gamma-aminobutyric acid has been researched along with Tuberous Sclerosis in 12 studies
gamma-Aminobutyric Acid: The most common inhibitory neurotransmitter in the central nervous system.
gamma-aminobutyric acid : A gamma-amino acid that is butanoic acid with the amino substituent located at C-4.
Tuberous Sclerosis: Autosomal dominant neurocutaneous syndrome classically characterized by MENTAL RETARDATION; EPILEPSY; and skin lesions (e.g., adenoma sebaceum and hypomelanotic macules). There is, however, considerable heterogeneity in the neurologic manifestations. It is also associated with cortical tuber and HAMARTOMAS formation throughout the body, especially the heart, kidneys, and eyes. Mutations in two loci TSC1 and TSC2 that encode hamartin and tuberin, respectively, are associated with the disease.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Vigabatrin has been shown to be efficient in infants with infantile spasms and tuberous sclerosis, in open studies." | 9.08 | Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosis. ( Chiron, C; Dulac, O; Dumas, C; Jambaqué, I; Mumford, J, 1997) |
| "The purpose of this report is to review the efficacy and safety of vigabatrin in the treatment of infantile spasms in infants suffering from tuberous sclerosis complex." | 8.80 | Vigabatrin in the treatment of infantile spasms in tuberous sclerosis: literature review. ( Hancock, E; Osborne, JP, 1999) |
| " While seizures are usually intractable to medication in tuberous sclerosis complex (TSC), a common genetic cause of epilepsy, vigabatrin appears to have unique efficacy for epilepsy in TSC." | 7.79 | Vigabatrin inhibits seizures and mTOR pathway activation in a mouse model of tuberous sclerosis complex. ( McDaniel, SS; Rensing, NR; Wong, M; Zhang, B, 2013) |
| "The purpose of this study was to estimate the gamma-aminobutyric acid (GABA) and glutamate plus glutamine (Glx) concentrations in the cortical tubers of patients with tuberous sclerosis complex (TSC) using the MEGA-editing J-difference method and a stimulated echo-acquisition mode with a short echo time, and to determine which abnormality was more dominant between GABA and Glx in patients with TSC with epilepsy." | 7.75 | High gamma-aminobutyric acid level in cortical tubers in epileptic infants with tuberous sclerosis complex measured with the MEGA-editing J-difference method and a three-Tesla clinical MRI Instrument. ( Harada, M; Kubo, H; Matsuda, T; Mori, K; Nishitani, H; Nose, A; Taki, MM, 2009) |
| "We present the results of treatment with vigabatrin in the polytherapy of resistant infantile epilepsy." | 7.69 | [Treatment of refractory infantile epilepsy with vigabatrin in a series of 55 patients]. ( Hernández-Laín, A; López-Valdés, E; Mateos, F; Porta, J; Simón, R, 1996) |
| "Vigabatrin has been shown to be efficient in infants with infantile spasms and tuberous sclerosis, in open studies." | 6.68 | Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosis. ( Chiron, C; Dulac, O; Dumas, C; Jambaqué, I; Mumford, J, 1997) |
| "Of the 313 patients without tuberous sclerosis complex, 170 (54%) had complete cessation of their infantile spasms; of the 77 patients with tuberous sclerosis complex, 73 (95%) had complete cessation of their seizures." | 6.40 | Vigabatrin in the treatment of infantile spasms in tuberous sclerosis: literature review. ( Hancock, E; Osborne, JP, 1999) |
| "While seizures are usually intractable to medication in tuberous sclerosis complex (TSC), a common genetic cause of epilepsy, vigabatrin appears to have unique efficacy for epilepsy in TSC." | 5.39 | Vigabatrin inhibits seizures and mTOR pathway activation in a mouse model of tuberous sclerosis complex. ( McDaniel, SS; Rensing, NR; Wong, M; Zhang, B, 2013) |
