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LGS
MCID: LNN001
MIFTS: 60

Lennox-Gastaut Syndrome (LGS)

Categories: Metabolic diseases, Neuronal diseases, Rare 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 6 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
Lennoxgastaut Syndrome 76
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 Metabolic 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 begin in ealry 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.

MalaCards based summary : Lennox-Gastaut Syndrome, also known as epileptic encephalopathy lennox-gastaut type, is related to epilepsy with myoclonic-atonic seizures and epilepsy, 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, testes and eye, and related phenotypes are intellectual disability and generalized myoclonic seizures

Disease Ontology : 12 A childhood electroclinical syndrome that is characterized by frequent seizures and intellectual disability that present in early childhood.

Genetics Home Reference : 25 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.

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.

Wikipedia : 76 Lennox�??Gastaut syndrome (LGS) is a complex, rare, and severe childhood-onset epilepsy. It is... 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 81)
# Related Disease Score Top Affiliating Genes
1 epilepsy with myoclonic-atonic seizures 31.2 CHD2 SCN1A
2 epilepsy 31.0 CHD2 DCX GABRA1 GABRB3 SCN1A SLC2A1
3 focal epilepsy 30.7 SCN1A SLC2A1 TBC1D24
4 west syndrome 30.7 ALG13 MT-ND1 POMC SCN1A ST3GAL3 STXBP1
5 epileptic encephalopathy, early infantile, 6 30.2 CHD2 GABRA1 GABRB3 SCN1A SNRPE STXBP1
6 neurodegeneration with brain iron accumulation 5 11.9
7 trichorhinophalangeal syndrome, type ii 11.9
8 epileptic encephalopathy, early infantile, 15 11.4
9 scn1a-related seizure disorders 11.4
10 encephalopathy, acute, infection-induced 4 11.4
11 exostoses, multiple, type i 11.1
12 lymphomatoid granulomatosis 11.1
13 continuous spike-wave during slow sleep syndrome 10.9
14 encephalopathy 10.8
15 infantile epileptic encephalopathy 10.7
16 status epilepticus 10.5
17 tuberous sclerosis 10.4
18 acute necrotizing encephalopathy 10.3
19 angelman syndrome 10.2
20 autism 10.2
21 moyamoya disease 1 10.2
22 myoclonic epilepsy of unverricht and lundborg 10.2
23 leigh syndrome 10.2
24 polymicrogyria, bilateral frontoparietal 10.2
25 hepatitis 10.2
26 autism spectrum disorder 10.2
27 hypospadias 10.2
28 encephalomalacia 10.2
29 astrocytoma 10.2
30 acute disseminated encephalomyelitis 10.2
31 measles 10.2
32 tyrosinemia 10.2
33 intracranial hypertension 10.2
34 polymicrogyria 10.2
35 cutis verticis gyrata 10.2
36 dandy-walker complex 10.2
37 hemimegalencephaly 10.2
38 pachygyria 10.2
39 myoclonus 10.2
40 cerebral atrophy 10.2
41 dysphagia 10.2
42 myoclonic-astastic epilepsy 10.2
43 landau-kleffner syndrome 10.2 POMC TBC1D24
44 epilepsy with generalized tonic-clonic seizures 10.1 SCN1A TBC1D24
45 childhood disintegrative disease 10.1 GABRB3 POMC
46 generalized epilepsy with febrile seizures plus 10.1 GABRA1 SCN1A TBC1D24
47 benign epilepsy with centrotemporal spikes 10.1 CHD2 SLC2A1 TBC1D24
48 infancy electroclinical syndrome 10.1 POMC SCN1A STXBP1 TBC1D24
49 macrocephaly/megalencephaly syndrome, autosomal recessive 10.1 AKT3 STXBP1
50 lesch-nyhan syndrome 10.1

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



Diseases related to Lennox-Gastaut Syndrome
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Symptoms & Phenotypes for Lennox-Gastaut Syndrome

