References
- The Huntington’s Disease Collaborative Research GroupA novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromosomesCell19937269719838458085
- NuciforaFCJrSasakiMPetersMFInterference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicityScience200129155122423242811264541
- SubramaniamSSixtKMBarrowRSnyderSHRhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicityScience200932459321327133019498170
- RibeiroFMPaquetMFerreiraLTMetabotropic glutamate receptor-mediated cell signaling pathways are altered in a mouse model of Huntington’s diseaseJ Neurosci201030131632420053912
- AndrichJSaftCOstholtNMüllerTComplex movement behavior and progression of Huntington’s diseaseNeurosci Lett2007416327227417321683
- FrankSMarshallFPlumbSFunctional decline due to chorea in Huntington’s diseaseNeurology200462Suppl 5A204
- VerhagenMLMorrisMJFarmerCHuntington’s disease: a randomized, controlled trial using the NMDA-antagonist amantadineNeurology200259569469912221159
- BatesGHuntingtin aggregation and toxicity in Huntington’s diseaseLancet200336193691642164412747895
- ArrasateMMitraSSchweitzerESSegalMRFinkbeinerSInclusion body formation reduces levels of mutant huntingtin and the risk of neuronal deathNature2004431701080581015483602
- GlassMDragunowMFaullRLThe pattern of neurodegeneration in Huntington’s disease: a comparative study of cannabinoid, dopamine, adenosine and GABA(A) receptor alterations in the human basal ganglia in Huntington’s diseaseNeuroscience200097350551910828533
- AlbinRLReinerAAndersonKDPreferential loss of striato-external pallidal projection neurons in presymptomatic Huntington’s diseaseAnn Neurol19923144254301375014
- ReinerAAlbinRLAndersonKDD’AmatoCJPenneyJBYoungABDifferential loss of striatal projection neurons in Huntington diseaseProc Natl Acad Sci U S A19888515573357372456581
- MitchellIJCooperAJGriffithsMRThe selective vulnerability of striatopallidal neuronsProg Neurobiol199959669171910845758
- AntoniniALeendersKLSpiegelRStriatal glucose metabolism and dopamine D2 receptor binding in asymptomatic gene carriers and patients with Huntington’s diseaseBrain1996119Pt 6208520959010012
- AugoodSJFaullRLEmsonPCDopamine D1 and D2 receptor gene expression in the striatum in Huntington’s diseaseAnn Neurol19974222152219266732
- FeiginATangCMaYThalamic metabolism and symptom onset in preclinical Huntington’s diseaseBrain2007130Pt 112858286717893097
- AndrewsTCWeeksRATurjanskiNHuntington’s disease progression. PET and clinical observationsBrain1999122Pt 122353236310581228
- WeeksRAPicciniPHardingAEStriatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington’s diseaseAnn Neurol199640149548687191
- ChaJHKosinskiCMKernerJAAltered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease geneProc Natl Acad Sci U S A19989511648064859600992
- PerryTLHansenSKlosterMHuntington’s chorea. Deficiency of gamma-aminobutyric acid in brainN Engl J Med197328873373424345566
- PaulsenJSFunctional imaging in Huntington’s diseaseExp Neurol2009216227227719171138
- Gourfinkel-AnIParainKHartmannAChanges in GAD67 mRNA expression evidenced by in situ hybridization in the brain of R6/2 transgenic miceJ Neurochem20038661369137812950446
- NicniocaillBHaraldssonBHanssonOAltered striatal amino acid neurotransmitter release monitored using microdialysis in R6/1 Huntington transgenic miceEur J Neurosci200113120621011135020
- LiSHLiXJHuntingtin-protein interactions and the pathogenesis of Huntington’s diseaseTrends Genet200420314615415036808
- BenceNFSampatRMKopitoRRImpairment of the ubiquitin-proteasome system by protein aggregationScience200129255211552155511375494
- RavikumarBDudenRRubinszteinDCAggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagyHum Mol Genet20021191107111711978769
- ChaJHTranscriptional dysregulation in Huntington’s diseaseTrends Neurosci200023938739210941183
- CepedaCHurstRSCalvertCRTransient and progressive electrophysiological alterations in the corticostriatal pathway in a mouse model of Huntington’s diseaseJ Neurosci200323396196912574425
- LudolphACHeFSpencerPSHammerstadJSabriM3-Nitropropionic acid-exogenous animal neurotoxin and possible human striatal toxinCan J Neurol Sci19911844924981782616
