From Dr. Blair R. Leavitt Director of Clinical ResearchIntroduction to RESEARCH at the CHD |
| The Centre for Huntington Disease at the University of British Columbia Hospital in Vancouver BC Canada, offers expert multi-disciplinary care to individuals and families affected by HD. In addition, the CHD provides an opportunity to become involved in cutting-edge research, including clinical trials of potential new therapies for HD. The hope of all our research efforts is to improve our understanding of HD, advance the management of HD symptoms, and ultimately to find a cure for this devastating disorder. |
| What TREATMENTS are available for HD? |
| Treatments for HD can be divided into two categories: those that are aimed at treating the symptoms of the illness (like depression or chorea), and those that slow down the underlying progression of the illness. Although there are currently no treatments proven to slow down progression or to delay the onset of HD, there is research that aims to identify such treatments. In addition, development of improved symptomatic treatments is also an area of active clinical research. |
The entire HD community benefits from clinical research… every study we complete brings us closer to the day when a cure for HD is available. |
| WHY get involved in HD RESEARCH? |
| From scientists conducting basic studies in animals, to doctors running clinical trials, the entire HD research community is engaged in a cooperative endeavor to develop treatments and to eventually cure HD. Cooperation between researchers is critical to this effort, but these efforts would be futile without the engagement and active participation of HD patients and families. The CHD is active in a number of clinical trials. This provides an opportunity for families affected by HD to contribute directly to the research effort. Our research staff at the CHD have decades of experience working with families to ensure volunteers for our clinical trials are well informed and feel valued for their contribution. |
Joji Decolongon MSc, CCRPClinical Research Managertel: 604-822-7928 |
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FUNDING for RESEARCH at the CHD
HD research at the CHD is made possible through the generous support of individual donors, families, HD organizations, foundations, and industry sponsors. We are grateful to all of our supporters for their valuable contributions to the fight against HD.
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If you would like to help directly fund HD research at the Centre for HD at UBC and to advance the welfare of HD patients and their families, please consider donating to the CHD.
Clinical Research Trials:
Click on the name of the clinical trial to reveal details about the trial
This study aims to extend the success of TRACK-HD in identifying the best measurements to detect changes in HD gene-carriers who have not yet developed the symptoms of HD. TRACK-ON involves 240 participants from 4 international sites: Leiden (The Netherlands), London (UK), Paris (France), Vancouver (Canada). TRACK-ON launched April 2012 and will run for 3 years. Enrollment is closed. For more information, contact Joji Decolongon.
NEUROBIOLOGICAL PREDICTORS OF HUNTINGTON DISEASE The purpose of this study is to prepare for a trial of experimental drugs aimed at slowing or postponing the onset of HD. It aims to address questions about: (1) the earliest changes in the brain, intellect, and behaviour; (2) neurobiological factors that influence the age of onset; and (3) the most accurate tests that clinicians can use in detecting HD onset. The study is ongoing but enrollment is closed.For more information, contact Joji Decolongon.
A PHASE III, RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED, SAFETY AND EFFICACY STUDY OF COENZYME Q10 IN HD This is a clinical trial investigating the effect of high-dose Coenzyme Q10 (2400 mg/day) in slowing progression of HD. Coenzyme Q10 (CoQ10) is present in all human cells, and increases cell energy and combats harmful substances, functions that are particularly important in the brain. An earlier study (CARE-HD) investigating CoQ10 at a lower dose of 600 mg/day, yielded inconclusive results as to its effect in slowing HD progression. 2CARE is ongoing but enrollment is closed.For more information, contact Joji Decolongon.
A TRIAL OF MEMANTINE AS SYMPTOMATIC TREATMENT FOR EARLY HUNTINGTON DISEASE (MITIGATE-HD)The goal of this study is to determine the effect of memantine on movement, thinking, and emotional difficulties in HD patients. MITIGATE-HD is a single-centre, Phase IIb, randomized, double-blind, placebo-controlled trial of memantine treatment. This study enrolled 25 participants from September 2011 to September 2012 and was completed in January 2013.
