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MCID: LNN001
MIFTS: 58

Lennox-Gastaut Syndrome

Categories: Rare diseases, Neuronal diseases
Genes Variations Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Lennox-Gastaut Syndrome

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MalaCards integrated aliases for Lennox-Gastaut Syndrome:

Name: Lennox-Gastaut Syndrome 12 53 25 54 59 37 55 15 73
Epileptic Encephalopathy Lennox-Gastaut Type 53 29 6
Lennox Syndrome 12 73
Childhood Epileptic Encephalopathy with Diffuse Slow Spikes and Waves 25
Epileptic Encephalopathy, Lennox-Gastaut Type 13
Encephalopathy of Childhood 53
Lgs 25

Characteristics:

Orphanet epidemiological data:

59
lennox-gastaut syndrome
Inheritance: Autosomal dominant,Multigenic/multifactorial,Not applicable; Prevalence: 1-9/1000000 (Europe),1-5/10000 (Europe); Age of onset: Childhood;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Neuronal diseases
See all MalaCards categories (disease lists)
ICD10: 34
Orphanet: 59  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:0050561
Orphanet 59 ORPHA2382
UMLS via Orphanet 74 C0238111
MESH via Orphanet 45 C535500
ICD10 via Orphanet 34 G40.4
KEGG 37 H01813
Sources

Summaries for Lennox-Gastaut Syndrome

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NINDS : 54 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 infantile epileptic encephalopathy, and has symptoms including hemiplegia 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 intellectual disability and encephalopathy

NIH Rare Diseases : 53 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.

Genetics Home Reference : 25 Lennox-Gastaut syndrome is a form of severe epilepsy that begins in childhood. It is characterized by multiple types of seizures and intellectual disability.

Wikipedia : 76 Lennox–Gastaut syndrome (LGS) is a childhood-onset epilepsy that most often appears between the second... more...

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Related Diseases for Lennox-Gastaut Syndrome

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

(show top 50) (show all 64)
# Related Disease Score Top Affiliating Genes
1 myoclonic astatic epilepsy 33.4 SCN1A SLC2A1
2 infantile epileptic encephalopathy 31.4 SCN1A ST3GAL3 STXBP1
3 epileptic encephalopathy, early infantile, 15 31.1 AKT3 GABRB3 KCNQ2 SCN1A ST3GAL3 STXBP1
4 focal epilepsy 30.7 SCN1A SLC2A1 TBC1D24
5 epilepsy 30.3 GABRA1 GABRB3 KCNQ2 SCN1A SLC2A1 TBC1D24
6 west syndrome 29.9 KCNQ2 MT-ND1 POMC SCN1A ST3GAL3 STXBP1
7 epileptic encephalopathy, early infantile, 6 28.2 CHD2 GABRA1 GABRB3 KCNQ2 SCN1A SNRPE
8 neurodegeneration with brain iron accumulation 5 11.8
9 trichorhinophalangeal syndrome, type ii 11.3
10 scn1a-related seizure disorders 11.3
11 encephalopathy, acute, infection-induced 4 11.3
12 exostoses, multiple, type i 10.9
13 lymphomatoid granulomatosis 10.9
14 landau-kleffner syndrome 10.6 POMC TBC1D24
15 encephalopathy 10.6
16 epilepsy with generalized tonic-clonic seizures 10.5 SCN1A TBC1D24
17 status epilepticus 10.4
18 childhood disintegrative disease 10.3 GABRB3 POMC
19 epileptic encephalopathy, early infantile, 1 10.3 KCNQ2 TBC1D24
20 aging 10.3
21 cerebritis 10.3
22 hypothalamic hamartomas 10.2
23 tuberous sclerosis 10.2
24 undetermined early-onset epileptic encephalopathy 10.1 DNM1 GABRB2 STXBP1
25 adolescence-adult electroclinical syndrome 10.1 GABRA1 GABRB3 SCN1A TBC1D24
26 angelman syndrome 10.1
27 autism 10.1
28 myoclonic epilepsy of unverricht and lundborg 10.1
29 polymicrogyria, bilateral frontoparietal 10.1
30 alacrima, achalasia, and mental retardation syndrome 10.1
31 common variable immunodeficiency 10.1
32 hepatitis 10.1
33 autism spectrum disorder 10.1
34 hypospadias 10.1
35 encephalomalacia 10.1
36 astrocytoma 10.1
37 acute disseminated encephalomyelitis 10.1
38 neuronitis 10.1
39 measles 10.1
40 tyrosinemia 10.1
41 intracranial hypertension 10.1
42 polymicrogyria 10.1
43 cutis verticis gyrata 10.1
44 dandy-walker complex 10.1
45 hemimegalencephaly 10.1
46 pachygyria 10.1
47 cerebral atrophy 10.1
48 dysphagia 10.1
49 myoclonus 10.1
50 myoclonic-astastic epilepsy 10.1

