Page last updated: 2024-10-26

valproic acid and Huntington Disease

valproic acid has been researched along with Huntington Disease in 34 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.

Huntington Disease: A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)

Research Excerpts

ExcerptRelevanceReference
"Chorea in Huntington's Disease (HD) is usually treated with antidopaminergic neuroleptics like haloperidol, olanzapine and tiaprid or dopamine depleting drugs like tetrabenazine."2.72Dose-dependent improvement of myoclonic hyperkinesia due to Valproic acid in eight Huntington's Disease patients: a case series. ( Andrich, JE; Kraus, PH; Lauter, T; Przuntek, H; Saft, C, 2006)
"In a single-case study of Huntington's Chorea the effect of sodium valproate on motor behaviour was first compared with that of L-Dopa and than investigated further alone."2.64Sodium valproate in Huntington's chorea. ( Leijnse-Ybema, HJ; Tan, BK; Zee, MF, 1976)
"Using Alzheimer's disease and Huntington's disease as examples in the following article, some of latest data linking both the histone code and the various proteins that regulate this code to the pathogenesis of neurological disease are discussed."2.47Epigenetic treatment of neurological disease. ( Gray, SG, 2011)
"Among psychiatric disorders the acute symptoms of schizophrenia are exacerbated by enhanced GABA-ergic function."2.36Pharmacology of GABA. ( Meldrum, B, 1982)
"Treatment with rosiglitazone (5, 10 mg/kg) and VPA (100, 200 mg/kg) for 21 days significantly attenuated these behavioral, biochemical, and cellular alterations as compared to control (QA 200 nmol) group."1.40Rosiglitazone synergizes the neuroprotective effects of valproic acid against quinolinic acid-induced neurotoxicity in rats: targeting PPARγ and HDAC pathways. ( Chaudhary, T; Kumar, A; Mishra, J, 2014)
" Furthermore, long-term administration (over a year) of high doses of the agent (up to 2,400 mg per day; 92 mg per kilogram per day) did not seem to alter the slow progression of their disease."1.26Long-term treatment of juvenile Huntington's chorea with dipropylacetic acid. ( Bachman, DS; Butler, IJ; McKhann, GM, 1977)
"Treatment with haloperidol in all patients and with dipropylacetic acid in three patients did not appear to modify the CSF, HVA, and 5HIAA concentrations, the plasma DBH activity, or the DA uptake."1.26Biochemical aspects of Huntington's chorea. ( Algeri, S; Branciforti, A; Calderini, G; Caraceni, T; Consolazione, A; Dall'olio, A; Girotti, F; Morselli, PL; Riva, E; Spreafico, R, 1977)

Research

Studies (34)