A PROSPECTIVE REGISTRY STUDY IN A GLOBAL HD COHORT (ENROLL-HD)Launched in January 2013, ENROLL-HD aims to accelerate the development of therapies for HD. An observational trial, ENROLL-HD does not involve any intervention(s). This study is actively enrolling participants.For more information, contact Mike Adurogbangba
A MULTI-CENTRE, North American, open-label extension study of Prdopidine (ACR16) in the syptomatic treatment of Huntington disease. Started in 2011, this trial offered continued treatment to HD patients who previously participated in clinically controlled studies and who have wished to continue their treatment. The Open-HART study concluded in December 2017. For more information, contact Mike Adurogbangba
Open-Label Extension Study of Remacemide Hydrochloride (OLE) The purpose of this trial was to assess the safety of long-term (28-month) exposure to remacemide hydrochloride (an investigational medication) in people with Huntington's Disease, as well as analyse the effect of remacemide hydrochloride on chorea, mood and mental functioning. The study was terminated because CARE-HD results showed that remacemide does not benefit HD patients.
Prospective Huntington At Risk Observational Study (PHAROS) The PHAROS Project was an observational study in North America (US and Canada) of people ages 26 to 55 who were at risk for Huntington's Disease. During the study, subjects were evaluated every nine (9) months (for a minimum period of 3 years) using a clinical survey known as the Unified Huntington's Disease Rating Scale (UHDRS), which looks at movement, psychological and behavioral function. The study has finished.
Riluzole Dosing in Huntington's Disease (RID-HD) The purpose of this trial was to analyze the short term effect of riluzole on chorea, mood and mental functioning as well as assess tolerability of different doses. Riluzole decreases the release of glutamate from neurons. It is also possible that it protects neurons from cell death by other mechanisms. Results showed that although riluzole showed some effect on chorea at the higher dose tested, it had unfavourable side-effects and it did not appear to benefit other features of HD. The study has finished.
A Study of Treatment with Ethyl-eicosapentaenoate in Patients with HD (Ethyl-EPA) The purpose of this trial was to analyze the effect of ethyl-eicosapentaenoate (ethyl-EPA) on chorea, mood, and mental functioning. Ethyl-EPA may prevent degeneration of nerve cells by preventing stress and damage to their external and internal membranes. It was found that patients with a CAG repeat length of less than 45 showed some improvement over a 12-month period. Results from a follow-up study were inconclusive. The study has finished.
This is a small trial aimed at testing the safety of ISIS-HTTRx in a limited number of individuals with HD. Enrollment for this study is closed. Please continue to watch this site for updates.
A MULTI-CENTRE, International, cerebrospinal fluid collection initiative to facilitate therapeutic development for Huntington’s disease. Started in 2017, further enrollment will continue in Fall 2017. Please continue to watch this site for updates. For more information, contact Tuan Le.
Vaccinex and the Huntington Study Group (HSG) launched a trial for people with the gene mutation that causes HD who are either early in the progression of the disease or are not yet diagnosed with the disease.
The SIGNAL trial is designed to assess the safety, tolerability, and effectiveness of VX15, a novel monoclonal antibody, in people with late prodromal (not yet diagnosed) or early manifest (early in the progression of the disease) HD. It is a Phase 2, multi-center, randomized, double-blind, placebo-controlled study being conducted at approximately 30 sites across the United States and Canada. The trial seeks to enroll a total of 240 individuals, 21 or older. Please continue to watch this site for updates. For more information, contact Tuan Le.