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



Diseases related to Lennox-Gastaut Syndrome
Sources

Symptoms & Phenotypes for Lennox-Gastaut Syndrome

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Human phenotypes related to Lennox-Gastaut Syndrome:

59 32 (show all 19)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 intellectual disability 59 32 hallmark (90%) Very frequent (99-80%) HP:0001249
2 encephalopathy 59 32 hallmark (90%) Very frequent (99-80%) HP:0001298
3 eeg with focal sharp slow waves 59 32 hallmark (90%) Very frequent (99-80%) HP:0011195
4 aggressive behavior 59 32 frequent (33%) Frequent (79-30%) HP:0000718
5 autistic behavior 59 32 frequent (33%) Frequent (79-30%) HP:0000729
6 hyperactivity 59 32 frequent (33%) Frequent (79-30%) HP:0000752
7 mental deterioration 59 32 frequent (33%) Frequent (79-30%) HP:0001268
8 myoclonus 59 32 frequent (33%) Frequent (79-30%) HP:0001336
9 generalized tonic-clonic seizures 59 32 frequent (33%) Frequent (79-30%) HP:0002069
10 abnormality of brainstem morphology 59 32 frequent (33%) Frequent (79-30%) HP:0002363
11 falls 59 32 frequent (33%) Frequent (79-30%) HP:0002527
12 atypical absence seizures 59 32 frequent (33%) Frequent (79-30%) HP:0007270
13 generalized tonic seizures 59 32 frequent (33%) Frequent (79-30%) HP:0010818
14 atonic seizures 59 32 frequent (33%) Frequent (79-30%) HP:0010819
15 personality disorder 59 32 frequent (33%) Frequent (79-30%) HP:0012075
16 generalized myoclonic seizures 59 32 occasional (7.5%) Occasional (29-5%) HP:0002123
17 focal seizures 59 32 occasional (7.5%) Occasional (29-5%) HP:0007359
18 behavioral abnormality 59 Frequent (79-30%)
19 eeg abnormality 59 Frequent (79-30%)

UMLS symptoms related to Lennox-Gastaut Syndrome:


hemiplegia

MGI Mouse Phenotypes related to Lennox-Gastaut Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.13 ADGRG1 AKT3 CHD2 DCX DNM1 GABRA1
2 mortality/aging MP:0010768 9.93 POMC SCN1A SCNM1 SLC2A1 STXBP1 AKT3
3 nervous system MP:0003631 9.77 ADGRG1 AKT3 DCX DNM1 GABRA1 GABRB2
4 no phenotypic analysis MP:0003012 9.23 DNM1 GABRA1 GABRB3 MAPK10 POMC SCNM1
Sources