| "Although antiseizure medication is the first-line treatment for epilepsy, currently available medications are ineffective in a significant percentage of patients and have not clearly been demonstrated to have disease-specific effects for epilepsy." | 5.39 | Vigabatrin inhibits seizures and mTOR pathway activation in a mouse model of tuberous sclerosis complex. ( McDaniel, SS; Rensing, NR; Wong, M; Zhang, B, 2013) |
| "Vigabatrin has been shown to be efficient in infants with infantile spasms and tuberous sclerosis, in open studies." | 5.08 | Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosis. ( Chiron, C; Dulac, O; Dumas, C; Jambaqué, I; Mumford, J, 1997) |
| "The purpose of this report is to review the efficacy and safety of vigabatrin in the treatment of infantile spasms in infants suffering from tuberous sclerosis complex." | 4.80 | Vigabatrin in the treatment of infantile spasms in tuberous sclerosis: literature review. ( Hancock, E; Osborne, JP, 1999) |
| " While seizures are usually intractable to medication in tuberous sclerosis complex (TSC), a common genetic cause of epilepsy, vigabatrin appears to have unique efficacy for epilepsy in TSC." | 3.79 | Vigabatrin inhibits seizures and mTOR pathway activation in a mouse model of tuberous sclerosis complex. ( McDaniel, SS; Rensing, NR; Wong, M; Zhang, B, 2013) |
| "The purpose of this study was to estimate the gamma-aminobutyric acid (GABA) and glutamate plus glutamine (Glx) concentrations in the cortical tubers of patients with tuberous sclerosis complex (TSC) using the MEGA-editing J-difference method and a stimulated echo-acquisition mode with a short echo time, and to determine which abnormality was more dominant between GABA and Glx in patients with TSC with epilepsy." | 3.75 | High gamma-aminobutyric acid level in cortical tubers in epileptic infants with tuberous sclerosis complex measured with the MEGA-editing J-difference method and a three-Tesla clinical MRI Instrument. ( Harada, M; Kubo, H; Matsuda, T; Mori, K; Nishitani, H; Nose, A; Taki, MM, 2009) |
| "We present the results of treatment with vigabatrin in the polytherapy of resistant infantile epilepsy." | 3.69 | [Treatment of refractory infantile epilepsy with vigabatrin in a series of 55 patients]. ( Hernández-Laín, A; López-Valdés, E; Mateos, F; Porta, J; Simón, R, 1996) |
| "To study the pharmacokinetics of vigabatrin in a patient affected with tuberous sclerosis who developed major agitation and aggression, while receiving vigabatrin orally (1." | 3.69 | Pharmacokinetics of the S(+) and R(-) enantiomers of vigabatrin during chronic dosing in a patient with renal failure. ( Chiron, C; Guillonneau, M; Jacqz-Aigrain, E; Loirat, C; Macher, MA; Montes, C; Rey, E, 1997) |
| "Vigabatrin has been shown to be efficient in infants with infantile spasms and tuberous sclerosis, in open studies." | 2.68 | Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosis. ( Chiron, C; Dulac, O; Dumas, C; Jambaqué, I; Mumford, J, 1997) |
| "Of the 313 patients without tuberous sclerosis complex, 170 (54%) had complete cessation of their infantile spasms; of the 77 patients with tuberous sclerosis complex, 73 (95%) had complete cessation of their seizures." | 2.40 | Vigabatrin in the treatment of infantile spasms in tuberous sclerosis: literature review. ( Hancock, E; Osborne, JP, 1999) |
| "Cortical tubers in patients with tuberous sclerosis complex (TSC) are highly associated with intractable epilepsy." | 1.43 | Expression and cellular distribution of transient receptor potential vanilloid 4 in cortical tubers of the tuberous sclerosis complex. ( Chen, B; Chen, X; Li, S; Liang, C; Liu, S; Sun, F; Wang, L; Wei, Y; Yang, H; Yang, M; Yue, J, 2016) |
| "While seizures are usually intractable to medication in tuberous sclerosis complex (TSC), a common genetic cause of epilepsy, vigabatrin appears to have unique efficacy for epilepsy in TSC." | 1.39 | Vigabatrin inhibits seizures and mTOR pathway activation in a mouse model of tuberous sclerosis complex. ( McDaniel, SS; Rensing, NR; Wong, M; Zhang, B, 2013) |