TimeframeStudies, this research(%)All Research%
pre-199017 (50.00)18.7374
1990's1 (2.94)18.2507
2000's7 (20.59)29.6817
2010's9 (26.47)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Williams, A1
Sarkar, S1
Cuddon, P1
Ttofi, EK1
Saiki, S1
Siddiqi, FH1
Jahreiss, L1
Fleming, A1
Pask, D1
Goldsmith, P1
O'Kane, CJ1
Floto, RA1
Rubinsztein, DC1
Whiting, WL1
Velasco, R1
Stewart, JT1
Mishra, J1
Chaudhary, T1
Kumar, A1
Lin, F1
Qin, ZH1
Linares, GR1
Chiu, CT2
Scheuing, L1
Leng, Y1
Liao, HM1
Maric, D1
Chuang, DM2
Salazar, Z1
Tschopp, L1
Calandra, C1
Micheli, F1
Cowell, RM1
Talati, P1
Blake, KR1
Meador-Woodruff, JH1
Russell, JW1
Zádori, D1
Geisz, A1
Vámos, E1
Vécsei, L1
Klivényi, P1
El-Akabawy, G1
Medina, LM1
Jeffries, A1
Price, J1
Modo, M1
Liu, G1
Leeds, P1
Tallaksen-Greene, SJ1
Albin, RL1
Gray, SG1
Cuturic, M1
Abramson, RK1
Moran, RR1
Hardin, JW1
Frank, EM1
Sellers, AA1
Saft, C1
Lauter, T1
Kraus, PH1
Przuntek, H1
Andrich, JE1
Tremolizzo, L1
Rodriguez-Menendez, V1
DiFrancesco, JC1
Sala, G1
Galbussera, A1
Appollonio, I1
Ferrarese, C1
Previdi, P1
Borgonovi, R1
Meldrum, B1
Puca, FM1
Genco, S1
Minervini, MG1
Specchio, LM1
Napoletano, V1
Brancasi, B1
Reggio, F1
Marsden, CD1
Sheehy, MP1
Grove, VE1
Quintanilla, J1
DeVaney, GT1
Shoulson, I1
Kartzinel, R1
Chase, TN1
Lenman, JA1
Ferguson, IT1
Fleming, AM1
Herzberg, M1
Robb, JE1
Turnbull, MJ1
Tan, BK1
Leijnse-Ybema, HJ1
Zee, MF1
Bachman, DS1
Butler, IJ1
McKhann, GM1
Pearce, I1
Heathfield, KW1
Pearce, MJ1
Caraceni, T2
Calderini, G1
Consolazione, A1
Riva, E1
Algeri, S1
Girotti, F2
Spreafico, R1
Branciforti, A1
Dall'olio, A1
Morselli, PL1
Symington, GR1
Leonard, DP1
Shannon, PJ1
Vajda, FJ1
Perry, TL2
Hansen, S1
Giovannini, P1
Avanzini, G1
Schwarcz, R1
Bennett, JP1
Coyle, JT1
Ikonen, E1
Salo, A1
Somer, M1
Somer, H1
Pääkkönen, L1
Peltonen, L1
Gram, L1
Bentsen, KD1

Reviews

4 reviews available for valproic acid and Huntington Disease

ArticleYear
Degradation of misfolded proteins by autophagy: is it a strategy for Huntington's disease treatment?
    Journal of Huntington's disease, 2013, Volume: 2, Issue:2

    Topics: Autophagy; Carbamazepine; Humans; Huntingtin Protein; Huntington Disease; Lithium; Mutant Proteins;

2013
Epigenetic treatment of neurological disease.
    Epigenomics, 2011, Volume: 3, Issue:4

    Topics: Alzheimer Disease; Butylamines; Cell Differentiation; Endoplasmic Reticulum Stress; Epigenesis, Gene

2011
Pharmacology of GABA.
    Clinical neuropharmacology, 1982, Volume: 5, Issue:3

    Topics: 4-Aminobutyrate Transaminase; Anxiety Disorders; Autonomic Nervous System; Barbiturates; Benzodiazep

1982
Valproate: an updated review.
    Acta neurologica Scandinavica, 1985, Volume: 72, Issue:2

    Topics: Abnormalities, Drug-Induced; Brain Diseases; Chemical and Drug Induced Liver Injury; Coma; Dyskinesi

1985

Trials

5 trials available for valproic acid and Huntington Disease

ArticleYear
Dose-dependent improvement of myoclonic hyperkinesia due to Valproic acid in eight Huntington's Disease patients: a case series.
    BMC neurology, 2006, Feb-28, Volume: 6

    Topics: Adult; Anticonvulsants; Dose-Response Relationship, Drug; Female; Humans; Huntington Disease; Hyperk

2006
GABA and movement disorders.
    Advances in biochemical psychopharmacology, 1981, Volume: 30

    Topics: Anticonvulsants; Baclofen; Clinical Trials as Topic; Double-Blind Method; Dyskinesia, Drug-Induced;

1981
Huntington's disease: treatment with dipropylacetic acid and gamma-aminobutyric acid.
    Neurology, 1976, Volume: 26, Issue:1

    Topics: Adult; Aminobutyrates; Clinical Trials as Topic; Dopamine; Drug Evaluation; Drug Therapy, Combinatio

1976
Sodium valproate in Huntington's chorea.
    Clinical neurology and neurosurgery, 1976, Volume: 79, Issue:1

    Topics: Clinical Trials as Topic; Drug Evaluation; Drug Tolerance; Female; Humans; Huntington Disease; Levod