Drugs & Therapeutics for Lennox-Gastaut Syndrome

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

(show all 32)
# 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,Not Applicable
3 Central Nervous System Depressants Phase 4
4 Protective Agents Phase 4,Phase 3,Phase 1,Not Applicable
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
Lamotrigine Approved, Investigational Phase 3 84057-84-1 3878
11
Valproic Acid Approved, Investigational Phase 3 99-66-1 3121
12
Ezogabine Approved, Investigational Phase 3,Phase 2 150812-12-7 121892
13 Strawberry Approved, Nutraceutical Phase 3
14 tannic acid Approved, Nutraceutical Phase 3
15 Anticonvulsants Phase 3,Phase 2,Phase 1
16 Pharmaceutical Solutions Phase 3
17 Listerine Phase 3
18 Anti-Obesity Agents Phase 3,Phase 1
19 Neuroprotective Agents Phase 3,Phase 1
20 calcium channel blockers Phase 3
21 Calcium, Dietary Phase 3
22 Diuretics, Potassium Sparing Phase 3
23 Excitatory Amino Acid Antagonists Phase 3
24 Excitatory Amino Acids Phase 3
25 Neurotransmitter Agents Phase 3,Phase 2
26 Sodium Channel Blockers Phase 3
27 Neurotransmitter Uptake Inhibitors Phase 3,Phase 2
28 Serotonin Agents Phase 3,Phase 2
29 Serotonin Uptake Inhibitors Phase 3,Phase 2
30
Serotonin Investigational, Nutraceutical Phase 3,Phase 2 50-67-9 5202
31
Dronabinol Approved, Illicit Phase 1 1972-08-3 16078
32 Fluorodeoxyglucose F18

Interventional clinical trials:

(show all 36)
# 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 Clinical Evaluation of BW430C in Epilepsy Completed NCT00395694 Phase 3 lamictal
15 Study of Perampanel as Adjunctive Treatment for Inadequately Controlled Seizures Associated With Lennox-Gastaut Syndrome Recruiting NCT02834793 Phase 3 Perampanel;Placebo
16 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
17 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
18 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
19 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
20 Clobazam in Subjects With Lennox-Gastaut Syndrome Completed NCT00162981 Phase 2 Clobazam Low Dose;Clobazam High Dose
21 Add-on Therapy With Low Dose Fenfluramine in Lennox Gastaut Epilepsy Active, not recruiting NCT02655198 Phase 2 Fenfluramine
22 A Study to Assess the Safety and Tolerability of ZX008 in Children and Young Adults With DS or LGS Currently Taking CBD Enrolling by invitation NCT03467113 Phase 1, Phase 2 ZX008 02 and 0.8 mg/kg/day
23 Study in Pediatric Subjects With Epilepsy Terminated NCT01494584 Phase 2 ezogabine/retigabine
24 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
25 Cannabidiol in Children With Refractory Epileptic Encephalopathy Recruiting NCT03024827 Phase 1 CanniMed® 1:20
26 The Effects of Cannabidiol (CBD) on Electrical and Autonomic Cardiac Function in Children With Severe Epilepsy Unknown status NCT02815540 Not Applicable Cannabadiol
27 Epilepsy Phenome/Genome Project Unknown status NCT00552045
28 European Registry of Anti-Epileptic Drug Use in Patients With Lennox-Gastaut Syndrome (LGS) Completed NCT01991041
29 A Study of the Safety of Topiramate Given in Combination With Other Medications in Adults and Children With Seizures Completed NCT00297349 Topiramate
30 Ketogenic Diet for Child Epilepsy and Seizure Control Completed NCT00004729 Not Applicable
31 Special Drug Use Investigation for LAMICTAL® (Long Term) Completed NCT01863602 Lamotrigine tablets
32 Metabolic Abnormalities in Children With Epilepsy Completed NCT00001325 18 FDG
33 Trial to Assess Vagus Nerve Stimulation Therapy in Children With Lennox-Gastaut Syndrome Recruiting NCT02632149 Early Phase 1
34 Post-marketing Surveillance of Long-term Administration of Inovelon Tablets in Patients With Lennox-Gastaut Syndrome Active, not recruiting NCT02175173 Rufinamide
35 The Pharmacokinetics of Cannabidiol (CBD) and Its Effects in Children With Severe Epilepsy Withdrawn NCT02910297
36 Turmeric as Treatment in Epilepsy Withdrawn NCT03254680 Not Applicable

