LGS
MCID: LNN001
MIFTS: 57

Lennox-Gastaut Syndrome (LGS)

Categories: Neuronal diseases, Rare diseases
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Aliases & Classifications for Lennox-Gastaut Syndrome

MalaCards integrated aliases for Lennox-Gastaut Syndrome:

Name: Lennox-Gastaut Syndrome 11 19 42 52 58 75 53 5 14 71 33
Encephalopathy of Childhood 19 5
Lennox Syndrome 11 71
Childhood Epileptic Encephalopathy with Diffuse Slow Spikes and Waves 42
Epileptic Encephalopathy Lennox-Gastaut Type 19
Lennox Gastaut Syndrome 43
Lgs 42

Characteristics:


Inheritance:

Autosomal dominant,Multigenic/multifactorial 58

Prevelance:

1-9/1000000 (Europe) 1-5/10000 (Europe) 58

Age Of Onset:

Childhood,Infancy 58

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Disease Ontology 11 DOID:0050561
MeSH 43 D065768
MESH via Orphanet 44 C535500
ICD10 via Orphanet 32 G40.4
UMLS via Orphanet 72 C0238111
Orphanet 58 ORPHA2382
ICD11 33 651135242
UMLS 71 C0238111 C0598392

Summaries for Lennox-Gastaut Syndrome

MedlinePlus Genetics: 42 Lennox-Gastaut syndrome is a severe condition characterized by recurrent seizures (epilepsy) that begin early in life. Affected individuals have multiple types of seizures, a particular pattern of brain activity (called slow spike-and-wave) measured by a test called an electroencephalogram (EEG), and impaired mental abilities.In Lennox-Gastaut syndrome, epilepsy begins in early childhood, usually between ages 3 and 5. The most common seizure type is tonic seizures, which cause the muscles to stiffen (contract) uncontrollably. These seizures typically occur during sleep; they may also occur during wakefulness and cause sudden falls. Also common are atypical absence seizures, which cause a very brief partial or complete loss of consciousness. Additionally, many affected individuals have episodes called drop attacks, which cause sudden falls that can result in serious or life-threatening injuries. Drop attacks may be caused by sudden loss of muscle tone (described as atonic) or by abnormal muscle contraction (described as tonic). Other types of seizures have been reported less frequently in people with Lennox-Gastaut syndrome. Seizures associated with Lennox-Gastaut syndrome often do not respond well to therapy with anti-epileptic medications.Although each seizure episode associated with Lennox-Gastaut syndrome is usually brief, more than two-thirds of affected individuals experience prolonged periods of seizure activity (known as status epilepticus) or episodes of many seizures that occur in a cluster.Most children with Lennox-Gastaut syndrome have intellectual disability or learning problems even before seizures begin. These problems may worsen over time, particularly if seizures are very frequent or severe. Some affected children develop additional neurological abnormalities and behavioral problems. Many also have delayed development of motor skills such as sitting and crawling. As a result of their seizures and intellectual disability, most people with Lennox-Gastaut syndrome require help with the usual activities of daily living. However, a small percentage of affected adults live independently.People with Lennox-Gastaut syndrome have a higher risk of death than their peers of the same age. Although the increased risk is not fully understood, it is partly due to poorly controlled seizures and injuries from falls.

MalaCards based summary: Lennox-Gastaut Syndrome, also known as encephalopathy of childhood, is related to epilepsy and encephalopathy, and has symptoms including hemiplegia An important gene associated with Lennox-Gastaut Syndrome is CHD2 (Chromodomain Helicase DNA Binding Protein 2), and among its related pathways/superpathways are Sweet Taste Signaling and Neuropathic Pain-Signaling in Dorsal Horn Neurons. The drugs Liver Extracts and Rufinamide have been mentioned in the context of this disorder. Affiliated tissues include brain, eye and liver, and related phenotypes are intellectual disability and encephalopathy

NINDS: 52 Lennox-Gastaut syndrome is a severe form of epilepsy. Seizures begin in early childhood, usually before the age of 4 years. Children, adolescents, and adults with Lennox-Gastaut syndrome have multiple types of seizures that vary among individuals. Common seizure types include: tonic seizures (stiffening of the body, upward eye gaze, dilated pupils, and altered breathing patterns) atypical absences (staring spells) atonic seizures (brief loss of muscle tone, which could cause abrupt falls) myoclonic seizures (sudden muscle jerks), and generalized tonic-clonic seizures (muscle stiffness and rhythmic jerking). There may be periods of frequent seizures mixed with relatively seizure-free periods. Although not always present at the onset of seizures, most people with Lennox-Gastaut syndrome experience some degree of impaired intellectual functioning or information processing, along with developmental delays and behavioral disturbances. A particular patten of brain electric activity can be seen using electroencephalogram (EEG). Lennox-Gastaut syndrome can be caused by a variety of conditions, including brain malformations, tuberous sclerosis, perinatal asphyxia, severe head injury, central nervous system infection, and inherited genetic and inherited degenerative or metabolic conditions. In 30-35 percent of individuals, no cause can be found.