| "Although antiseizure medication is the first-line treatment for epilepsy, currently available medications are ineffective in a significant percentage of patients and have not clearly been demonstrated to have disease-specific effects for epilepsy." | 1.39 | Vigabatrin inhibits seizures and mTOR pathway activation in a mouse model of tuberous sclerosis complex. ( McDaniel, SS; Rensing, NR; Wong, M; Zhang, B, 2013) |
| "Vigabatrin is an irreversible inhibitor of GABA-transaminase, effective in the treatment of drug-resistant epilepsy and reported to be eliminated unchanged by renal excretion." | 1.30 | Pharmacokinetics of the S(+) and R(-) enantiomers of vigabatrin during chronic dosing in a patient with renal failure. ( Chiron, C; Guillonneau, M; Jacqz-Aigrain, E; Loirat, C; Macher, MA; Montes, C; Rey, E, 1997) |
| " A pharmacokinetic study of the S(+) and R(-) enantiomers of vigabatrin was performed before and during dialysis." | 1.30 | Pharmacokinetics of the S(+) and R(-) enantiomers of vigabatrin during chronic dosing in a patient with renal failure. ( Chiron, C; Guillonneau, M; Jacqz-Aigrain, E; Loirat, C; Macher, MA; Montes, C; Rey, E, 1997) |
| " Although vigabatrin is known to have stereoselective kinetics, the difference in plasma dry concentrations and pharmacokinetics of the S(+) and R(-) enantiomers that we observed during long term administration at high doses in a patient with impaired renal function, has not been reported before." | 1.30 | Pharmacokinetics of the S(+) and R(-) enantiomers of vigabatrin during chronic dosing in a patient with renal failure. ( Chiron, C; Guillonneau, M; Jacqz-Aigrain, E; Loirat, C; Macher, MA; Montes, C; Rey, E, 1997) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 7 (58.33) | 18.2507 |
| 2000's | 2 (16.67) | 29.6817 |
| 2010's | 3 (25.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Zhang, B | 1 |
| McDaniel, SS | 1 |
| Rensing, NR | 1 |
| Wong, M | 1 |
| Chen, X | 1 |
| Yang, M | 1 |
| Sun, F | 1 |
| Liang, C | 1 |
| Wei, Y | 1 |
| Wang, L | 1 |
| Yue, J | 1 |
| Chen, B | 1 |
| Li, S | 1 |
| Liu, S | 1 |
| Yang, H | 1 |
| Taki, MM | 1 |
| Harada, M | 2 |
| Mori, K | 2 |
| Kubo, H | 1 |
| Nose, A | 1 |
| Matsuda, T | 1 |
| Nishitani, H | 1 |
| Mori, T | 1 |
| Toda, Y | 1 |
| Fujii, E | 1 |
| Miyazaki, M | 1 |
| Kagami, S | 1 |
| Valencia, I | 1 |
| Legido, A | 1 |
| Yelin, K | 1 |
| Khurana, D | 1 |
| Kothare, SV | 1 |
| Katsetos, CD | 1 |
| Curatolo, P | 1 |
| Aicardi, J | 1 |
| Mumford, JP | 1 |
| Dumas, C | 2 |
| Wood, S | 1 |
| López-Valdés, E | 1 |
| Hernández-Laín, A | 1 |
| Simón, R | 1 |
| Porta, J | 1 |
| Mateos, F | 1 |
| Chiron, C | 2 |
| Jambaqué, I | 1 |
| Mumford, J | 1 |
| Dulac, O | 1 |
| Jacqz-Aigrain, E | 1 |
| Guillonneau, M | 1 |
| Rey, E | 1 |
| Macher, MA | 1 |
| Montes, C | 1 |
| Loirat, C | 1 |
| Hancock, E | 1 |
| Osborne, JP | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Prednisolone vs. Vigabatrin in the First-line Treatment of Infantile Spasms[NCT02299115] | Phase 3 | 0 participants (Actual) | Interventional | 2017-09-05 | Withdrawn (stopped due to Most centres are now using oral steroids as 1st line treatment so question of efficacy is no longer of high interest.) | ||
| Efficacy of Vigabatrin With High Dose Prednisolone Combination Therapy Versus Vigabatrin Alone for Infantile Spasm: a Randomized Trial[NCT04302116] | 250 participants (Anticipated) | Interventional | 2020-05-18 | Recruiting | |||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
1 review available for gamma-aminobutyric acid and Tuberous Sclerosis
| Article | Year |
|---|---|
Vigabatrin in the treatment of infantile spasms in tuberous sclerosis: literature review.