Search NIH Clinical Center for Lennox-Gastaut Syndrome

Inferred drug relations via UMLS 73 / NDF-RT 51 :
Sources

Genetic Tests for Lennox-Gastaut Syndrome

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Genetic tests related to Lennox-Gastaut Syndrome:

# Genetic test Affiliating Genes
1 Epileptic Encephalopathy Lennox-Gastaut Type 29 MAPK10
Sources

Anatomical Context for Lennox-Gastaut Syndrome

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MalaCards organs/tissues related to Lennox-Gastaut Syndrome:

41
Brain, Eye, Cortex, Thalamus, Liver, Heart
Sources

Publications for Lennox-Gastaut Syndrome

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Articles related to Lennox-Gastaut Syndrome:

(show top 50) (show all 325)
# Title Authors Year
1
Addition of Cannabidiol to Current Antiepileptic Therapy Reduces Drop Seizures in Children and Adults With Treatment-Resistant Lennox-Gastaut Syndrome. ( 29887290 )
2018
2
Changes in functional brain network topology after successful and unsuccessful corpus callosotomy for Lennox-Gastaut Syndrome. ( 29467376 )
2018
3
Cannabidiol for drop seizures in Lennox-Gastaut syndrome. ( 29395274 )
2018
4
Optimizing clobazam treatment in patients with Lennox-Gastaut syndrome. ( 29202277 )
2018
5
Few individuals with Lennox-Gastaut syndrome have autism spectrum disorder: a comparison with Dravet syndrome. ( 29558884 )
2018
6
Cannabidiol in patients with seizures associated with Lennox-Gastaut syndrome (GWPCARE4): a randomised, double-blind, placebo-controlled phase 3 trial. ( 29395273 )
2018
7
Effect of Cannabidiol on Drop Seizures in the Lennox-Gastaut Syndrome. ( 29768152 )
2018
8
Rufinamide for the treatment of Lennox-Gastaut syndrome: evidence from clinical trials and clinical practice. ( 29313492 )
2018
9
Lennox-Gastaut Syndrome: A State of the Art Review. ( 28346953 )
2017
10
Assessment of treatment patterns and healthcare costs associated with probable Lennox-Gastaut syndrome. ( 28609734 )
2017
11
Spectral characteristics of intracranial electroencephalographic activity in patients with Lennox-Gastaut syndrome. ( 27686687 )
2017
12
Adult motor phenotype differentiates Dravet syndrome from Lennox-Gastaut syndrome and links SCN1A to early onset parkinsonian features. ( 28186331 )
2017
13
Lennox-Gastaut Syndrome: A Prospective Follow-up Study. ( 28479797 )
2017
14
Dosing considerations for rufinamide in patients with Lennox-Gastaut syndrome: Phase III trial results and real-world clinical data. ( 28284045 )
2017
15
Understanding Lennox-Gastaut syndrome: insights from focal epilepsy patients with Lennox-Gastaut features. ( 28584915 )
2017
16
Early Diagnosis and Treatment of Lennox-Gastaut Syndrome. ( 28689466 )
2017
17
Real-world data on rufinamide treatment in patients with Lennox-Gastaut syndrome: Results from a European noninterventional registry study. ( 28927712 )
2017
18
Lennox-Gastaut syndrome: a comprehensive review. ( 29124439 )
2017
19
Use and cost comparison of clobazam to other antiepileptic drugs for treatment of Lennox-Gastaut syndrome. ( 28740620 )
2017
20
Cognitive network reorganization following surgical control of seizures in Lennox-Gastaut syndrome. ( 28295228 )
2017
21
Ophthalmologic Features of Lennox-Gastaut Syndrome. ( 28471101 )
2017
22
Thalamocortical functional connectivity in Lennox-Gastaut syndrome is abnormally enhanced in executive-control and default-mode networks. ( 29098688 )
