valproic acid has been researched along with Rett Syndrome in 16 studies
Valproic Acid: A fatty acid with anticonvulsant and anti-manic properties that is used in the treatment of EPILEPSY and BIPOLAR DISORDER. The mechanisms of its therapeutic actions are not well understood. It may act by increasing GAMMA-AMINOBUTYRIC ACID levels in the brain or by altering the properties of VOLTAGE-GATED SODIUM CHANNELS.
valproic acid : A branched-chain saturated fatty acid that comprises of a propyl substituent on a pentanoic acid stem.
Rett Syndrome: An inherited neurological developmental disorder that is associated with X-LINKED INHERITANCE and may be lethal in utero to hemizygous males. The affected female is normal until the age of 6-25 months when progressive loss of voluntary control of hand movements and communication skills; ATAXIA; SEIZURES; autistic behavior; intermittent HYPERVENTILATION; and HYPERAMMONEMIA appear. (From Menkes, Textbook of Child Neurology, 5th ed, p199)
| Excerpt | Relevance | Reference |
|---|---|---|
| " We exploited 2 different animal models of autism, the methyl CpG binding protein 2-null (Mecp2y/-) mouse model of Rett syndrome and mice prenatally exposed to valproic acid, and found increased ATM levels." | 4.02 | The DNA repair protein ATM as a target in autism spectrum disorder. ( Antonucci, F; Bifari, F; Cambria, C; Desiato, G; Ferrara, S; Focchi, E; Landsberger, N; Matteoli, M; Menna, E; Murru, L; Passafaro, M; Pizzamiglio, L; Ponzoni, L; Sala, M, 2021) |
| " VPA and CBZ proved to be equally effective in Rett patients who presented seizures within the typical age range (4-5 years), while Lamotrigine (LTG) was effective for patients in whom epilepsy started later." | 3.81 | Antiepileptic drugs in Rett Syndrome. ( Baglietto, MG; Calevo, MG; Canevini, M; Giacomini, T; Giordano, L; Hayek, Y; Pintaudi, M; Renieri, A; Russo, S; Traverso, M; Veneselli, E; Vignoli, A, 2015) |
| "Magnetic resonance (MR) multimodality evoked potentials (MEPs) and clinical findings were correlated in a 47-year-old epileptic man in whom parenteral valproic acid (VPA) therapy induced severe comatose hyperammonemic encephalopathy without biological signs of hepatotoxicity (or hepatocytic dysfunction)." | 3.73 | Comparison of clinical, magnetic resonance and evoked potentials data in a case of valproic-acid-related hyperammonemic coma. ( Duprez, T; Grandin, C; Guérit, JM; Hantson, P; Nassogne, MC, 2005) |
| "Valproic acid (VPA) was the most prescribed single therapy in young patients (<15years), whereas carbamazepine (CBZ) was preferred by clinicians in older patients." | 1.46 | Effectiveness and tolerability of antiepileptic drugs in 104 girls with Rett syndrome. ( Canevini, MP; La Briola, F; Nowbut, MS; Peron, A; Savini, MN; Turner, K; Vignoli, A, 2017) |
| "Valproic acid (VPA) has been used widely to treat mood disorder, epilepsy, and a growing number of other disorders." | 1.40 | VPA alleviates neurological deficits and restores gene expression in a mouse model of Rett syndrome. ( Guo, W; Igarashi, K; Irie, K; Nakashima, K; Otsuka I, M; Tsujimura, K; Zhao, X, 2014) |
| "Rett Syndrome is an X-linked progressive neurological disorder caused by inactivation of one allele of the MECP2 gene." | 1.37 | Clonal Rett Syndrome cell lines to test compounds for activation of wild-type MeCP2 expression. ( Corey, DR; Sakurai, F; Yu, D, 2011) |
| "The epileptic seizures described in this report have not been previously documented in Rett syndrome." | 1.32 | Rett syndrome: a case presenting with atypical seizures. Neurophysiological and clinical aspects. ( Jerez-García, P; Marco-Giner, J; Onsurbe-Ramírez, I; Pardal-Fernández, JM, 2004) |
| "Rett's syndrome is a neurodevelopmental disorder which is caused by a mutation on the x-chromosome; thus, it only affects the female sex." | 1.30 | [Rett's syndrome: pathophysiology and anesthesiological implications]. ( Häuser, FM; Lennartz, H; Lukasewitz, P; Wichert, A, 1999) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 1 (6.25) | 18.2507 |
| 2000's | 8 (50.00) | 29.6817 |
| 2010's | 6 (37.50) | 24.3611 |
| 2020's | 1 (6.25) | 2.80 |
| Authors | Studies |
|---|---|
| Yu, D | 1 |
| Sakurai, F | 1 |
| Corey, DR | 1 |
| Pizzamiglio, L | 1 |
| Focchi, E | 1 |
| Cambria, C | 1 |
| Ponzoni, L | 1 |
| Ferrara, S | 1 |
| Bifari, F | 1 |
| Desiato, G | 1 |
| Landsberger, N | 1 |
| Murru, L | 1 |
| Passafaro, M | 1 |
| Sala, M | 1 |
| Matteoli, M | 1 |
| Menna, E | 1 |
| Antonucci, F | 1 |
| Guo, W | 1 |
| Tsujimura, K | 1 |
| Otsuka I, M | 1 |
| Irie, K | 1 |