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Human phenotypes related to Lennox-Gastaut Syndrome:

59 32 (show all 20)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 intellectual disability 59 32 hallmark (90%) Very frequent (99-80%) HP:0001249
2 generalized myoclonic seizures 59 32 occasional (7.5%) Occasional (29-5%) HP:0002123
3 myoclonus 59 32 frequent (33%) Frequent (79-30%) HP:0001336
4 falls 59 32 frequent (33%) Frequent (79-30%) HP:0002527
5 generalized tonic-clonic seizures 59 32 frequent (33%) Frequent (79-30%) HP:0002069
6 aggressive behavior 59 32 frequent (33%) Frequent (79-30%) HP:0000718
7 mental deterioration 59 32 frequent (33%) Frequent (79-30%) HP:0001268
8 encephalopathy 59 32 hallmark (90%) Very frequent (99-80%) HP:0001298
9 autistic behavior 59 32 frequent (33%) Frequent (79-30%) HP:0000729
10 hyperactivity 59 32 frequent (33%) Frequent (79-30%) HP:0000752
11 abnormality of brainstem morphology 59 32 frequent (33%) Frequent (79-30%) HP:0002363
12 eeg with focal sharp slow waves 59 32 hallmark (90%) Very frequent (99-80%) HP:0011195
13 atypical absence seizures 59 32 frequent (33%) Frequent (79-30%) HP:0007270
14 generalized tonic seizures 59 32 frequent (33%) Frequent (79-30%) HP:0010818
15 atonic seizures 59 32 frequent (33%) Frequent (79-30%) HP:0010819
16 personality disorder 59 32 frequent (33%) Frequent (79-30%) HP:0012075
17 eeg abnormality 59 Frequent (79-30%)
18 behavioral abnormality 59 Frequent (79-30%)
19 focal seizures 59 Occasional (29-5%)
20 focal-onset seizure 32 occasional (7.5%) HP:0007359

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 10.1 ADGRG1 AKT3 CHD2 DCX DNM1 GABRA1
2 mortality/aging MP:0010768 9.93 AKT3 CHD2 CUX2 DCX DNM1 GABRA1
3 nervous system MP:0003631 9.77 ADGRG1 AKT3 CUX2 DCX DNM1 GABRA1
4 no phenotypic analysis MP:0003012 9.28 CUX2 DNM1 GABRA1 GABRB3 MAPK10 POMC
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 Strawberry Approved Phase 3
7 tannic acid Approved Phase 3
8
Benzocaine Approved, Investigational Phase 3 94-09-7, 1994-09-7 2337
9 sodium fluoride Approved Phase 3 7681-49-4
10
Clobazam Approved, Illicit Phase 3,Phase 2 22316-47-8 2789
11
Topiramate Approved Phase 3,Phase 1 97240-79-4 5284627
12
Lamotrigine Approved, Investigational Phase 3 84057-84-1 3878
13
Valproic Acid Approved, Investigational Phase 3 99-66-1 3121
14
Ezogabine Approved, Investigational Phase 3,Phase 2 150812-12-7 121892
15 Anticonvulsants Phase 3,Phase 2,Phase 1
16 Pharmaceutical Solutions Phase 3,Phase 1,Phase 2
17 Listerine Phase 3
18 Neuroprotective Agents Phase 3,Phase 1
19 Anti-Obesity Agents Phase 3,Phase 1
20 Diuretics, Potassium Sparing Phase 3
21 Excitatory Amino Acids Phase 3
22 calcium channel blockers Phase 3
23 Sodium Channel Blockers Phase 3
24 Excitatory Amino Acid Antagonists Phase 3
25 Calcium, Dietary Phase 3
26 Neurotransmitter Agents Phase 3,Phase 1,Phase 2
27 Serotonin Agents Phase 3,Phase 1,Phase 2
28 Serotonin Uptake Inhibitors Phase 3,Phase 1,Phase 2
29 Neurotransmitter Uptake Inhibitors Phase 3,Phase 1,Phase 2
30
Serotonin Investigational, Nutraceutical Phase 3,Phase 1,Phase 2 50-67-9 5202
31
Dronabinol Approved, Illicit Phase 1 1972-08-3 16078
32 Fluorodeoxyglucose F18