2017
23
Expert Opinion on the Management of Lennox-Gastaut Syndrome: Treatment Algorithms and Practical Considerations. ( 29085326 )
2017
24
Lennox-Gastaut syndrome in adulthood: Long-term clinical follow-up of 38 patients and analysis of their recorded seizures. ( 29126048 )
2017
25
Use of perampanel in children and adolescents with Lennox-Gastaut Syndrome. ( 28715780 )
2017
26
Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions. ( 28461749 )
2017
27
Electroencephalography Network Effects of Corpus Callosotomy in Patients with Lennox-Gastaut Syndrome. ( 28928710 )
2017
28
Predictive role of brain connectivity for resective surgery in Lennox-Gastaut syndrome. ( 27417063 )
2016
29
Challenges in identifying Lennox-Gastaut syndrome in adults: A case series illustrating its changing nature. ( 26977407 )
2016
30
Nocturnal interictal epileptic discharges in adult Lennox-Gastaut syndrome: the effect of sleep stage and time of night. ( 26842220 )
2016
31
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
32
Lacosamide in Lennox-Gastaut Syndrome? Caution Is Still Needed. ( 27827811 )
2016
33
Treatment of Adults with Lennox-Gastaut Syndrome: Further Analysis of Efficacy and Safety/Tolerability of Rufinamide. ( 26861566 )
2016
34
An unfortunate challenge: Ketogenic diet for the treatment of Lennox-Gastaut syndrome in tyrosinemia type 1. ( 27052529 )
2016
35
Anesthetic management of a child with Lennox-Gastaut syndrome with intractable epilepsy posted for intracranial surgery! ( 27857813 )
2016
36
Brainstem dysfunction in patients with late-onset Lennox-Gastaut syndrome: Voxel-based morphometry and tract-based spatial statistics study. ( 27994367 )
2016
37
Therapeutic effects of the ketogenic diet in children with Lennox-Gastaut syndrome. ( 27846468 )
2016
38
Clobazam-treated patients with Lennox-Gastaut syndrome experienced fewer seizure-related injuries than placebo patients during trial OV-1012. ( 27145465 )
2016
39
Resective surgery combined with corpus callosotomy for children with non-focal lesional Lennox-Gastaut syndrome. ( 27629372 )
2016
40
Transcranial Direct Current Stimulation for Treatment of Childhood Pharmacoresistant Lennox-Gastaut Syndrome: A Pilot Study. ( 27199889 )
2016
41
Combined surgical intervention with vagus nerve stimulation following corpus callosotomy in patients with Lennox-Gastaut syndrome. ( 26979179 )
2016
42
Pontine and cerebral atrophy in Lennox-Gastaut syndrome. ( 26808205 )
2016
43
Adjunctive use of electroconvulsive therapy in patients with Lennox-Gastaut syndrome and drug-resistant epilepsy: A pilot study. ( 28210578 )
2016
44
Drug-resistant epilepsy after treatment for childhood acute lymphocytic leukaemia: from focal epilepsy to Lennox-Gastaut syndrome. ( 27934621 )
2016
45
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
46
Abnormal cognitive network interactions in Lennox-Gastaut syndrome: A potential mechanism of epileptic encephalopathy. ( 26945476 )
2016
47
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
48
Clobazam and Aggression-Related Adverse Events in Pediatric Patients With Lennox-Gastaut Syndrome. ( 26245776 )
2015
49
Comprehensive overview: efficacy, tolerability, and cost-effectiveness of clobazam in Lennox-Gastaut syndrome. ( 26089675 )
2015
50
The causal epileptic network identifies the primary epileptogenic zone in Lennox-Gastaut syndrome. ( 26513494 )
2015
Sources