GARD: 19 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. 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.

Orphanet: 58 A rare, severe early-onset developmental epileptic encephalopathy characterized by the triad of intellectual impairment, multiple seizure types, and typical electroencephalography (EEG) abnormalities.

Disease Ontology: 11 A childhood electroclinical syndrome that is characterized by frequent seizures and intellectual disability that present in early childhood.

Wikipedia: 75 Lennox-Gastaut syndrome (LGS) is a complex, rare, and severe childhood-onset epilepsy. It is... more...

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 279)
# Related Disease Score Top Affiliating Genes
1 epilepsy 32.0 STXBP1 ST3GAL3 SCN8A SCN2A SCN1A KCNT1
2 encephalopathy 31.9 STXBP1 SCN1A CDKL5
3 west syndrome 31.8 STXBP1 ST3GAL3 SCN8A SCN2A SCN1A KCNT1
4 status epilepticus 31.7 SCN1A KCNQ2 CACNA1A
5 childhood absence epilepsy 31.7 STXBP1 SCN8A SCN2A SCN1A KCNT1 KCNQ2
6 epilepsy with myoclonic-atonic seizures 31.6 SCN1A CHD2
7 dravet syndrome 31.6 STXBP1 SCN8A SCN2A SCN1A KCNT1 KCNQ2
8 focal epilepsy 31.6 SCN8A SCN2A SCN1A KCNT1 KCNQ2 GABRG2
9 autism 31.3 STXBP1 SCN8A SCN2A SCN1A GABRG2 GABRB3
10 autism spectrum disorder 31.3 STXBP1 SCN2A SCN1A MT-ND1 GABRG2 GABRB3
11 developmental and epileptic encephalopathy 94 31.3 CHD2 CACNA1A
12 epilepsy, idiopathic generalized 31.1 STXBP1 SCN8A SCN2A SCN1A KCNT1 KCNQ2
13 febrile seizures 31.1 SCN8A SCN2A SCN1A GABRG2
14 early myoclonic encephalopathy 31.0 STXBP1 SCN8A SCN2A SCN1A MT-ND1 KCNT1
15 developmental and epileptic encephalopathy 30.9 STXBP1 ST3GAL3 SCN8A SCN2A SCN1A KCNT1
16 aceruloplasminemia 30.8 STXBP1 SCN8A DNM1 CACNA1A
17 landau-kleffner syndrome 30.8 STXBP1 SCN2A SCN1A KCNQ2 GABRG2 CDKL5
18 rett syndrome 30.7 STXBP1 SCN8A SCN2A SCN1A GABRB3 DNM1
19 progressive myoclonus epilepsy 30.7 STXBP1 SCN8A SCN2A SCN1A KCNT1 KCNQ2
20 benign epilepsy with centrotemporal spikes 30.7 STXBP1 SCN8A SCN2A SCN1A KCNT1 KCNQ2
21 mitochondrial encephalomyopathy 30.7 MT-ND1 FASTKD2 EPRS1
22 generalized epilepsy with febrile seizures plus 30.6 STXBP1 SCN8A SCN2A SCN1A KCNT1 KCNQ2
23 alcohol-related neurodevelopmental disorder 30.6 STXBP1 CDKL5
24 photosensitive epilepsy 30.3 STXBP1 SCN2A SCN1A KCNT1 KCNQ2 GABRG2
25 peripheral nervous system disease 30.3 SCN8A SCN2A MT-ND1 EPRS1 CACNA1A
26 neurodegeneration with brain iron accumulation 5 11.7
27 trichorhinophalangeal syndrome, type ii 11.5
28 encephalopathy, acute, infection-induced 4 11.4
29 lissencephaly 1 11.3
30 chromosome 15q11-q13 duplication syndrome 11.3
31 developmental and epileptic encephalopathy 90 11.3
32 developmental and epileptic encephalopathy 15 11.3
33 visual epilepsy 11.1
34 exostoses, multiple, type i 11.0
35 ehlers-danlos syndrome, arthrochalasia type, 1 10.8
36 acute necrotizing encephalitis 10.6
37 tuberous sclerosis 1 10.5
38 tuberous sclerosis 2 10.5
39 tuberous sclerosis 10.5
40 chd2-related neurodevelopmental disorders 10.5
41 developmental and epileptic encephalopathy 43 10.5 SCN1A GABRG2 GABRB3
42 familial or sporadic hemiplegic migraine 10.5 SCN1A CACNA1A
43 juvenile absence epilepsy 10.5 SCN1A GABRG2 GABRB3
44 reflex epilepsy 10.5 SCN2A SCN1A GABRG2
45 developmental and epileptic encephalopathy 52 10.5 SCN8A SCN1A CACNA1A
46 generalized epilepsy with febrile seizures plus, type 2 10.5 SCN2A SCN1A GABRG2
47 generalized epilepsy with febrile seizures plus, type 7 10.5 SCN1A KCNT1 GABRG2
48 developmental and epileptic encephalopathy 13 10.5 SCN8A SCN2A SCN1A
49 developmental and epileptic encephalopathy 74 10.5 GABRG2 GABRB3
50 febrile seizures, familial, 11 10.5 SCN1A GABRG2

