
6 A Study to Investigate the Efficacy and Safety of Cannabidiol (GWP42003-P; CBD) as Adjunctive Treatment for Seizures Associated With Lennox-Gastaut Syndrome in Children and Adults Completed NCT02224560 Phase 3 GWP42003-P;Placebo control
7 Safety and Effectiveness of Open-Label Clobazam in Subjects With Lennox-Gastaut Syndrome Completed NCT01160770 Phase 3 Clobazam
8 Phase III Randomized, Double-Blind, Placebo-Controlled Study of Oral Topiramate for Lennox-Gastaut Syndrome Completed NCT00004776 Phase 3 topiramate
9 Clobazam in Patients With Lennox-Gastaut Syndrome Completed NCT00518713 Phase 3 Clobazam Low Dose;Clobazam Medium Dose;Clobazam High Dose;Placebo
10 A Study of the Efficacy and Safety of Topiramate as an add-on Therapy in the Treatment of Epilepsy Patients With Lennox-Gastaut Syndrome Completed NCT00236756 Phase 3 topiramate
11 A Long Term Extension Study of E2080 in Lennox-Gastaut Patients Completed NCT01151540 Phase 3 E2080
12 Cannabidiol Oral Solution as an Adjunctive Treatment for Treatment-resistant Seizure Disorder Completed NCT02318602 Phase 3 Cannabidiol Oral Solution
13 Study to Determine the Efficacy and Safety of Adjunctive Topiramate in the Treatment of Obsessive-Compulsive Disorder Completed NCT00187928 Phase 3 Topiramate
14 Study of Perampanel as Adjunctive Treatment for Inadequately Controlled Seizures Associated With Lennox-Gastaut Syndrome Recruiting NCT02834793 Phase 3 Perampanel;Placebo
15 A Study to Investigate the Efficacy and Safety of ZX008 (Fenfluramine Hydrochloride) as an Adjunctive Therapy in Children and Adults With Lennox-Gastaut Syndrome Recruiting NCT03355209 Phase 3 ZX008 02 and 0.8 mg/kg/day
16 GWPCARE5 - An Open Label Extension Study of Cannabidiol (GWP42003-P) in Children and Young Adults With Dravet or Lennox-Gastaut Syndromes Enrolling by invitation NCT02224573 Phase 3 GWP42003-P
17 Long-term, Open-label Safety Extension Study of Retigabine/Ezogabine in Pediatric Subjects (>= 12 Years Old) With POS or LGS Terminated NCT01668654 Phase 3 retigabine/ezogabine
18 Cannabidiol Oral Solution as an Adjunctive Therapy for Treatment of Participants With Inadequately Controlled Lennox-Gastaut Syndrome Withdrawn NCT02318537 Phase 3 Cannabidiol Oral Solution;Placebo Solution
19 Clobazam in Subjects With Lennox-Gastaut Syndrome Completed NCT00162981 Phase 2 Clobazam Low Dose;Clobazam High Dose
20 Add-on Therapy With Low Dose Fenfluramine in Lennox Gastaut Epilepsy Active, not recruiting NCT02655198 Phase 2 Fenfluramine
21 Study in Pediatric Subjects With Epilepsy Terminated NCT01494584 Phase 2 ezogabine/retigabine
22 A Study of the Pharmacokinetics, Safety and Tolerability of Topiramate in Infants (Age 1-24 Months) With Refractory Partial-onset Seizures Completed NCT00233012 Phase 1 Topiramate
23 Cannabidiol in Children With Refractory Epileptic Encephalopathy Recruiting NCT03024827 Phase 1 CanniMed® 1:20
24 Epilepsy Phenome/Genome Project Unknown status NCT00552045
25 European Registry of Anti-Epileptic Drug Use in Patients With Lennox-Gastaut Syndrome (LGS) Completed NCT01991041
26 A Study of the Safety of Topiramate Given in Combination With Other Medications in Adults and Children With Seizures Completed NCT00297349 Topiramate
27 Ketogenic Diet for Child Epilepsy and Seizure Control Completed NCT00004729
28 Special Drug Use Investigation for LAMICTAL® (Long Term) Completed NCT01863602 Lamotrigine tablets
29 Metabolic Abnormalities in Children With Epilepsy Completed NCT00001325 18 FDG
30 Trial to Assess Vagus Nerve Stimulation Therapy in Children With Lennox-Gastaut Syndrome Recruiting NCT02632149 Early Phase 1
31 Post-marketing Surveillance of Long-term Administration of Inovelon Tablets in Patients With Lennox-Gastaut Syndrome Active, not recruiting NCT02175173 Rufinamide
32 The Effects of Cannabidiol (CBD) on Electrical and Autonomic Cardiac Function in Children With Severe Epilepsy Not yet recruiting NCT02815540 Cannabadiol
33 Turmeric as Treatment in Epilepsy Not yet recruiting NCT03254680
34 The Pharmacokinetics of Cannabidiol (CBD) and Its Effects in Children With Severe Epilepsy Withdrawn NCT02910297