Variations for Lennox-Gastaut Syndrome

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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 Likely pathogenic rs121917995 GRCh37 Chromosome 2, 166848878: 166848878
2 SCN1A NM_006920.4(SCN1A): c.4874G> A (p.Arg1625Gln) single nucleotide variant Likely pathogenic rs121917995 GRCh38 Chromosome 2, 165992368: 165992368
Sources

Expression for Lennox-Gastaut Syndrome

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

Pathways for Lennox-Gastaut Syndrome

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

# Super pathways Score Top Affiliating Genes
1
Neuroscience 4
12.36 DCX KCNQ2 POMC SCN1A STXBP1
2
GABAergic synapse 37
Show member pathways
 12.28 GABRA1 GABRB2 GABRB3 MAPK10 MT-ND1
3
Apoptosis Pathway 72
Show member pathways
 11.98 AKT3 GABRA1 GABRB2 GABRB3 MAPK10
4
Insulin resistance 37
Show member pathways
 11.67 AKT3 MAPK10 SLC2A1
5
L1CAM interactions 66
Show member pathways
 11.65 DCX DNM1 KCNQ2 SCN1A
6
Ligand-gated ion channel transport 66
Show member pathways
 11.2 GABRA1 GABRB2 GABRB3
7
Nicotine addiction 37
11.1 GABRA1 GABRB2 GABRB3
8
Adipocytokine signaling pathway 37
10.76 AKT3 MAPK10 POMC SLC2A1
9
Benzodiazepine Pathway, Pharmacodynamics 61
Show member pathways
 10.73 GABRA1 GABRB2
10
Synaptic Neurotransmission Pathways: GABAergic Inhibition 65
10.36 GABRA1 GABRB2 GABRB3
Sources

GO Terms for Lennox-Gastaut Syndrome

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

# Name GO ID Score Top Affiliating Genes
1 neuron projection GO:0043005 9.65 DCX GABRA1 GABRB2 GABRB3 MAPK10
2 postsynaptic membrane GO:0045211 9.62 DNM1 GABRA1 GABRB2 GABRB3
3 node of Ranvier GO:0033268 9.4 KCNQ2 SCN1A
4 axon initial segment GO:0043194 9.37 KCNQ2 SCN1A
5 chloride channel complex GO:0034707 9.33 GABRA1 GABRB2 GABRB3
6 GABA receptor complex GO:1902710 8.96 GABRA1 GABRB2
7 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 14)
# Name GO ID Score Top Affiliating Genes
1 ion transport GO:0006811 9.93 GABRA1 GABRB2 GABRB3 KCNQ2 SCN1A
2 chemical synaptic transmission GO:0007268 9.81 GABRA1 GABRB2 GABRB3 KCNQ2
3 ion transmembrane transport GO:0034220 9.72 GABRA1 GABRB2 GABRB3 KCNQ2 SCN1A
4 chloride transmembrane transport GO:1902476 9.65 GABRA1 GABRB2 GABRB3
5 chloride transport GO:0006821 9.61 GABRA1 GABRB2 GABRB3
6 neuron development GO:0048666 9.58 GABRB2 GABRB3 MAPK10
7 regulation of membrane potential GO:0042391 9.56 GABRA1 GABRB2 GABRB3 SCN1A
8 nervous system process GO:0050877 9.54 GABRA1 GABRB2 GABRB3
9 innervation GO:0060384 9.52 GABRB2 GABRB3
10 layer formation in cerebral cortex GO:0021819 9.49 ADGRG1 DCX
11 inner ear receptor cell development GO:0060119 9.46 GABRB2 GABRB3
12 gamma-aminobutyric acid signaling pathway GO:0007214 9.33 GABRA1 GABRB2 GABRB3
13 synaptic transmission, GABAergic GO:0051932 9.13 DNM1 GABRA1 GABRB2
14 cellular response to histamine GO:0071420 8.8 GABRA1 GABRB2 GABRB3

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 9.5 GABRA1 GABRB2 GABRB3
2 ion channel activity GO:0005216 9.46 GABRA1 GABRB2 GABRB3 SCN1A
3 extracellular ligand-gated ion channel activity GO:0005230 9.43 GABRA1 GABRB2 GABRB3
4 GABA-gated chloride ion channel activity GO:0022851 9.4 GABRA1 GABRB3
5 inhibitory extracellular ligand-gated ion channel activity GO:0005237 9.37 GABRA1 GABRB2
6 GABA receptor activity GO:0016917 8.96 GABRA1 GABRB2
7 GABA-A receptor activity GO:0004890 8.8 GABRA1 GABRB2 GABRB3
Sources

Sources for Lennox-Gastaut Syndrome

About this section
3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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