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:

58 30 (show all 21)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 intellectual disability 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001249
2 encephalopathy 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001298
3 eeg with focal sharp slow waves 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0011195
4 myoclonus 58 30 Frequent (33%) Frequent (79-30%)
HP:0001336
5 mental deterioration 58 30 Frequent (33%) Frequent (79-30%)
HP:0001268
6 bilateral tonic-clonic seizure 58 30 Frequent (33%) Frequent (79-30%)
HP:0002069
7 autistic behavior 58 30 Frequent (33%) Frequent (79-30%)
HP:0000729
8 aggressive behavior 58 30 Frequent (33%) Frequent (79-30%)
HP:0000718
9 hyperactivity 58 30 Frequent (33%) Frequent (79-30%)
HP:0000752
10 abnormal brainstem morphology 58 30 Frequent (33%) Frequent (79-30%)
HP:0002363
11 falls 58 30 Frequent (33%) Frequent (79-30%)
HP:0002527
12 atonic seizure 58 30 Frequent (33%) Frequent (79-30%)
HP:0010819
13 atypical absence seizure 58 30 Frequent (33%) Frequent (79-30%)
HP:0007270
14 personality disorder 58 30 Frequent (33%) Frequent (79-30%)
HP:0012075
15 generalized tonic seizure 30 Frequent (33%) HP:0010818
16 focal-onset seizure 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0007359
17 generalized myoclonic seizure 30 Occasional (7.5%) HP:0002123
18 eeg abnormality 58 Frequent (79-30%)
19 behavioral abnormality 58 Frequent (79-30%)
20 generalized myoclonic seizures 58 Occasional (29-5%)
21 generalized tonic seizures 58 Frequent (79-30%)

UMLS symptoms related to Lennox-Gastaut Syndrome:


hemiplegia

MGI Mouse Phenotypes related to Lennox-Gastaut Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 nervous system MP:0003631 10.1 CACNA1A CDKL5 CUX2 DNM1 GABRB3 GABRG2
2 normal MP:0002873 10.02 CACNA1A CHD2 DNM1 EPRS1 GABRB3 GABRG2
3 behavior/neurological MP:0005386 9.97 CACNA1A CDKL5 CHD2 DNM1 GABRB3 GABRG2
4 no phenotypic analysis MP:0003012 9.87 CACNA1A CDKL5 CUX2 DNM1 GABRB3 MAPK10
5 respiratory system MP:0005388 9.5 CACNA1A CHD2 EPRS1 KCNQ2 SCN1A SCN2A
6 mortality/aging MP:0010768 9.47 CACNA1A CHD2 CUX2 DNM1 EPRS1 FASTKD2

Drugs & Therapeutics for Lennox-Gastaut Syndrome

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

(show all 21)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Liver Extracts Phase 4
2
Rufinamide Approved Phase 3 106308-44-5 129228
3
Clobazam Approved, Illicit Phase 3 22316-47-8 2789
4
Topiramate Approved Phase 3 97240-79-4 5284627
5
Sodium fluoride Approved Phase 3 7681-49-4
6
Ethanol Approved Phase 3 64-17-5 702
7 Strawberry Approved Phase 3
8
Benzocaine Approved, Investigational Phase 3 1994-09-7, 94-09-7 2337
9
Tannic acid Approved Phase 3 1401-55-4 16129878 16129778
10
Fenfluramine Approved, Illicit, Investigational, Withdrawn Phase 3 458-24-2 3337
11
Ezogabine Approved, Investigational Phase 3 150812-12-7 121892
12 Sodium Channel Blockers Phase 3
13 Diuretics, Potassium Sparing Phase 3
14 Anti-Anxiety Agents Phase 3
15 Psychotropic Drugs Phase 3
16 GABA Agonists Phase 3
17 Hypoglycemic Agents Phase 3
18 Listerine Phase 3
19 Neurotransmitter Agents Phase 3
20 Serotonin Uptake Inhibitors Phase 3
21
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202

Interventional clinical trials:

(show all 50)
# Name Status NCT ID Phase Drugs
1 Transcranial Direct Current Stimulation for Treatment of Childhood Pharmacoresistant Lennox-Gastaut Syndrome, A Pilot Study Completed NCT02731300 Phase 4
2 A Long-term Safety Study to Assess the Potential for Chronic Liver Injury in Participants Treated With Epidiolex (Cannabidiol) Oral Solution Recruiting NCT05044819 Phase 4 Cannabidiol
3 Double Blind, Randomised, Cross-over Study Melatonin Versus Placebo in the Lennox-Gastaut Syndrome: Neurophysiological and Neuropsychological Effects Withdrawn NCT01370486 Phase 4 melatonin;placebo
4 An Open-Label Exploratory Investigation of Cognitive Outcomes With Cannabidiol Oral Solution (EPIDIOLEX®; GWP42003-P) Withdrawn NCT04133480 Phase 4 GWP42003-P
5 A Prospective Multi-Center Single-Arm Clinical Trial on Cognitive Effect of Cannabidiol (CBD-OS®) on Dravet Syndrome and Lennox-Gastaut Syndrome Unknown status NCT04611438 Phase 3 Cannabidiol
6 Double-Blind, Placebo-Controlled, Efficacy and Safety Study of Clobazam in Patients With Lennox-Gastaut Syndrome Completed NCT00518713 Phase 3 Clobazam Low Dose;Clobazam Medium Dose;Clobazam High Dose;Placebo
7 A Double-Blind Trial of Topiramate in Subjects With Lennox-Gastaut Syndrome. Completed NCT00236756 Phase 3 topiramate
8 Phase III Randomized, Double-Blind, Placebo-Controlled Study of Oral Topiramate for Lennox-Gastaut Syndrome Completed NCT00004776 Phase 3 topiramate
9 A Placebo-Controlled, Double-Blind Comparative Study of E2080 in Lennox-Gastaut Syndrome Patients Completed NCT01146951 Phase 3 Rufinamide (E2080);Placebo
10 Safety and Effectiveness of Open-Label Clobazam in Subjects With Lennox-Gastaut Syndrome Completed NCT01160770 Phase 3 Clobazam
11 A Randomized, Double-blind, Placebo-controlled 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 20 mg/kg/day Dose;Placebo
12 A Multicenter, Randomized, Controlled, Open-label Study to Evaluate the Cognitive Development Effects and Safety, and Pharmacokinetics of Adjunctive Rufinamide Treatment in Pediatric Subjects 1 to Less Than 4 Years of Age With Inadequately Controlled Lennox-Gastaut Syndrome Completed NCT01405053 Phase 3 Rufinamide;Any other approved Antiepileptic Drug
13 A Randomized, Double-blind, Placebo-controlled 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
14 A Long Term Extension Study of E2080 in Lennox-Gastaut Patients Completed NCT01151540 Phase 3 Rufinamide
15 An Open Label Extension Study to Investigate the Safety of Cannabidiol (GWP42003-P; CBD) in Children and Adults With Inadequately Controlled Dravet or Lennox-Gastaut Syndromes. Completed NCT02224573 Phase 3 GWP42003-P
16 A Randomized, Double-blind, Placebo-Controlled Study to Investigate the Efficacy and Safety of Carisbamate (YKP509) as Adjunctive Treatment for Seizures Associated With Lennox-Gastaut Syndrome in Children and Adults, With Optional Open-Label Extension Recruiting NCT05219617 Phase 3 Carisbamate
17 A Phase 3, Prospective, Open-Label, Multisite, Extension of Phase 3 Studies To Assess the Long-Term Safety and Tolerability of Soticlestat as Adjunctive Therapy in Subjects With Dravet Syndrome or Lennox-Gastaut Syndrome (ENDYMION 2) Recruiting NCT05163314 Phase 3 Soticlestat
18 A Multicenter, Randomized, Double-Blind, Placebo-Controlled, Parallel Group Study to Evaluate the Efficacy, Safety, and Tolerability of Soticlestat as Adjunctive Therapy in Pediatric and Adult Subjects With Lennox-Gastaut Syndrome (LGS) Recruiting NCT04938427 Phase 3 Soticlestat;Placebo