- GunawardenaSGoldsteinLSPolyglutamine diseases and transport problems: deadly traffic jams on neuronal highwaysArch Neurol2005621465115642849
- BrowneSEBealMFThe energetics of Huntington’s diseaseNeurochem Res200429353154615038601
- HarperPSThe epidemiology of Huntington’s diseaseHum Genet19928943653761535611
- MorrisonPJJohnstonWPNevinNCThe epidemiology of Huntington’s disease in Northern IrelandJ Med Genet19953275245307562964
- PeterlinBKobalJTeranNEpidemiology of Huntington’s disease in SloveniaActa Neurol Scand2009119637137518976322
- AlonsoMEOchoaABollMCClinical and genetic characteristics of Mexican Huntington’s disease patientsMov Disord200924132012201519672992
- SimpsonSAJohnstonAWThe prevalence and patterns of care of Huntington’s chorea in GrampianBr J Psychiatry19891557998042533515
- PenneyJBJrYoungABShoulsonIHuntington’s disease in Venezuela: 7 years of follow-up on symptomatic and asymptomatic individualsMov Disord19905293992139171
- HarperPSThe epidemiology of Huntington’s diseaseBatesGHarperPJonesLHuntington’s disease3rd edOxfordMonographs on Medical Genetics2002
- RibaiPNguyenKHahn-BarmaVPsychiatric and cognitive difficulties as indicators of juvenile huntington disease onset in 29 patientsArch Neurol200764681381917562929
- BiglanKMRossCALangbehnDRPREDICT-HD Investigators of the Huntington Study GroupMotor abnormalities in premanifest persons with Huntington’s disease: the PREDICT-HD studyMov Disord200924121763177219562761
- TabriziSJLangbehnDRLeavittBRTRACK-HD investigatorsBiological and clinical manifestations of Huntington’s disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline dataLancet Neurol20098979180119646924
- Huntington Study Group PHAROS Investigators (Shoulson I, primary author)At risk for Huntington’s disease: The PHAROS (Prospective Huntington At Risk Observational Study) cohort enrolledArch Neurol200663799199616831969
- Huntington Study GroupCooperative Huntington’s Observational Research Trial [ClinicalTrials.gov identifier NCT00313495]US National Institutes of Health Available from: http://www.clinicaltrials.govAccessed May 3, 2010
- SolomonACStoutJCJohnsonSAPredict-HD Investigators of the Huntington Study GroupVerbal episodic memory declines prior to diagnosis in Huntington’s diseaseNeuropsychologia20074581767177617303196
- JohnsonSAStoutJCSolomonACPredict-HD Investigators of the Huntington Study GroupBeyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington’s diseaseBrain2007130Pt 71732174417584778
- PaulsenJSLangbehnDRStoutJCPredict-HD Investigators and Coordinators of the Huntington Study GroupDetection of Huntington’s disease decades before diagnosis: the Predict-HD studyJ Neurol Neurosurg Psychiatry200879887488018096682
- DuffKPaulsenJSBeglingerLJPredict-HD Investigators of the Huntington Study GroupPsychiatric symptoms in Huntington’s disease before diagnosis: the predict-HD studyBiol Psychiatry200762121341134617481592
- Huntington Study GroupUnified Huntington’s Disease Rating Scale: reliability and consistencyMov Disord19961121361428684382
- The American College of Medical Genetics/American Society of Human Genetics Huntington Disease Genetic Testing Working GroupACMG/ASHG statement. Laboratory guidelines for Huntington disease genetic testingAm J Hum Genet1998625124312479545416
- AndrichJArningLWieczorekSKrausPHGoldRSaftCHuntington’s disease as caused by 34 CAG repeatsMov Disord200823687988118307262
- KenneyCPowellSJankovicJAutopsy-proven Huntington’s disease with 29 trinucleotide repeatsMov Disord200722112713017115386
- SnellRGMacMillanJCCheadleJPRelationship between trinucleotide repeat expansion and phenotypic variation in Huntington’s diseaseNat Genet1993443933978401588
- DiMLSquitieriFNapolitanoGCampanellaGTrofatterJAConneallyPMSuicide risk in Huntington’s diseaseJ Med Genet19933042932958487273
- PollardJHurry Up and Wait: a cognitive care companion9/25/08 142 pagesLulu press
- AdamORJankovicJSymptomatic treatment of Huntington diseaseNeurotherapeutics20085218119718394562
- PhillipsWShannonKMBarkerRAThe current clinical management of Huntington’s diseaseMov Disord200823111491150418581443
- RozeESaudouFCabocheJPathophysiology of Huntington’s disease: from huntingtin functions to potential treatmentsCurr Opin Neurol200821449750318607213