1976
Sodium valproate in Huntington's disease.
    The American journal of psychiatry, 1978, Volume: 135, Issue:3

    Topics: Adult; Arousal; Clinical Trials as Topic; Drug Evaluation; Female; Humans; Huntington Disease; Middl

1978

Other Studies

25 other studies available for valproic acid and Huntington Disease

ArticleYear
Novel targets for Huntington's disease in an mTOR-independent autophagy pathway.
    Nature chemical biology, 2008, Volume: 4, Issue:5

    Topics: Animals; Autophagy; Calcium Channels, L-Type; Clonidine; Cyclic AMP; Humans; Huntington Disease; Imi

2008
Treatment of Behavioral Disinhibition in Huntington's Disease With Valproic Acid.
    Journal of clinical psychopharmacology, 2018, Volume: 38, Issue:1

    Topics: Aggression; Anticonvulsants; Female; Humans; Huntington Disease; Middle Aged; Problem Behavior; Valp

2018
Rosiglitazone synergizes the neuroprotective effects of valproic acid against quinolinic acid-induced neurotoxicity in rats: targeting PPARγ and HDAC pathways.
    Neurotoxicity research, 2014, Volume: 26, Issue:2

    Topics: Animals; Body Weight; Brain; Disease Models, Animal; Drug Synergism; Histone Deacetylase Inhibitors;

2014
Preconditioning mesenchymal stem cells with the mood stabilizers lithium and valproic acid enhances therapeutic efficacy in a mouse model of Huntington's disease.
    Experimental neurology, 2016, Volume: 281

    Topics: Animals; Antimanic Agents; Cytokines; Disease Models, Animal; Dopamine and cAMP-Regulated Phosphopro

2016
Pisa syndrome and parkinsonism secondary to valproic acid in Huntington's disease.
    Movement disorders : official journal of the Movement Disorder Society, 2008, Dec-15, Volume: 23, Issue:16

    Topics: Aged; Anticonvulsants; Humans; Huntington Disease; Male; Parkinson Disease, Secondary; Sensation Dis

2008
Identification of novel targets for PGC-1alpha and histone deacetylase inhibitors in neuroblastoma cells.
    Biochemical and biophysical research communications, 2009, Feb-06, Volume: 379, Issue:2

    Topics: Apoptosis; Biological Transport; Caspase 3; Cell Line, Tumor; Enzyme Inhibitors; Fatty Acids; Gene E

2009
Valproate ameliorates the survival and the motor performance in a transgenic mouse model of Huntington's disease.
    Pharmacology, biochemistry, and behavior, 2009, Volume: 94, Issue:1

    Topics: 3,4-Dihydroxyphenylacetic Acid; Animals; Behavior, Animal; Corpus Striatum; Disease Models, Animal;

2009
Purmorphamine increases DARPP-32 differentiation in human striatal neural stem cells through the Hedgehog pathway.
    Stem cells and development, 2011, Volume: 20, Issue:11

    Topics: Antigens, Differentiation; Brain; Brain-Derived Neurotrophic Factor; Bucladesine; Calbindin 2; Calbi

2011
Combined treatment with the mood stabilizers lithium and valproate produces multiple beneficial effects in transgenic mouse models of Huntington's disease.
    Neuropsychopharmacology : official publication of the American College of Neuropsychopharmacology, 2011, Volume: 36, Issue:12

    Topics: Affect; Animals; Disease Models, Animal; Dose-Response Relationship, Drug; Drug Therapy, Combination

2011
Treating mouse models of Huntington disease.
    Neuropsychopharmacology : official publication of the American College of Neuropsychopharmacology, 2011, Volume: 36, Issue:12

    Topics: Affect; Animals; Female; Huntington Disease; Lithium; Male; Valproic Acid

2011
Serum carnitine levels and levocarnitine supplementation in institutionalized Huntington's disease patients.
    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2013, Volume: 34, Issue:1

    Topics: Adult; Anticonvulsants; Carnitine; Databases, Factual; Diet; Dietary Supplements; Female; Hospitaliz