Search NIH Clinical Center for Lennox-Gastaut Syndrome

Inferred drug relations via UMLS 69 / NDF-RT 47 :


Genetic Tests for Lennox-Gastaut Syndrome

Genetic tests related to Lennox-Gastaut Syndrome:

# Genetic test Affiliating Genes
1 Epileptic Encephalopathy Lennox-Gastaut Type 28

Anatomical Context for Lennox-Gastaut Syndrome

MalaCards organs/tissues related to Lennox-Gastaut Syndrome:

38
Brain, Eye, Cortex, Thalamus, Liver, Heart

Publications for Lennox-Gastaut Syndrome

Articles related to Lennox-Gastaut Syndrome:

(show top 50) (show all 321)
# Title Authors Year
1
Rufinamide for the treatment of Lennox-Gastaut syndrome: evidence from clinical trials and clinical practice. ( 29313492 )
2018
2
Cannabidiol for drop seizures in Lennox-Gastaut syndrome. ( 29395274 )
2018
3
Optimizing clobazam treatment in patients with Lennox-Gastaut syndrome. ( 29202277 )
2018
4
Cannabidiol in patients with seizures associated with Lennox-Gastaut syndrome (GWPCARE4): a randomised, double-blind, placebo-controlled phase 3 trial. ( 29395273 )
2018
5
Real-world data on rufinamide treatment in patients with Lennox-Gastaut syndrome: Results from a European noninterventional registry study. ( 28927712 )
2017
6
Thalamocortical functional connectivity in Lennox-Gastaut syndrome is abnormally enhanced in executive-control and default-mode networks. ( 29098688 )
2017
7
Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions. ( 28461749 )
2017
8
Spectral characteristics of intracranial electroencephalographic activity in patients with Lennox-Gastaut syndrome. ( 27686687 )
2017
9
Early Diagnosis and Treatment of Lennox-Gastaut Syndrome. ( 28689466 )
2017
10
Lennox-Gastaut Syndrome: A Prospective Follow-up Study. ( 28479797 )
2017
11
Dosing considerations for rufinamide in patients with Lennox-Gastaut syndrome: Phase III trial results and real-world clinical data. ( 28284045 )
2017
12
Lennox-Gastaut syndrome in adulthood: Long-term clinical follow-up of 38 patients and analysis of their recorded seizures. ( 29126048 )
2017
13
Electroencephalography Network Effects of Corpus Callosotomy in Patients with Lennox-Gastaut Syndrome. ( 28928710 )
2017
14
Use of perampanel in children and adolescents with Lennox-Gastaut Syndrome. ( 28715780 )
2017
15
Expert Opinion on the Management of Lennox-Gastaut Syndrome: Treatment Algorithms and Practical Considerations. ( 29085326 )
2017
16
Adult motor phenotype differentiates Dravet syndrome from Lennox-Gastaut syndrome and links SCN1A to early onset parkinsonian features. ( 28186331 )
2017
17
Lennox-Gastaut Syndrome: A State of the Art Review. ( 28346953 )
2017
18
Assessment of treatment patterns and healthcare costs associated with probable Lennox-Gastaut syndrome. ( 28609734 )
2017
19
Use and cost comparison of clobazam to other antiepileptic drugs for treatment of Lennox-Gastaut syndrome. ( 28740620 )
2017
20
Lennox-Gastaut syndrome: a comprehensive review. ( 29124439 )
2017
21
Cognitive network reorganization following surgical control of seizures in Lennox-Gastaut syndrome. ( 28295228 )
2017
22
Understanding Lennox-Gastaut syndrome: insights from focal epilepsy patients with Lennox-Gastaut features. ( 28584915 )
2017
23
Ophthalmologic Features of Lennox-Gastaut Syndrome. ( 28471101 )
2017
24
Safety and pharmacokinetic profile of rufinamide in pediatric patients aged less than 4 years with Lennox-Gastaut syndrome: An interim analysis from a multicenter, randomized, active-controlled, open-label study. ( 26805435 )
2016
25