Interventional clinical trials:

(show all 40)
# 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 Efficacy and Safety of GWP42003-P for Seizures Associated With Lennox-Gastaut Syndrome in Children and Adults Completed NCT02224560 Phase 3 GWP42003-P;Placebo control
6 Safety and Effectiveness of Open-Label Clobazam in Subjects With Lennox-Gastaut Syndrome Completed NCT01160770 Phase 3 Clobazam
7 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 20 mg/kg/day Dose;Placebo
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 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 Recruiting NCT03650452 Phase 2 TAK-935;Placebo
22 A Phase 2, Prospective, Interventional, Open-Label, Multi-Site, Extension Study to Assess the Long-Term Safety and Tolerability of TAK-935 (OV935) as Adjunctive Therapy in Patients With Rare Epilepsy Recruiting NCT03635073 Phase 2 TAK-935
23 A Study to Assess the Safety and Tolerability of ZX008 in Children and Young Adults With DS or LGS Currently Taking CBD Active, not recruiting NCT03467113 Phase 1, Phase 2 ZX008 02 and 0.8 mg/kg/day
24 Add-on Therapy With Low Dose Fenfluramine in Lennox Gastaut Epilepsy Active, not recruiting NCT02655198 Phase 2 Fenfluramine
25 Study in Pediatric Subjects With Epilepsy Terminated NCT01494584 Phase 2 ezogabine/retigabine
26 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
27 Carisbamate in Adult & Pediatric Subjects With Lennox-Gastaut Syndrome Recruiting NCT03731715 Phase 1 Carisbamate
28 Cannabidiol in Children With Refractory Epileptic Encephalopathy Recruiting NCT03024827 Phase 1 CanniMed® 1:20
29 Trial to Assess Vagus Nerve Stimulation Therapy in Children With Lennox-Gastaut Syndrome Unknown status NCT02632149 Early Phase 1
30 The Effects of Cannabidiol (CBD) on Electrical and Autonomic Cardiac Function in Children With Severe Epilepsy Unknown status NCT02815540 Not Applicable Cannabadiol
31 European Registry of Anti-Epileptic Drug Use in Patients With Lennox-Gastaut Syndrome (LGS) Completed NCT01991041
32 A Study of the Safety of Topiramate Given in Combination With Other Medications in Adults and Children With Seizures Completed NCT00297349 Topiramate
33 Ketogenic Diet for Child Epilepsy and Seizure Control Completed NCT00004729 Not Applicable
34 Special Drug Use Investigation for LAMICTAL® (Long Term) Completed NCT01863602 Lamotrigine tablets
35 Epilepsy Phenome/Genome Project Completed NCT00552045
36 Metabolic Abnormalities in Children With Epilepsy Completed NCT00001325 18 FDG
37 Post-marketing Surveillance of Long-term Administration of Inovelon Tablets in Patients With Lennox-Gastaut Syndrome Recruiting NCT02175173 Rufinamide
38 An Extended Access Program (EAP) for Participants Who Have Completed Rufinamide Study E2080-G000-303 Available NCT03778424 Rufinamide
39 The Pharmacokinetics of Cannabidiol (CBD) and Its Effects in Children With Severe Epilepsy Withdrawn NCT02910297
40 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
Sources

Anatomical Context for Lennox-Gastaut Syndrome

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MalaCards organs/tissues related to Lennox-Gastaut Syndrome:

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

Publications for Lennox-Gastaut Syndrome

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Articles related to Lennox-Gastaut Syndrome:

(show top 50) (show all 354)
# Title Authors Year
1
Lennox-Gastaut Syndrome in Mitochondrial Disease. ( 30554497 )
2019
2
Addition of Cannabidiol to Current Antiepileptic Therapy Reduces Drop Seizures in Children and Adults With Treatment-Resistant Lennox-Gastaut Syndrome. ( 29887290 )
2018
3
Changes in functional brain network topology after successful and unsuccessful corpus callosotomy for Lennox-Gastaut Syndrome. ( 29467376 )
2018
4
Cannabidiol for drop seizures in Lennox-Gastaut syndrome. ( 29395274 )
2018
5
Optimizing clobazam treatment in patients with Lennox-Gastaut syndrome. ( 29202277 )
2018
6
Few individuals with Lennox-Gastaut syndrome have autism spectrum disorder: a comparison with Dravet syndrome. ( 29558884 )
2018
7
Cannabidiol in patients with seizures associated with Lennox-Gastaut syndrome (GWPCARE4): a randomised, double-blind, placebo-controlled phase 3 trial. ( 29395273 )
2018
8
Effect of Cannabidiol on Drop Seizures in the Lennox-Gastaut Syndrome. ( 29768152 )
2018
9
Rufinamide for the treatment of Lennox-Gastaut syndrome: evidence from clinical trials and clinical practice. ( 29313492 )
2018
10
The refractory epilepsy screening tool for Lennox-Gastaut syndrome (REST-LGS). ( 30537670 )
2018
11
Long-term Outcome of Resective Epilepsy Surgery in Patients With Lennox-Gastaut Syndrome. ( 30194277 )
2018
12
Evaluation of long-term safety, tolerability, and behavioral outcomes with adjunctive rufinamide in pediatric patients (≥1 to <4 years old) with Lennox-Gastaut syndrome: Final results from randomized study 303. ( 30309816 )
2018
13
Improved seizure control and regaining cognitive milestones after vagus nerve stimulation revision surgery in Lennox-Gastaut syndrome. ( 30364578 )
2018
14
The pharmacological management of Lennox-Gastaut syndrome and critical literature review. ( 30391662 )
2018
15
Lennox-Gastaut Syndrome: In a Nutshell. ( 30410820 )
2018
16
Lacosamide-induced excessive laughing in a patient with Lennox-Gastaut syndrome. ( 30057867 )
2018
17
Efficacy and Safety of Adjunctive Cannabidiol in Patients with Lennox-Gastaut Syndrome: A Systematic Review and Meta-Analysis. ( 30132269 )
2018
18
Cannabidiol in the Lennox-Gastaut Syndrome. ( 30134132 )
2018
19
Cannabidiol in the Lennox-Gastaut Syndrome. ( 30136545 )
2018
20
Cannabidiol in the Lennox-Gastaut Syndrome. ( 30136546 )
2018
21
A pilot, open-label study of the effectiveness and tolerability of low-dose ZX008 (fenfluramine HCl) in Lennox-Gastaut syndrome. ( 30146701 )
2018
22
Rufinamide efficacy and safety in children aged 1-4 years with Lennox-Gastaut syndrome. ( 30166208 )
2018
23
Burden of illness in patients with possible Lennox-Gastaut syndrome: A retrospective claims-based study. ( 30241056 )
2018
24
Evolution and course of early life developmental encephalopathic epilepsies: Focus on Lennox-Gastaut syndrome. ( 30255934 )
2018
25
A Case of Lennox-Gastaut Syndrome in a 6-Year-Old Child with Moyamoya Disease. ( 30271075 )
2018
26
Sulthiame add-on therapy in children with Lennox-Gastaut syndrome: A study of 44 patients. ( 30286409 )
2018
27
Lennox-Gastaut Syndrome: A State of the Art Review. ( 28346953 )
2017
28
Assessment of treatment patterns and healthcare costs associated with probable Lennox-Gastaut syndrome. ( 28609734 )
2017
29
