Page last updated: 2024-10-26

valproic acid and Angelman Syndrome

valproic acid has been researched along with Angelman Syndrome in 8 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.

Angelman Syndrome: A syndrome characterized by multiple abnormalities, MENTAL RETARDATION, and movement disorders. Present usually are skull and other abnormalities, frequent infantile spasms (SPASMS, INFANTILE); easily provoked and prolonged paroxysms of laughter (hence happy); jerky puppetlike movements (hence puppet); continuous tongue protrusion; motor retardation; ATAXIA; MUSCLE HYPOTONIA; and a peculiar facies. It is associated with maternal deletions of chromosome 15q11-13 and other genetic abnormalities. (From Am J Med Genet 1998 Dec 4;80(4):385-90; Hum Mol Genet 1999 Jan;8(1):129-35)

Research Excerpts

ExcerptRelevanceReference
"The onset of epilepsy is most prevalent between 1 and 3 years of age; however, approximately 25% of patients developed epilepsy before one year of age."2.72Epilepsy in Angelman syndrome: A scoping review. ( Samanta, D, 2021)
"Angelman syndrome is a neurogenetic disorder caused by lack of UBE3A gene expression from the maternally inherited chromosome 15 due to various 15q11-q13 abnormalities."2.44Epilepsy in Angelman syndrome. ( Boyd, SG; Cheron, G; Dan, B; Pelc, K, 2008)
"Epilepsy was controlled with valproate at the age of 6 years."2.42A study of EEG and epilepsy profile in Wolf-Hirschhorn syndrome and considerations regarding its correlation with other chromosomal disorders. ( Fiore, LA; Freitas, A; Kim, CA; Valente, KD, 2003)
"After several years without seizures, 24 out of 30 had a relapse, which was quickly stopped in 23 out of 30 patients."1.72[Epilepsy in Angelman syndrome]. ( Bobylova, MY; Glukhova, LY; Kuzmich, GV; Mukhin, KY; Pylayeva, OA, 2022)
"Epilepsy was most common among those with maternal deletions and unknown subtypes, with catastrophic epilepsies present in only these two subtypes."1.35Epilepsy in Angelman syndrome: a questionnaire-based assessment of the natural history and current treatment options. ( Braun, EK; Bruno, P; Conant, KD; Nespeca, MP; Said, RR; Thibert, RL; Thiele, EA, 2009)
"A 25-year-old girl with mild mental retardation had a 6-year history of absence seizures, with occasional head drop."1.31Mild generalized epilepsy and developmental disorder associated with large inv dup(15). ( Canevini, MP; Canger, R; Cavani, S; Chifari, R; Elia, M; Guerrini, R; Pierluigi, M; Sgrò, V, 2002)

Research

Studies (8)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's4 (50.00)29.6817
2010's1 (12.50)24.3611
2020's3 (37.50)2.80

Authors

AuthorsStudies
Bobylova, MY1
Mukhin, KY1
Kuzmich, GV1
Glukhova, LY1
Pylayeva, OA1
Ascoli, M1
Elia, M2
Gasparini, S1
Bonanni, P1
Mastroianni, G1
Cianci, V1
Neri, S1
Pascarella, A1
Santangelo, D1
Aguglia, U1
Ferlazzo, E1
Samanta, D1
Jamal, I1
Kumar, V1
Vatsa, N1
Shekhar, S1
Singh, BK1
Sharma, A1
Jana, NR1
Thibert, RL1
Conant, KD1
Braun, EK1
Bruno, P1
Said, RR1
Nespeca, MP1
Thiele, EA1
Chifari, R1
Guerrini, R1
Pierluigi, M1
Cavani, S1
Sgrò, V1
Canger, R1
Canevini, MP1
Valente, KD1
Freitas, A1
Fiore, LA1
Kim, CA1
Pelc, K1
Boyd, SG1
Cheron, G1
Dan, B1

Reviews

4 reviews available for valproic acid and Angelman Syndrome

ArticleYear
Therapeutic approach to neurological manifestations of Angelman syndrome.
    Expert review of clinical pharmacology, 2022, Volume: 15, Issue:7

    Topics: Angelman Syndrome; Anticonvulsants; Epilepsy; Epilepsy, Generalized; Humans; Valproic Acid

2022
Epilepsy in Angelman syndrome: A scoping review.
    Brain & development, 2021, Volume: 43, Issue:1

    Topics: Angelman Syndrome; Anticonvulsants; Child; Child, Preschool; Electroencephalography; Epilepsy; Femal

2021
A study of EEG and epilepsy profile in Wolf-Hirschhorn syndrome and considerations regarding its correlation with other chromosomal disorders.
    Brain & development, 2003, Volume: 25, Issue:4

    Topics: Angelman Syndrome; Anticonvulsants; Child; Chromosome Deletion; Chromosome Disorders; Chromosomes, H

2003
Epilepsy in Angelman syndrome.
    Seizure, 2008, Volume: 17, Issue:3

    Topics: Angelman Syndrome; Electroencephalography; Epilepsy; Humans; Valproic Acid

2008

Other Studies

4 other studies available for valproic acid and Angelman Syndrome

ArticleYear
[Epilepsy in Angelman syndrome].
    Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova, 2022, Volume: 122, Issue:7

    Topics: Angelman Syndrome; Anticonvulsants; Child; Electroencephalography; Epilepsy; Ethosuximide; Female; H

2022
Rescue of altered HDAC activity recovers behavioural abnormalities in a mouse model of Angelman syndrome.
    Neurobiology of disease, 2017, Volume: 105

    Topics: Angelman Syndrome; Animals; Anxiety; Brain; Cell Line, Transformed; Cognition Disorders; Disease Mod

2017
Epilepsy in Angelman syndrome: a questionnaire-based assessment of the natural history and current treatment options.
    Epilepsia, 2009, Volume: 50, Issue:11

    Topics: Adolescent; Adult; Angelman Syndrome; Anticonvulsants; Child; Child, Preschool; Clonazepam; Comorbid

2009
Mild generalized epilepsy and developmental disorder associated with large inv dup(15).
    Epilepsia, 2002, Volume: 43, Issue:9

    Topics: Adult; Age of Onset; Angelman Syndrome; Anticonvulsants; Chromosome Aberrations; Chromosome Inversio

2002