| Igarashi, K | 1 |
| Nakashima, K | 1 |
| Zhao, X | 1 |
| Pintaudi, M | 1 |
| Calevo, MG | 1 |
| Vignoli, A | 2 |
| Baglietto, MG | 1 |
| Hayek, Y | 1 |
| Traverso, M | 1 |
| Giacomini, T | 1 |
| Giordano, L | 1 |
| Renieri, A | 1 |
| Russo, S | 1 |
| Canevini, M | 1 |
| Veneselli, E | 1 |
| Savini, MN | 1 |
| Nowbut, MS | 1 |
| Peron, A | 1 |
| Turner, K | 1 |
| La Briola, F | 1 |
| Canevini, MP | 1 |
| Dealberto, MJ | 1 |
| Sarazin, FF | 1 |
| Vecsler, M | 1 |
| Simon, AJ | 1 |
| Amariglio, N | 1 |
| Rechavi, G | 1 |
| Gak, E | 1 |
| Leonard, H | 1 |
| Downs, J | 1 |
| Jian, L | 1 |
| Bebbington, A | 1 |
| Jacoby, P | 1 |
| Nagarajan, L | 1 |
| Ravine, D | 1 |
| Woodhead, H | 1 |
| Pardal-Fernández, JM | 1 |
| Jerez-García, P | 1 |
| Onsurbe-Ramírez, I | 1 |
| Marco-Giner, J | 1 |
| Grubben, B | 1 |
| De Jonghe, P | 1 |
| Cras, P | 1 |
| Demey, HE | 1 |
| Parizel, PM | 1 |
| Hantson, P | 1 |
| Grandin, C | 1 |
| Duprez, T | 1 |
| Nassogne, MC | 1 |
| Guérit, JM | 1 |
| Rath, A | 1 |
| Naryanan, TJ | 1 |
| Chowdhary, GV | 1 |
| Murthy, JM | 1 |
| Alqahtani, S | 1 |
| Federico, P | 1 |
| Myers, RP | 1 |
| Penning, C | 1 |
| van der Wouden, JC | 1 |
| Pupko, HA | 1 |
| Häuser, FM | 1 |
| Lukasewitz, P | 1 |
| Wichert, A | 1 |
| Lennartz, H | 1 |
16 other studies available for valproic acid and Rett Syndrome
| Article | Year |
|---|---|
Clonal Rett Syndrome cell lines to test compounds for activation of wild-type MeCP2 expression.
Topics: Clone Cells; Epigenesis, Genetic; Fibroblasts; Gene Expression Profiling; Humans; Methyl-CpG-Binding | 2011 |
The DNA repair protein ATM as a target in autism spectrum disorder.
Topics: Animals; Ataxia Telangiectasia Mutated Proteins; Autism Spectrum Disorder; Behavior, Animal; Disease | 2021 |
VPA alleviates neurological deficits and restores gene expression in a mouse model of Rett syndrome.
Topics: Animals; Disease Models, Animal; Female; Gene Expression Regulation; Gene Knockout Techniques; Methy | 2014 |
Antiepileptic drugs in Rett Syndrome.
Topics: Adolescent; Adult; Aged; Anticonvulsants; Carbamazepine; Epilepsy; Female; Humans; Lamotrigine; Male | 2015 |
Effectiveness and tolerability of antiepileptic drugs in 104 girls with Rett syndrome.
Topics: Adolescent; Adult; Age Factors; Anticonvulsants; Carbamazepine; Child; Child, Preschool; Drug Therap | 2017 |
Valproate-induced hyperammonemic encephalopathy without cognitive sequelae: a case report in the psychiatric setting.
Topics: Adult; Antimanic Agents; Female; Humans; Mental Disorders; Rett Syndrome; Valproic Acid | 2008 |
MeCP2 deficiency downregulates specific nuclear proteins that could be partially recovered by valproic acid in vitro.
Topics: Animals; Cell Line, Tumor; Cell Nucleus; Disease Models, Animal; Enzyme Inhibitors; Gene Silencing; | 2010 |
Valproate and risk of fracture in Rett syndrome.
Topics: Adolescent; Adult; Anticonvulsants; Australia; Child; Child, Preschool; Drug Administration Schedule | 2010 |
Rett syndrome: a case presenting with atypical seizures. Neurophysiological and clinical aspects.
Topics: Anticonvulsants; Child; Electroencephalography; Female; Humans; Rett Syndrome; Seizures; Valproic Ac | 2004 |
Valproate-induced hyperammonemic encephalopathy: imaging findings on diffusion-weighted MRI.
Topics: Brain; Brain Mapping; Diffusion Magnetic Resonance Imaging; Epilepsy; Humans; Male; Middle Aged; Mul | 2004 |
Comparison of clinical, magnetic resonance and evoked potentials data in a case of valproic-acid-related hyperammonemic coma.
Topics: Atrophy; Coma; Evoked Potentials; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Rett Syndro | 2005 |
Valproate-induced hyperammonemic encephalopathy with normal liver function.
Topics: Adolescent; Adult; Aged; Anticonvulsants; Epilepsy; Female; Humans; Liver; Liver Function Tests; Mal | 2005 |
A case of valproate-induced hyperammonemic encephalopathy: look beyond the liver.
Topics: Adolescent; Anticonvulsants; Diagnosis, Differential; Electroencephalography; Epilepsy, Temporal Lob | 2007 |
Increased plasma leptin levels and valproate use in patients with Rett syndrome.
Topics: Anticonvulsants; Biomarkers; Growth Disorders; Humans; Leptin; Rett Syndrome; Valproic Acid | 2007 |
Possible new clinical sign of hyperammonemia.
Topics: Adolescent; Anticonvulsants; Diagnosis, Differential; Electroencephalography; Epilepsy, Temporal Lob | 2008 |
[Rett's syndrome: pathophysiology and anesthesiological implications].
Topics: Adult; Anesthesia; Anticonvulsants; Blood Coagulation Disorders; Epilepsy; Female; Humans; Kidney Ca | 1999 |