An Observational Report of Worsening Seizures with Increase in Total Charge Delivered Per Day by Vagus Nerve Stimulation in 4 Patients with Lennox-Gastaut Syndrome. ( 26705197 )
2016
26
Abnormal cognitive network interactions in Lennox-Gastaut syndrome: A potential mechanism of epileptic encephalopathy. ( 26945476 )
2016
27
Nocturnal interictal epileptic discharges in adult Lennox-Gastaut syndrome: the effect of sleep stage and time of night. ( 26842220 )
2016
28
Challenges in identifying Lennox-Gastaut syndrome in adults: A case series illustrating its changing nature. ( 26977407 )
2016
29
Predictive role of brain connectivity for resective surgery in Lennox-Gastaut syndrome. ( 27417063 )
2016
30
Lacosamide in Lennox-Gastaut Syndrome? Caution Is Still Needed. ( 27827811 )
2016
31
Drug-resistant epilepsy after treatment for childhood acute lymphocytic leukaemia: from focal epilepsy to Lennox-Gastaut syndrome. ( 27934621 )
2016
32
Brainstem dysfunction in patients with late-onset Lennox-Gastaut syndrome: Voxel-based morphometry and tract-based spatial statistics study. ( 27994367 )
2016
33
Combined surgical intervention with vagus nerve stimulation following corpus callosotomy in patients with Lennox-Gastaut syndrome. ( 26979179 )
2016
34
Long-term safety and seizure outcome in Japanese patients with Lennox-Gastaut syndrome receiving adjunctive rufinamide therapy: An open-label study following a randomized clinical trial. ( 26827266 )
2016
35
Therapeutic effects of the ketogenic diet in children with Lennox-Gastaut syndrome. ( 27846468 )
2016
36
Resective surgery combined with corpus callosotomy for children with non-focal lesional Lennox-Gastaut syndrome. ( 27629372 )
2016
37
Anesthetic management of a child with Lennox-Gastaut syndrome with intractable epilepsy posted for intracranial surgery! ( 27857813 )
2016
38
Adjunctive use of electroconvulsive therapy in patients with Lennox-Gastaut syndrome and drug-resistant epilepsy: A pilot study. ( 28210578 )
2016
39
Pontine and cerebral atrophy in Lennox-Gastaut syndrome. ( 26808205 )
2016
40
Clobazam-treated patients with Lennox-Gastaut syndrome experienced fewer seizure-related injuries than placebo patients during trial OV-1012. ( 27145465 )
2016
41
An unfortunate challenge: Ketogenic diet for the treatment of Lennox-Gastaut syndrome in tyrosinemia type 1. ( 27052529 )
2016
42
Transcranial Direct Current Stimulation for Treatment of Childhood Pharmacoresistant Lennox-Gastaut Syndrome: A Pilot Study. ( 27199889 )
2016
43
Treatment of Adults with Lennox-Gastaut Syndrome: Further Analysis of Efficacy and Safety/Tolerability of Rufinamide. ( 26861566 )
2016
44
In response: Lennox-Gastaut syndrome may be a curable, reversible epileptic encephalopathy. ( 25778756 )
2015
45
The causal epileptic network identifies the primary epileptogenic zone in Lennox-Gastaut syndrome. ( 26513494 )
2015
46
Efficacy and tolerability of add-on Lacosamide treatment in adults with Lennox-Gastaut syndrome: An observational study. ( 26606192 )
2015
47
Retention rates of rufinamide in pediatric epilepsy patients with and without Lennox-Gastaut Syndrome. ( 25847334 )
2015
48
Clobazam is equally safe and efficacious for seizures associated with Lennox-Gastaut syndrome across different age groups: Post hoc analyses of short- and long-term clinical trial results. ( 25940107 )
2015
49
Lennox-Gastaut syndrome. Management update. ( 26166587 )
2015
50
Perceived efficacy of cannabidiol-enriched cannabis extracts for treatment of pediatric epilepsy: A potential role for infantile spasms and Lennox-Gastaut syndrome. ( 25935511 )
2015