HD Clinical Research Publications
Byrne LM, Rodrigues FB, Blennow K, Durr A, Leavitt BR, Roos RAC, Scahill RI, Tabrizi SJ, Zetterberg H, Langbehn D, Wild EJ (2017) Neurofilament light protein in blood as a potential biomarker of neurodegeneration in Huntington’s disease: a retrospective cohort analysis [ePub ahead of print]
Quinti L, Dayalan Naidu S, Träger U, Chen X, Kegel-Gleason K, Llères D, Connolly C, Chopra V, Low C, Moniot S, Sapp E, Tousley AR, Vodicka P, Van Kanegan MJ, Kaltenbach LS, Crawford LA, Fuszard M, Higgins M, Miller JRC, Farmer RE, Potluri V, Samajdar S, Meisel L, Zhang N, Snyder A, Stein R, Hersch SM, Ellerby LM, Weerapana E, Schwarzschild MA, Steegborn C, Leavitt BR, Degterev A, Tabrizi SJ, Lo DC, DiFiglia M, Thompson LM, Dinkova-Kostova AT, Kazantsev AG. (2017) KEAP1-modifying small molecule reveals muted NRF2 signaling responses in neural stem cells from Huntington's disease patients. Proceedings of the National Academy of Sciences USA May 22 SG [ePub ahead of print]
McColgan P, Razi A, Gregory S, Seunarine KK, Durr A, Roos RAC, Leavitt BR, Scahill RI, Clark CA, Lanbehn D, Rees G, Tabrizi SJ and the Track On-HD Investigators (2017) Structural and functional brain network correlates of depressive symptoms in Premanifest Huntington’s disease. Human Brain Mapping Jun;38(6):2819-2829
McColgan P, Seunarine KK, Gregory S, Razi A, Papoutsi M, Long JD, Mills JA, Johnson E, Durr A, Roos RAC, Leavitt BR, Stout JC, Scahill RI, Clark CA, Rees G, Tabrizi SJ, TRACK ON Investigators (2017) Topological length of white matter connections predicts their rate of atrophy in premanifest Huntington's disease. Journal of Clinical Investigation Insight Apr 20;2(8)
Gregory S, Long JD, Klöppel S, Razi A, Scheller E, Minkova L, Papoutsi M, Mills JA, Durr A, Leavitt BR, Roos RA, Stout JC, Scahill RI, Langbehn DR, Tabrizi SJ, and Rees G (2017) Operationalizing compensation over time in neurodegenerative disease. Brain Apr 1;140(4):1158-1165
McColgan P, Gregory S, Razi a, Seunarine KK, Gargouri F, Durr A, Roos RAC, Leavitt BR, Scahill RI, Clark CA, Tabrizi SJ, Rees G and the Track On-HD Investigators (2017) White matter predicts functional connectivity in premanifest Huntington’s disease. Annals of Clinical and Translational Neurology Jan 16;4(2):106-118
McGarry A, McDermott M, Kieburtz K, de Blieck EA, Beal F, Marder K, Ross C, Shoulson I, Gilbert P, Mallonee WM, Guttman M, Wojcieszek J, Kumar R, LeDoux MS, Jenkins M, Rosas HD, Nance M, Biglan K, Como P, Dubinsky RM, Shannon KM, O'Suilleabhain P, Chou K, Walker F, Martin W, Wheelock VL, McCusker E, Jankovic J, Singer C, Sanchez-Ramos J, Scott B, Suchowersky O, Factor SA, Higgins DS Jr, Molho E, Revilla F, Caviness JN, Friedman JH, Perlmutter JS, Feigin A, Anderson K, Rodriguez R, McFarland NR, Margolis RL, Farbman ES, Raymond LA, Suski V, Kostyk S, Colcher A, Seeberger L, Epping E, Esmail S, Diaz N, Fung WL, Diamond A, Frank S, Hanna P, Hermanowicz N, Dure LS, Cudkowicz M; Huntington Study Group 2CARE Investigators and Coordinators (2017) A randomized, double-blind, placebo-controlled trial of coenzyme Q10 in Huntington disease. Neurology Jan 10;88(2):152-159
Carvalho JO, Long JD, Westervelt HJ, Smith MM, Bruce JM, Kim JI, Mills JA, Paulsen JS; PREDICT-HD Investigators And Coordinators Of The Huntington Study Group (2016) The impact of oculomotor functioning on neuropsychological performance in Huntington disease. Journal of Clinical and Experimental Neuropsycholy. Jan 08;38(2):217-26
Minkova L, Eickhoff SB, Abdulkadir A, Kaller CP, Peter J, Scheller E, Lahr J, Roos RA, Durr A, Leavitt BR, Tabrizi SJ, Klöppel S; TRACK-HD Investigators (2016) Large-scale brain network abnormalities in Huntington's disease revealed by structural covariance. Human Brain Mapping. 37(1): 67-80