Spectral characteristics of intracranial electroencephalographic activity in patients with Lennox-Gastaut syndrome. ( 27686687 )
2017
30
Adult motor phenotype differentiates Dravet syndrome from Lennox-Gastaut syndrome and links SCN1A to early onset parkinsonian features. ( 28186331 )
2017
31
Lennox-Gastaut Syndrome: A Prospective Follow-up Study. ( 28479797 )
2017
32
Dosing considerations for rufinamide in patients with Lennox-Gastaut syndrome: Phase III trial results and real-world clinical data. ( 28284045 )
2017
33
Understanding Lennox-Gastaut syndrome: insights from focal epilepsy patients with Lennox-Gastaut features. ( 28584915 )
2017
34
Early Diagnosis and Treatment of Lennox-Gastaut Syndrome. ( 28689466 )
2017
35
Real-world data on rufinamide treatment in patients with Lennox-Gastaut syndrome: Results from a European noninterventional registry study. ( 28927712 )
2017
36
Lennox-Gastaut syndrome: a comprehensive review. ( 29124439 )
2017
37
Use and cost comparison of clobazam to other antiepileptic drugs for treatment of Lennox-Gastaut syndrome. ( 28740620 )
2017
38
Cognitive network reorganization following surgical control of seizures in Lennox-Gastaut syndrome. ( 28295228 )
2017
39
Ophthalmologic Features of Lennox-Gastaut Syndrome. ( 28471101 )
2017
40
Thalamocortical functional connectivity in Lennox-Gastaut syndrome is abnormally enhanced in executive-control and default-mode networks. ( 29098688 )
2017
41
Expert Opinion on the Management of Lennox-Gastaut Syndrome: Treatment Algorithms and Practical Considerations. ( 29085326 )
2017
42
Lennox-Gastaut syndrome in adulthood: Long-term clinical follow-up of 38 patients and analysis of their recorded seizures. ( 29126048 )
2017
43
Use of perampanel in children and adolescents with Lennox-Gastaut Syndrome. ( 28715780 )
2017
44
Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions. ( 28461749 )
2017
45
Electroencephalography Network Effects of Corpus Callosotomy in Patients with Lennox-Gastaut Syndrome. ( 28928710 )
2017
46
Predictive role of brain connectivity for resective surgery in Lennox-Gastaut syndrome. ( 27417063 )
2016
47
Challenges in identifying Lennox-Gastaut syndrome in adults: A case series illustrating its changing nature. ( 26977407 )
2016
48
Nocturnal interictal epileptic discharges in adult Lennox-Gastaut syndrome: the effect of sleep stage and time of night. ( 26842220 )
2016
49
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
50
Lacosamide in Lennox-Gastaut Syndrome? Caution Is Still Needed. ( 27827811 )
2016
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 MAPK10 MAPK10, EX10-14DEL deletion Uncertain significance
2 SCN1A NM_006920.4(SCN1A): c.4874G> A (p.Arg1625Gln) single nucleotide variant Likely pathogenic rs121917995 GRCh37 Chromosome 2, 166848878: 166848878
3 SCN1A NM_006920.4(SCN1A): c.4874G> A (p.Arg1625Gln) single nucleotide variant Likely pathogenic rs121917995 GRCh38 Chromosome 2, 165992368: 165992368
4 POLG NM_002693.2(POLG): c.1402A> G (p.Asn468Asp) single nucleotide variant Uncertain significance rs145843073 GRCh37 Chromosome 15, 89870429: 89870429
5 POLG NM_002693.2(POLG): c.1402A> G (p.Asn468Asp) single nucleotide variant Uncertain significance rs145843073 GRCh38 Chromosome 15, 89327198: 89327198