19 A Two-Part Study of ZX008 in Children and Adults With Lennox-Gastaut Syndrome (LGS); Part 1: A Randomized, Double-blind, Placebo-controlled Trial of Two Fixed Doses of ZX008 (Fenfluramine Hydrochloride) Oral Solution as Adjunctive Therapy for Seizures in Children and Adults With LGS, Followed by Part 2: An Open-label Extension to Assess Long-Term Safety of ZX008 in Children and Adults With LGS Active, not recruiting NCT03355209 Phase 3 ZX008 0.2 or 0.8 mg/kg/day;Matching Placebo
20 An Open-Label Extension Trial to Assess the Long-Term Safety of ZX008 (Fenfluramine Hydrochloride) Oral Solution as an Adjunctive Therapy for Seizures in Patients With Rare Seizure Disorders Such as Epileptic Encephalopathies Including Dravet Syndrome and Lennox-Gastaut Syndrome Active, not recruiting NCT03936777 Phase 3 ZX008 (Fenfluramine Hydrochloride)
21 RTG113388, a Long-term, Open-label Safety Extension Study of Retigabine/Ezogabine in Pediatric Subjects With Partial Onset Seizures (>= 12 Years Old) and Subjects With Lennox-Gastaut Syndrome (>=12 Years Old) Terminated NCT01668654 Phase 3 retigabine/ezogabine
22 A Multicenter, Double-Blind, Randomized, Placebo-Controlled Trial With an Open-Label Extension Phase of Perampanel as Adjunctive Treatment in Subjects at Least 2 Years of Age With Inadequately Controlled Seizures Associated With Lennox-Gastaut Syndrome Terminated NCT02834793 Phase 3 Placebo;Perampanel
23 A Multicenter, Randomized, Double-blind, Placebo-controlled, Interventional Study to Assess the Safety and Efficacy of Pharmaceutical Cannabidiol Oral Solution as an Adjunctive Therapy for Treatment of Subjects With Inadequately Controlled Lennox-Gastaut Syndrome Withdrawn NCT02318537 Phase 3 Cannabidiol Oral Solution;Placebo Solution
24 Safety and Efficacy of Clobazam in Subjects With Lennox-Gastaut Syndrome Completed NCT00162981 Phase 2 Clobazam Low Dose;Clobazam High Dose
25 A Phase 2, Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy, Safety, and Tolerability of TAK-935 (OV935) as an Adjunctive Therapy in Pediatric Patients With Developmental and/or Epileptic Encephalopathies Completed NCT03650452 Phase 2 TAK-935;Placebo
26 RNS System Feasibility Study of Thalamocortical Brain-Responsive Neurostimulation for the Treatment of Lennox-Gastaut Syndrome Recruiting NCT05339126 Phase 2
27 A Phase 2, Multicenter, Open-label, Long-term Safety Study of LP352 in Subjects With Developmental and Epileptic Encephalopathy Who Completed Study LP352-201 and Are Candidates for Continuous Treatment for Up to 52 Weeks Recruiting NCT05626634 Phase 2 LP352
28 Randomized, Double-blind, Placebo-controlled, Parallel-group, Dose-escalation Study to Investigate the Safety, Tolerability, PK, PD, and Exploratory Efficacy of LP352 in Subjects With Developmental and Epileptic Encephalopathies Recruiting NCT05364021 Phase 1, Phase 2 LP352;Placebo
29 A Phase 2, Prospective, Interventional, Open-Label, Multi-Site, Extension Study to Assess the Long-Term Safety and Tolerability of Soticlestat (TAK-935) as Adjunctive Therapy in Subjects With Developmental Epileptic Encephalopathies Including Dravet Syndrome, Lennox Gastaut Syndrome, CDKL5 Deficiency Disorder, and Chromosome 15 Duplication Syndrome (ENDYMION 1) Active, not recruiting NCT03635073 Phase 2 Soticlestat