- ImarisioSCarmichaelJKorolchukVHuntington’s disease: from pathology and genetics to potential therapiesBiochem J200841219120918466116
- WalkerFOHuntington’s diseaseLancet2007369955721822817240289
- JankovicJTreatment of hyperkinetic movement disordersLancet Neurol20098984485619679276
- BonelliRMWenningGKPharmacological management of Huntington’s disease: an evidence-based reviewCurr Pharm Des200612212701272016842168
- MestreTFerreiraJCoelhoMMRosaMSampaioCTherapeutic interventions for symptomatic treatment in Huntington’s diseaseCochrane Database Sys Rev20093CD006456
- NakamuraKAminoffMJHuntington’s disease: clinical characteristics, pathogenesis and therapiesDrugs Today (Barc)20074329711617353947
- HandleyOJNajiJJDunnettSBRosserAEPharmaceutical, cellular and genetic therapies for Huntington’s diseaseClin Sci (Lond)20061101738816336206
- BonelliRMHofmannPA review of the treatment options for Huntington’s diseaseExpert Opin Pharmacother20045476777615102562
- GrimbergenYARoosRATherapeutic options for Huntington’s diseaseCurr Opin Investig Drugs2003415154
- FrankSAJankovicJAdvances in the pharmacological management of Huntington’s DiseaseDrugs201070556157120329804
- PaleacuDTetrabenazine in the treatment of Huntington’s DiseaseNeuropsychiatr Dis Treat20073554555119381278
- BagchiSPDifferential interactions of phencyclidine with tetrabenazine and reserpine affecting intraneuronal dopamineBiochem Pharmacol19833219285128566626259
- PettiboneDJPfluegerABTotaroJATetrabenazine-induced depletion of brain monoamines: mechanism by which desmethylimipramine protects cortical norepinephrineEur J Pharmacol198410234431436
- MehvarRJamaliFConcentration-effect relationships of tetrabenazine and dihydrotetrabenazine in the ratJ Pharm Sci19877664614653625491
- ThibautFFaucheuxBAMarquezJRegional distribution of monoamine vesicular uptake sites in the mesencephalon of control subjects and patients with Parkinson’s disease: a postmortem study using tritiated tetrabenazineBrain Res1995692122332438548292
- KenneyCHunterCDavidsonAJankovicJShort-term effects of tetrabenazine on chorea associated with Huntington’s diseaseMov Disord2007221101317078062
- SchermanDHenryJPReserpine binding to bovine chromaffin granule membranes. Characterization and comparison with dihydrotetrabenazine bindingMol Pharmacol19842511131226708929
- MehvarRJamaliFWatsonMWSkeltonDPharmacokinetics of tetrabenazine and its major metabolite in man and rat. Bioavailability and dose dependency studiesDrug Metab Dispos19871522502552882986
- RobertsMSWatsonHMMcLeanSMillingenKSDetermination of therapeutic plasma concentrations of tetrabenazine and an active metabolite by high-performance liquid chromatographyJ Chromatogr198122611751826459332
- RobertsMSMcLeanSMillingenKSGallowayHMThe pharmacokinetics of tetrabenazine and its hydroxy metabolite in patients treated for involuntary movement disordersEur J Clin Pharmacol19862967037083709613
- Huntington Study GroupTetrabenazine as antichorea therapy in Huntington disease: a randomized controlled trialNeurology200666336637216476934
- JankovicJBeachJLong-term effects of tetrabenazine in hyperkinetic movement disordersNeurology19974823583629040721
- KenneyCHunterCJankovicJLong-term tolerability of tetrabenazine in the treatment of hyperkinetic movement disordersMov Disord200722219319717133512
- FasanoACadedduFGuidubaldiAThe long-term effect of tetrabenazine in the management of Huntington diseaseClin Neuropharmacol200831631331819050408
- FrankSTetrabenazine as anti-chorea therapy in Huntington disease: an open-label continuation study. Huntington Study Group/TETRA-HD InvestigatorsBMC Neurol200996220021666
- FrankSOndoWFahnSA study of chorea after tetrabenazine withdrawal in patients with Huntington’s diseaseClin Neuropharmacol200831312713318520979
- QuinnNMarsdenCDA double blind trial of sulpiride in Huntington’s disease and tardive dyskinesiaJ Neurol Neurosurg Psychiatry19844788448476236286
- DerooverJBaroFBourguignonRPSmetsPTiapride versus placebo: a double-blind comparative study in the management of Huntington’s choreaCurr Med Res Opin1984953293386241563
- LeonardDPKidsonMABrownJGShannonPJTaryanSA double blind trial of lithium carbonate and haloperidol in Huntington’s choreaAust N Z J Psychiatry197592115118125578