6 ZEB2 NM_014795.3(ZEB2): c.3267A> C (p.Glu1089Asp) single nucleotide variant Uncertain significance GRCh38 Chromosome 2, 144389829: 144389829
7 ZEB2 NM_014795.3(ZEB2): c.3267A> C (p.Glu1089Asp) single nucleotide variant Uncertain significance GRCh37 Chromosome 2, 145147396: 145147396
8 GATM NM_001482.2(GATM): c.367G> A (p.Ala123Thr) single nucleotide variant Uncertain significance GRCh37 Chromosome 15, 45661641: 45661641
9 GATM NM_001482.2(GATM): c.367G> A (p.Ala123Thr) single nucleotide variant Uncertain significance GRCh38 Chromosome 15, 45369443: 45369443
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
12.31 DCX POMC SCN1A STXBP1
2
GABAergic synapse 37
Show member pathways
 12.23 GABRA1 GABRB2 GABRB3 MAPK10 MT-ND1
3
Apoptosis Pathway 72
Show member pathways
 11.98 AKT3 GABRA1 GABRB2 GABRB3 MAPK10
4
L1CAM interactions 66
Show member pathways
 11.73 DCX DNM1 SCN1A
5
Insulin resistance 37
Show member pathways
 11.62 AKT3 MAPK10 SLC2A1
6
Ligand-gated ion channel transport 66
Show member pathways
 11.17 GABRA1 GABRB2 GABRB3
7
Nicotine addiction 37
11.03 GABRA1 GABRB2 GABRB3
8
Adipocytokine signaling pathway 37
10.76 AKT3 MAPK10 POMC SLC2A1
9
Benzodiazepine Pathway, Pharmacodynamics 61
Show member pathways
 10.69 GABRA1 GABRB2
10
Synaptic Neurotransmission Pathways: GABAergic Inhibition 65
10.36 GABRA1 GABRB2 GABRB3
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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 postsynaptic membrane GO:0045211 9.62 DNM1 GABRA1 GABRB2 GABRB3
2 GABA-ergic synapse GO:0098982 9.43 GABRA1 GABRB2 GABRB3
3 chloride channel complex GO:0034707 9.33 GABRA1 GABRB2 GABRB3
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 11)
# Name GO ID Score Top Affiliating Genes
1 ion transmembrane transport GO:0034220 9.78 GABRA1 GABRB2 GABRB3 SCN1A
2 chloride transmembrane transport GO:1902476 9.58 GABRA1 GABRB2 GABRB3
3 chloride transport GO:0006821 9.54 GABRA1 GABRB2 GABRB3
4 innervation GO:0060384 9.49 GABRB2 GABRB3
5 layer formation in cerebral cortex GO:0021819 9.46 ADGRG1 DCX
6 regulation of membrane potential GO:0042391 9.46 GABRA1 GABRB2 GABRB3 SCN1A
7 synaptic transmission, GABAergic GO:0051932 9.43 GABRA1 GABRB2
8 nervous system process GO:0050877 9.43 GABRA1 GABRB2 GABRB3
9 inner ear receptor cell development GO:0060119 9.32 GABRB2 GABRB3
10 gamma-aminobutyric acid signaling pathway GO:0007214 9.13 GABRA1 GABRB2 GABRB3
11 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 ion channel activity GO:0005216 9.56 GABRA1 GABRB2 GABRB3 SCN1A
2 chloride channel activity GO:0005254 9.54 GABRA1 GABRB2 GABRB3
3 extracellular ligand-gated ion channel activity GO:0005230 9.5 GABRA1 GABRB2 GABRB3
4 GABA-gated chloride ion channel activity GO:0022851 9.43 GABRA1 GABRB3
5 inhibitory extracellular ligand-gated ion channel activity GO:0005237 9.4 GABRA1 GABRB2
6 transmitter-gated ion channel activity involved in regulation of postsynaptic membrane potential GO:1904315 9.33 GABRA1 GABRB2 GABRB3
7 GABA receptor activity GO:0016917 8.96 GABRA1 GABRB2
8 GABA-A receptor activity GO:0004890 8.8 GABRA1 GABRB2 GABRB3
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Sources for Lennox-Gastaut Syndrome

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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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