30 Add-on Therapy With Low Dose Fenfluramine in Lennox Gastaut Epilepsy Active, not recruiting NCT02655198 Phase 2 Fenfluramine
31 A 20-Week Multicenter, Randomized, Double-Blind, Placebo-Controlled, Proof of Concept Trial of EPX-100 (Clemizole Hydrochloride) as Adjunctive Therapy in Children With Lennox-Gastaut Syndrome Not yet recruiting NCT05066217 Phase 2 Clemizole HCl;Placebo to match EPX-100
32 Open-label, Multiple Dose Study to Evaluate the Parmacokinetics, Safety and Tolerability of Ezogabine/Retigabine as Adjunctive Treatment in Subjects Aged From 12 Years to Less Than 18 Years With Partial Onset Seizures or Lennox-Gastaut Syndrome Terminated NCT01494584 Phase 2 ezogabine/retigabine
33 The Effects of Cannabidiol (CBD) on Electrical and Autonomic Cardiac Function in Children Terminated NCT02815540 Phase 1, Phase 2 Cannabidiol
34 An Open-Label Trial to Assess the Safety of ZX008 (Fenfluramine Hydrochloride) Oral Solution in Combination With Cannabidiol, as an Adjunctive Therapy in Children and Young Adults With Dravet Syndrome or Lennox-Gastaut Syndrome Completed NCT03467113 Phase 1 ZX008 0.2 to 0.8 mg/kg/day;Cannabidiol
35 Phase I, Open-Label, Pharmacokinetic, Dose Escalation Study of Carisbamate in Adult and Pediatric Subjects With Lennox-Gastaut Syndrome Recruiting NCT03731715 Phase 1 Carisbamate
36 Phase 1, Open-Label Study of Carisbamate in Adult and Pediatric Subjects With Lennox-Gastaut Syndrome Active, not recruiting NCT04062981 Phase 1 Carisbamate
37 The Clinical Research on PINS Vagus Nerve Stimulator for Treatment of Lennox-Gastaut in Children Unknown status NCT02632149 Early Phase 1
38 European Registry of Anti-epileptic Drug Use in Patients With Lennox-Gastaut Syndrome (LGS). Completed NCT01991041
39 An Open-label Observation Study of Topiramate Administration as Adjuvant Therapy for Focal Epilepsy, Lennox-Gastaut Syndrome Epileptic Seizures and Generalized Tonoclonic Seizures in Adults and Children Aged 2 Years and Older Completed NCT00297349 Topiramate
40 Post-marketing Surveillance of Long-term Administration of Inovelon Tablets in Patients With Lennox-Gastaut Syndrome Completed NCT02175173 Rufinamide
41 Epilepsy Phenome/Genome Project: A Phenotype/Genotype Analysis of Epilepsy Completed NCT00552045
42 Efficacy of the Ketogenic Diet -- a Blinded Study Completed NCT00004729
43 An Extended Access Program (EAP) for Rufinamide in Pediatric Participants With Inadequately Controlled Lennox-Gastaut Syndrome Available NCT03778424 Rufinamide
44 An Extended Access Program (EAP) for Perampanel Available NCT02307578 Perampanel
45 Comparative Effectiveness of Palliative Surgery Versus Additional Anti-Seizure Medications for Lennox-Gastaut Syndrome - PCORI Not yet recruiting NCT05374824
46 Observational, Prospective, Multicenter Study of Epidyolex® (Cannabidiol CBD 100 mg/ml) Oral Solution, as Adjunctive Treatment for Seizures Associated With Lennox-Gastaut Syndrome (LGS) and Dravet Syndrome (DS) Not yet recruiting NCT05485831 Epidiolex 100 mg/mL Oral Solution
47 Children's Adaptive Deep Brain Stimulation for Epilepsy Trial (CADET): Pilot Not yet recruiting NCT05437393
48 Long-term Cardiac Monitoring in Epilepsy: Comparative Group Study to Assess Risk of Interictal and Ictal Cardiac Dysfunction Suspended NCT03955432
49 Turmeric as Treatment in Epilepsy Withdrawn NCT03254680
50 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 71 / NDF-RT 50 :