Variations for Lennox-Gastaut Syndrome

ClinVar genetic disease variations for Lennox-Gastaut Syndrome:

6
# Gene Variation Type Significance SNP ID Assembly Location
1 SCN1A NM_006920.4(SCN1A): c.4874G> A (p.Arg1625Gln) single nucleotide variant Pathogenic/Likely pathogenic rs121917995 GRCh37 Chromosome 2, 166848878: 166848878

Expression for Lennox-Gastaut Syndrome

Search GEO for disease gene expression data for Lennox-Gastaut Syndrome.

Pathways for Lennox-Gastaut Syndrome

GO Terms for Lennox-Gastaut Syndrome

Cellular components related to Lennox-Gastaut Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 chloride channel complex GO:0034707 9.33 GABRA1 GABRB2 GABRB3
2 node of Ranvier GO:0033268 9.32 KCNQ2 SCN1A
3 axon initial segment GO:0043194 9.26 KCNQ2 SCN1A
4 GABA receptor complex GO:1902710 8.96 GABRA1 GABRB2
5 GABA-A receptor complex GO:1902711 8.8 GABRA1 GABRB2 GABRB3

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

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 transmembrane transport GO:0055085 9.8 GABRA1 GABRB2 GABRB3 KCNQ2 SCN1A SLC2A1
2 chloride transmembrane transport GO:1902476 9.65 GABRA1 GABRB2 GABRB3
3 ion transmembrane transport GO:0034220 9.65 GABRA1 GABRB2 GABRB3 KCNQ2 SCN1A
4 chloride transport GO:0006821 9.61 GABRA1 GABRB2 GABRB3
5 neuron development GO:0048666 9.54 GABRB2 GABRB3 MAPK10
6 innervation GO:0060384 9.51 GABRB2 GABRB3
7 layer formation in cerebral cortex GO:0021819 9.46 ADGRG1 DCX
8 inner ear receptor cell development GO:0060119 9.4 GABRB2 GABRB3
9 gamma-aminobutyric acid signaling pathway GO:0007214 9.33 GABRA1 GABRB2 GABRB3
10 cellular response to histamine GO:0071420 9.13 GABRA1 GABRB2 GABRB3
11 synaptic transmission, GABAergic GO:0051932 8.8 DNM1 GABRA1 GABRB2
12 transport GO:0006810 10.1 GABRA1 GABRB2 GABRB3 KCNQ2 KPNA7 SCN1A

Molecular functions related to Lennox-Gastaut Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 chloride channel activity GO:0005254 9.54 GABRA1 GABRB2 GABRB3
2 extracellular ligand-gated ion channel activity GO:0005230 9.5 GABRA1 GABRB2 GABRB3
3 ion channel activity GO:0005216 9.46 GABRA1 GABRB2 GABRB3 SCN1A
4 GABA-gated chloride ion channel activity GO:0022851 9.26 GABRA1 GABRB3
5 GABA receptor activity GO:0016917 8.96 GABRA1 GABRB2
6 GABA-A receptor activity GO:0004890 8.8 GABRA1 GABRB2 GABRB3

Sources for Lennox-Gastaut Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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