Topics: Anticonvulsants; gamma-Aminobutyric Acid; Humans; Infant; Spasms, Infantile; Tuberous Sclerosis; Vig | 1999 |
2 trials available for gamma-aminobutyric acid and Tuberous Sclerosis
| Article | Year |
|---|---|
Vigabatrin as initial therapy for infantile spasms: a European retrospective survey. Sabril IS Investigator and Peer Review Groups.
Topics: Age of Onset; Anticonvulsants; Child, Preschool; Dose-Response Relationship, Drug; Drug Administrati | 1996 |
Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosis.
Topics: Anticonvulsants; Female; gamma-Aminobutyric Acid; Humans; Hydrocortisone; Infant; Infant, Newborn; M | 1997 |
9 other studies available for gamma-aminobutyric acid and Tuberous Sclerosis
| Article | Year |
|---|---|
Vigabatrin inhibits seizures and mTOR pathway activation in a mouse model of tuberous sclerosis complex.
Topics: Animals; Anticonvulsants; Astrocytes; Brain; Cell Proliferation; Cells, Cultured; Disease Models, An | 2013 |
Expression and cellular distribution of transient receptor potential vanilloid 4 in cortical tubers of the tuberous sclerosis complex.
Topics: Cerebral Cortex; Child; Child, Preschool; Cyclic AMP-Dependent Protein Kinases; Female; gamma-Aminob | 2016 |
High gamma-aminobutyric acid level in cortical tubers in epileptic infants with tuberous sclerosis complex measured with the MEGA-editing J-difference method and a three-Tesla clinical MRI Instrument.
Topics: Biomarkers; Cerebral Cortex; Child; Child, Preschool; Epilepsy; Female; gamma-Aminobutyric Acid; Hum | 2009 |
Decreased benzodiazepine receptor and increased GABA level in cortical tubers in tuberous sclerosis complex.
Topics: Aspartic Acid; Brain; Case-Control Studies; Child; Child, Preschool; Female; Flumazenil; gamma-Amino | 2012 |
Anomalous inhibitory circuits in cortical tubers of human tuberous sclerosis complex associated with refractory epilepsy: aberrant expression of parvalbumin and calbindin-D28k in dysplastic cortex.
Topics: Adolescent; Adult; Biomarkers; Calbindin 1; Calbindins; Calcium; Calcium-Binding Proteins; Cerebral | 2006 |
Vigabatrin for refractory partial seizures in children with tuberous sclerosis.
Topics: Adolescent; Anticonvulsants; Child; Child, Preschool; gamma-Aminobutyric Acid; Humans; Infant; Retro | 1994 |
[Treatment of refractory infantile epilepsy with vigabatrin in a series of 55 patients].
Topics: Adolescent; Anticonvulsants; Brain; Child; Child, Preschool; Epilepsy; Female; gamma-Aminobutyric Ac | 1996 |
Pharmacokinetics of the S(+) and R(-) enantiomers of vigabatrin during chronic dosing in a patient with renal failure.
Topics: Adult; Anticonvulsants; Enzyme Inhibitors; gamma-Aminobutyric Acid; Humans; Kidney Failure, Chronic; | 1997 |
Vigabatrin: new indication. An advance in infantile spasms.
Topics: Anticonvulsants; Child; Child, Preschool; Clinical Trials as Topic; Drug Evaluation; France; gamma-A | 1998 |