clobazam
Clonazepam
COLBAZAM

Cochrane evidence based reviews: lennox gastaut syndrome

Genetic Tests for Lennox-Gastaut Syndrome

Anatomical Context for Lennox-Gastaut Syndrome

Organs/tissues related to Lennox-Gastaut Syndrome:

MalaCards : Brain, Eye, Liver, Cortex, Prefrontal Cortex, Spinal Cord, Thalamus

Publications for Lennox-Gastaut Syndrome

Articles related to Lennox-Gastaut Syndrome:

(show top 50) (show all 1599)
# Title Authors PMID Year
1
SCN1A mutation screening in adult patients with Lennox-Gastaut syndrome features. 53 62
19782004 2009
2
Therapeutic and clinical foundations of cannabidiol therapy for difficult-to-treat seizures in children and adults with refractory epilepsies. 62
36206806 2023
3
Clinical efficacy and safety of cannabidiol for pediatric refractory epilepsy indications: A systematic review and meta-analysis. 62
36206805 2023
4
Centromedian thalamic deep brain stimulation for drug-resistant epilepsy: single-center experience. 62
35395630 2022
5
Outcomes from a Spanish Expanded Access Program on cannabidiol treatment in pediatric and adult patients with epilepsy. 62
36327646 2022
6
Current and future pharmacotherapy options for drug-resistant epilepsy. 62
36154780 2022
7
Lennox Gastaut Syndrome - A strategic shift in diagnosis over time? 62
36332460 2022
8
Modified Atkins diet versus levetiracetam for non-surgical drug-resistant epilepsy in children: A randomized open-label study. 62
36306706 2022
9
Specialty pharmacy integration and the role of an advanced certified pharmacy technician in prescription cannabidiol access. 62
35985958 2022
10
Electroencephalography-functional magnetic resonance imaging of epileptiform discharges: Noninvasive investigation of the whole brain. 62
35822919 2022
11
An Italian consensus on the management of Lennox-Gastaut syndrome. 62
35981474 2022
12
Positive Impact of Inpatient Respiratory Rehabilitation in a Rare Case of Acute Necrotizing Encephalopathy. 62
36407176 2022
13
Management of Lennox-Gastaut syndrome with deep brain stimulation: A systematic literature review. 62
36252974 2022
14
Tonic Seizures in a Patient With Lennox-Gastaut Syndrome Manifest as "Icicles" Rather Than "Flames" on Quantitative EEG Analysis. 62
36308754 2022
15
Cognition, adaptive skills and epilepsy disability/severity in patients with Lennox-Gastaut syndrome undergoing deep brain stimulation for epilepsy in the ESTEL trial. 62
35932526 2022
16
A novel homozygous missense mutation in the FASTKD2 gene leads to Lennox-Gastaut syndrome. 62
35729327 2022
17
Psychobehavioural and Cognitive Adverse Events of Anti-Seizure Medications for the Treatment of Developmental and Epileptic Encephalopathies. 62
36194365 2022
18
Fenfluramine provides clinically meaningful reduction in frequency of drop seizures in patients with Lennox-Gastaut syndrome: Interim analysis of an open-label extension study. 62
36196777 2022
19
A phase 2, randomized, double-blind, placebo-controlled study to evaluate the efficacy and safety of soticlestat as adjunctive therapy in pediatric patients with Dravet syndrome or Lennox-Gastaut syndrome (ELEKTRA). 62
35841234 2022
20
Design and synthesis of aryl-piperidine derivatives as potent and selective PET tracers for cholesterol 24-hydroxylase (CH24H). 62
35863274 2022
21
Effects of Cannabidiol on Adaptive Behavior and Quality of Life in Pediatric Patients With Treatment-Resistant Epilepsy. 62
36062772 2022
22
Long-term outcome of developmental and epileptic encephalopathies. 62
35489823 2022
23
Characterization of cortical activity in juvenile myoclonic epilepsy by gradient magnetic field topography. 62
35853311 2022
24
Effect of fenfluramine on seizures and comorbidities in SCN8A-developmental and epileptic encephalopathy: A case series. 62
35802036 2022
25
Paroxysmal fast activity is a biomarker of treatment response in deep brain stimulation for Lennox-Gastaut syndrome. 62
36114808 2022
26
Perampanel as adjuvant treatment in epileptic encephalopathies: A multicenter study in routine clinical practice. 62
35870432 2022
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Prescription cannabidiol for seizure disorder management: Initial drug-drug interaction management by specialty pharmacists. 62
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Obstructive and central sleep apnoea in a patient with medically intractable epilepsy. 62
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Combined Neuromodulation (Vagus Nerve Stimulation and Deep Brain Stimulation) in Patients With Refractory Generalized Epilepsy: An Observational Study. 62
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Complete Corpus Callosotomy for Refractory Epilepsy in Children. 62
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Safety and efficacy of cathodal transcranial direct current stimulation in patients with Lennox Gastaut Syndrome: An open-label, prospective, single-center, single-blinded, pilot study. 62
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Callosotomy vs Vagus Nerve Stimulation in the Treatment of Lennox-Gastaut Syndrome: A Systematic Review With Meta-Analysis. 62
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Correction: DBS of Thalamic Centromedian Nucleus for Lennox-Gastaut Syndrome (ESTEL Trial). 62
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Diagnosing Lennox-Gastaut syndrome in an adult and its direct impact in epilepsy care 62
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Variable Expression of GABAA Receptor Subunit Gamma 2 Mutation in a Nuclear Family Displaying Developmental and Encephalopathic Phenotype. 62
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Systematic studies in acute necrotizing encephalopathy of childhood: The way forward. 62
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Outcomes in influenza and RANBP2 mutation-associated acute necrotizing encephalopathy of childhood. 62
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Fever, Seizures and Encephalopathy: From Bush Fires to Firestorms. 62
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Molecular Insights into the Role of Pathogenic nsSNPs in GRIN2B Gene Provoking Neurodevelopmental Disorders. 62
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Different epilepsy course of a novel AHDC1 mutation in a female monozygotic twin pair. 62
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Short-term and long-term efficacy and safety of antiseizure medications in Lennox Gastaut syndrome: A network meta-analysis. 62
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Does Deep Brain Stimulation Work in Lennox-Gastaut Syndrome? Well…it Depends. 62
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Epilepsy syndromes in cerebral palsy: varied, evolving and mostly self-limited. 62
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[Optimal Use of Perampanel in the Treatment of Patients with Epilepsy Based on the Clinical Evidence and Characteristics]. 62
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The Optimal Target and Connectivity for Deep Brain Stimulation in Lennox-Gastaut Syndrome. 62
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International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions. 62
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Efficacy and Safety of Fenfluramine for the Treatment of Seizures Associated With Lennox-Gastaut Syndrome: A Randomized Clinical Trial. 62
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Variations for Lennox-Gastaut Syndrome

ClinVar genetic disease variations for Lennox-Gastaut Syndrome:

5
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 GABRG2 NM_198904.4(GABRG2):c.406C>T (p.Arg136Ter) SNV Pathogenic
205540 rs796052504 GRCh37: 5:161524722-161524722
GRCh38: 5:162097716-162097716
2 CHD2 NM_001271.4(CHD2):c.3199dup (p.Ile1067fs) DUP Pathogenic
1705832 GRCh37: 15:93527689-93527690
GRCh38: 15:92984459-92984460
3 DNM1 NM_004408.4(DNM1):c.590-2A>G SNV Pathogenic
1705841 GRCh37: 9:130982265-130982265
GRCh38: 9:128219986-128219986
4 MAPK10 MAPK10, EX10-14DEL DEL Uncertain Significance
6746 GRCh37:
GRCh38:
5 GATM NM_001482.3(GATM):c.367G>A (p.Ala123Thr) SNV Uncertain Significance
560642 rs1566840902 GRCh37: 15:45661641-45661641
GRCh38: 15:45369443-45369443
6 ZEB2 NM_014795.4(ZEB2):c.3267A>C (p.Glu1089Asp) SNV Uncertain Significance
534635 rs1553960775 GRCh37: 2:145147396-145147396
GRCh38: 2:144389829-144389829
7 POLG NM_002693.3(POLG):c.1402A>G (p.Asn468Asp) SNV Uncertain Significance
206596 rs145843073 GRCh37: 15:89870429-89870429
GRCh38: 15:89327198-89327198
8 KCNQ3 NM_004519.4(KCNQ3):c.688C>T (p.Arg230Cys) SNV Not Provided
205963 rs796052676 GRCh37: 8:133192493-133192493
GRCh38: 8:132180246-132180246
9 SCN1A, LOC102724058 NM_001165963.4(SCN1A):c.4907G>A (p.Arg1636Gln) SNV Not Provided
68557 rs121917995 GRCh37: 2:166848878-166848878
GRCh38: 2:165992368-165992368

Expression for Lennox-Gastaut Syndrome

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

Pathways for Lennox-Gastaut Syndrome

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

(show all 11)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.82 MAPK10 KCNT1 GABRG2 GABRB3 CDKL5 CACNA1A
2
Show member pathways
12.39 SCN8A SCN2A SCN1A MAPK10 KCNT1
3
Show member pathways
12.38 SCN8A SCN2A SCN1A CACNA1A
4 12.3 KCNQ2 SCN1A SCN2A SCN8A STXBP1
5
Show member pathways
11.83 SCN8A SCN2A SCN1A KCNQ2 DNM1
6 11.32 STXBP1 DNM1 CACNA1A
7 11.03 SCN8A SCN2A SCN1A
8 10.79 STXBP1 SCN1A CHD2
9 10.75 SCN8A SCN2A SCN1A KCNQ2
10 10.71 GABRG2 GABRB3
11 10.7 STXBP1 SCN8A SCN2A SCN1A CDKL5

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 membrane GO:0016020 10.48 CACNA1A GABRB3 GABRG2 GPR55 KCNQ2 KCNT1
2 membrane GO:0016021 10.48 CACNA1A GABRB3 GABRG2 GPR55 KCNQ2 KCNT1
3 axon GO:0030424 10.1 STXBP1 SCN8A SCN2A SCN1A GABRG2
4 glutamatergic synapse GO:0098978 10.07 STXBP1 SCN2A GABRG2 DNM1 CDKL5
5 axon initial segment GO:0043194 9.63 SCN8A SCN1A KCNQ2
6 sodium channel complex GO:0034706 9.43 SCN2A SCN1A
7 voltage-gated sodium channel complex GO:0001518 9.43 SCN8A SCN2A SCN1A
8 node of Ranvier GO:0033268 9.23 SCN8A SCN2A SCN1A KCNQ2

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

# Name GO ID Score Top Affiliating Genes
1 sodium ion transmembrane transport GO:0035725 10.03 SLC6A19 SCN8A SCN2A SCN1A
2 modulation of chemical synaptic transmission GO:0050804 9.88 DNM1 CDKL5 CACNA1A
3 neuronal action potential GO:0019228 9.88 SCN8A SCN2A SCN1A
4 transmembrane transport GO:0055085 9.85 SLC6A19 SCN8A SCN2A SCN1A KCNQ2 CACNA1A
5 regulation of monoatomic ion transmembrane transport GO:0034765 9.85 SCN8A SCN2A SCN1A KCNQ2 CACNA1A
6 cellular response to histamine GO:0071420 9.76 GABRG2 GABRB3
7 membrane depolarization during action potential GO:0086010 9.73 SCN1A SCN2A SCN8A
8 monoatomic cation transmembrane transport GO:0098655 9.62 SCN8A SCN2A SCN1A CACNA1A
9 monoatomic ion transport GO:0006811 9.56 SCN8A SCN2A SCN1A KCNT1 KCNQ2 GABRG2
10 monoatomic ion transmembrane transport GO:0034220 9.28 SCN8A SCN2A SCN1A KCNQ2 GABRG2 GABRB3

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

# Name GO ID Score Top Affiliating Genes
1 voltage-gated sodium channel activity GO:0005248 9.8 SCN8A SCN2A SCN1A
2 voltage-gated monoatomic ion channel activity GO:0005244 9.5 SCN8A SCN2A SCN1A KCNQ2 CACNA1A
3 monoatomic cation channel activity GO:0005261 9.46 SCN8A SCN2A SCN1A CACNA1A
4 sodium channel activity GO:0005272 9.43 SCN8A SCN2A SCN1A
5 monoatomic ion channel activity GO:0005216 9.17 SCN8A SCN2A SCN1A KCNQ2 GABRG2 GABRB3

Sources for Lennox-Gastaut Syndrome

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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