2013
Huntington's disease and HDACi: would sulpiride and valproate be of therapeutic value?
    Medical hypotheses, 2007, Volume: 69, Issue:4

    Topics: Anticonvulsants; Antipsychotic Agents; Histone Deacetylases; Humans; Huntington Disease; Motor Activ

2007
Myoclonus and Huntington's chorea: description of a case.
    Italian journal of neurological sciences, 1980, Volume: 1, Issue:3

    Topics: Adult; Clonazepam; Electroencephalography; Female; Humans; Huntington Disease; Myoclonus; Pedigree;

1980
[Sleep in chronic chorea patients after therapy with sodium valproate].
    Bollettino della Societa italiana di biologia sperimentale, 1984, May-30, Volume: 60, Issue:5

    Topics: Adult; Chorea; Chronic Disease; Female; gamma-Aminobutyric Acid; Humans; Huntington Disease; Male; M

1984
Improvement of Huntington's disease with olanzapine and valproate.
    The New England journal of medicine, 2000, Sep-28, Volume: 343, Issue:13

    Topics: Adult; Antimanic Agents; Antipsychotic Agents; Benzodiazepines; Drug Therapy, Combination; Female; H

2000
Sodium valproate in chorea.
    British medical journal, 1976, Nov-06, Volume: 2, Issue:6044

    Topics: Aged; Brain; Female; Gait; gamma-Aminobutyric Acid; Humans; Huntington Disease; Male; Movement; Vale

1976
Sodium valproate in chorea.
    British medical journal, 1976, Dec-25, Volume: 2, Issue:6051

    Topics: Adult; Aged; Brain Chemistry; gamma-Aminobutyric Acid; Humans; Huntington Disease; Middle Aged; Vale

1976
Long-term treatment of juvenile Huntington's chorea with dipropylacetic acid.
    Neurology, 1977, Volume: 27, Issue:2

    Topics: Child; Diseases in Twins; Female; Half-Life; Humans; Huntington Disease; Valerates; Valproic Acid

1977
Valproate sodium in Huntington chorea.
    Archives of neurology, 1977, Volume: 34, Issue:5

    Topics: Adolescent; Adult; Aged; Child; Female; Humans; Huntington Disease; Male; Middle Aged; Valerates; Va

1977
Biochemical aspects of Huntington's chorea.
    Journal of neurology, neurosurgery, and psychiatry, 1977, Volume: 40, Issue:6

    Topics: Adult; Age Factors; Blood Platelets; Brain Damage, Chronic; Dopamine; Dopamine beta-Hydroxylase; Fem

1977
Isoniazid and Huntington's chorea.
    The New England journal of medicine, 1978, May-11, Volume: 298, Issue:19

    Topics: Animals; Brain; gamma-Aminobutyric Acid; Humans; Huntington Disease; Isoniazid; Rats; Valproic Acid

1978
Biochemical effects in man and rat of three drugs which can increase brain GABA content.
    Journal of neurochemistry, 1978, Volume: 30, Issue:4

    Topics: Acetates; Adult; Amino Acids; Aminobutyrates; Aminooxyacetic Acid; Animals; Brain; gamma-Aminobutyri

1978
Pharmacology of Huntington's chorea. Personal experience.
    European neurology, 1977, Volume: 16, Issue:1-6

    Topics: Acetates; Adult; Aminobutyrates; Blood Cell Count; Bromocriptine; Female; gamma-Aminobutyric Acid; H

1977
Inhibitors of GABA metabolism: implications for Huntington's disease.
    Annals of neurology, 1977, Volume: 2, Issue:4

    Topics: 4-Aminobutyrate Transaminase; Alkynes; Aminocaproates; Aminocaproic Acid; Animals; Caudate Nucleus;

1977
Terminal deletion of chromosome 4p (4p16.3) shows a breakpoint between loci linked to Huntington disease.
    American journal of medical genetics, 1992, Jul-01, Volume: 43, Issue:4

    Topics: Abnormalities, Multiple; Adolescent; Chromosome Banding; Chromosome Deletion; Chromosomes, Human, Pa

1992