- BarrANFischerJHKollerWCSpuntALSinghalASerum haloperidol concentration and choreiform movements in Huntington’s diseaseNeurology198838184882962009
- BonelliRMMahnertFANiederwieserGOlanzapine for Huntington’s disease: an open label studyClin Neuropharmacol200225526326512410058
- BonelliRMNiederwieserGTriblGGKöltringerPHigh-dose olanzapine in Huntington’s diseaseInt Clin Psychopharmacol2002172919311890191
- PaleacuDAncaMGiladiNOlanzapine in Huntington’s diseaseActa Neurol Scand2002105644144412027832
- SquitieriFCannellaMPiorcelliniABrusaLSimonelliMRuggieriSShort-term effects of olanzapine in Huntington diseaseNeuropsychiatry Neuropsychol Behav Neurol2001141697211234911
- DippleHCThe use of olanzapine for movement disorder in Huntington’s disease: a first case reportJ Neurol Neurosurg Psychiatry199967112312410454874
- CankurtaranESOzalpESoygurHCakirAClinical experience with risperidone and memantine in the treatment of Huntington’s diseaseJ Natl Med Assoc20069881353135516916137
- ErdemogluAKBoratavCRisperidone in chorea and psychosis of Huntington’s diseaseEur J Neurol20029218218311882064
- MadhusoodananSBrennerRUse of risperidone in psychosis associated with Huntington’s diseaseAm J Geriatr Psychiatry1998643473499793585
- ParsaMASzigethyEVociJMMeltzerHYRisperidone in treatment of choreoathetosis of Huntington’s diseaseJ Clin Psychopharmacol199717213413510950488
- AlpayMKoroshetzWJQuetiapine in the treatment of behavioral disturbances in patients with Huntington’s diseasePsychosomatics2006471707216384811
- SeitzDPMillsonRCQuetiapine in the management of psychosis secondary to Huntington’s disease: a case reportCan J Psychiatry200449641315283541
- BonelliRMNiederwieserGQuetiapine in Huntington’s disease: a first case reportJ Neurol200224981114111512420714
- van VugtJPSieslingSVergeerMvan der VeldeEARoosRAClozapine versus placebo in Huntington’s disease: a double blind randomised comparative studyJ Neurol Neurosurg Psychiatry199763135399221965
- CiammolaASassoneJColciagoCAripiprazole in the treatment of Huntington’s disease: a case seriesNeuropsychiatr Dis Treat200951419557093
- BrusaLOrlacchioAMoschellaVIaniCBernardiGMercuriNBTreatment of the symptoms of Huntington’s disease: preliminary results comparing aripiprazole and tetrabenazineMov Disord200924112612919170197
- LinWCChouYHAripiprazole effects on psychosis and chorea in a patient with Huntington’s diseaseAm J Psychiatry200816591207120818765501
- LucettiCGambacciniGBernardiniSAmantadine in Huntington’s disease: open-label video-blinded studyNeurol Sci200223Suppl 2S83S8412548355
- StewartJTAdverse behavioral effects of amantadine therapy in Huntington’s diseaseSouth Med J19878010132413252958943
- Huntington Study GroupDosage effects of riluzole in Huntington’s disease: a multicenter placebo-controlled studyNeurology200361111551155614663041
- LandwehrmeyerGBDuboisBde YebenesJGRiluzole in Huntington’s disease: a 3-year, randomized controlled studyAnn Neurol200762326227217702031
- PeirisJBBoralessaHLionelNDClonazepam in the treatment of choreiform activityMed J Aust197618225227131236
- FrattolaLAlbizzatiMGAlemaniABassiSFerrareseCTrabucchiMAcute treatment of Huntington’s chorea with lisurideJ Neurol Sci19835922472536222164
- VitaleCMarconiSDiMLShort-term continuous infusion of apomorphine hydrochloride for treatment of Huntington’s chorea: a double blind, randomized cross-over trialMov Disord200722162359236417894335
- CorsiniGUOnaliPMasalaCCianchettiCMangoniAGessaGApomorphine hydrochloride-induced improvement in Huntington’s chorea: stimulation of dopamine receptorArch Neurol19783512730145840
- LowPAAllsopJLHalmagyiGMHuntington’s chorea: the rigid form (Westphal variant) treated with levodopaMed J Aust19741113933944275254
- LowPAAllsopJLHuntington’s chorea – the rigid form (Westphal variant) treated with l-DOPA: a case reportProc Aust Assoc Neurol197310045464275960
- MagnetMKBonelliRMKapfhammerHPAmantadine in the akinetic-rigid variant of Huntington’s diseaseAnn Pharmacother2004387811941196
- BonelliRMNiederwieserGDiezJGruberAKöltringerPPramipexole ameliorates neurologic and psychiatric symptoms in a Westphal variant of Huntington’s diseaseClin Neuropharmacol2002251586011852299
- http://www.xenazineusa.comAccessed 3 May 2010