Huntington Study Group PHAROS Investigators (2016) Clinical-Genetic Associations in the Prospective Huntington at Risk Observational Study (PHAROS): Implications for Clinical Trials. JAMA Neurology. 73(1): 102-10
Keogh R, Frost C, Owen G, Daniel RM, Langbehn DR, Leavitt BR, Durr A, Roos RA, Landwehrmeyer GB, Reilmann R, Borowsky B, Stout J, Craufurd D, Tabrizi SJ. (2016) Medication Use in Early-HD Participants in Track-HD: an Investigation of its Effects on Clinical Performance. PLoS Currents. 8
Wiecki TV, Antoniades CA, Stevenson A, Kennard C, Borowsky B, Owen G, Leavitt BR, Roos R, Durr A, Tabrizi SJ, Frank MJ (2016) Computational Cognitive Biomarker for Early-Stage Huntington's Disease. PLoS One. 11(2)
De Souza RA, Islam SA, McEwen LM, Mathelier A, Hill A, Mah SM, Wasserman WW, Kobor MS, Leavitt BR (2016) DNA methylation profiling in human Huntington's disease brain. Human Molecular Genetics 15:25 (10):2013-2030
Wagner L, Björkqvist M, Hult Lundh S, Wolf R, Börgel A, Schlenzig D, Ludwig HH, Rahfeld JU, Leavitt BR, Demuth HU, Petersén Å, von Hörsten S. Neuropeptide Y (NPY) in cerebrospinal fluid from patients with Huntington's Disease: increased NPY levels and differential degradation of the NPY1-30 fragment. Journal of Neurochemisty. Jun;137(5):820-37
Huntington Study Group, Frank S, Testa CM, Stamler D, Kayson E, Davis C, Edmondson MC, Kinel S, Leavitt BR, Oakes D, O'Neill C, Vaughan C, Goldstein J, Herzog M, Snively V, Whaley J Wong C, Suter G, Jankovic J, Jimenez-Shahed J, Hunter C, Claassen DO, Roman OC, Sung V, Smith J, Janicki S, Clouse R, Saint-Hilaire M, Hohler A, Turpin D, James RC, Rodriguez R, Rizer K, Anderson KE, Heller H, Carlson A, Criswell S, Racette BA, Revilla FJ, Nucifora F Jr, Margolis RL, Ong M, Mendis T, Mendis N, Singer C, Quesada M, Paulsen JS, Brashers-Krug T, Miller A, Kerr J, Dubinsky RM, Gray C, Factor SA, Sperin E, Molho E, Eglow M, Evans S, Kumar R, Reeves C, Samii A, Chouinard S, Beland M, Scott BL, Hickey PT, Esmail S, Fung WL, Gibbons C, Qi L, Colcher A, Hackmyer C, McGarry A, Klos K, Gudesblatt M, Fafard L, Graffitti L, Schneider DP, Dhall R, Wojcieszek JM, LaFaver K, Duker A, Neefus E, Wilson-Perez H, Shprecher D, Wall P, Blindauer KA, Wheeler L, Boyd JT, Houston E, Farbman ES, Agarwal P, Eberly SW, Watts A, Tariot PN, Feigin A, Evans S, Beck C, Orme C, Edicola J, Christopher E (2016) Effect of Deutetrabenazine on Chorea Among Patients With Huntington Disease: A Randomized Clinical Trial. Journal of American Medicine Association Jul 5;316(1):40-50
Labuschagne I, Cassidy AM, Scahill RI, Johnson EB, Rees E, O'Regan A, Queller S, Frost C, Leavitt BR, Dürr A, Roos R, Owen G, Borowsky B, Tabrizi SJ, Stout JC; TRACK-HD Investigators (2016) Visuospatial Processing Deficits Linked to Posterior Brain Regions in Premanifest and Early Stage Huntington's Disease. Journal of the International Neuropsychological Society Jul;22(6):595-608
Orth M, Gregory S, Scahill RI, Mayer IS, Minkova L, Klöppel S, Seunarine KK, Boyd L, Borowsky B, Reilmann R, Bernhard Landwehrmeyer G, Leavitt BR, Roos RA, Durr A, Rees G, Rothwell JC, Langbehn D, Tabrizi SJ; TRACK-On Investigators (2016) Natural variation in sensory-motor white matter organization influences manifestations of Huntington's disease. Human Brain Mapping Dec;37(12):4615-4628
Rodrigues FB, Byrne L, McColgan P, Robertson N, Tabrizi SJ, Leavitt BR, Zetterberg H, Wild EJ (2016) Cerebrospinal fluid total tau concentration predicts clinical phenotype in Huntington's disease. Journal of Neurochemistry Oct;139(1):22-5
Marie-Hélène Disatnik, Amit U. Joshi, Nay L. Saw, Mehrdad Shamloo, Blair R. Leavitt, Xin Qi, Daria Mochly-Rosen (2016) Potential biomarkers to follow the progression and treatment response of Huntington’s disease. Journal of Experimental Medicine Nov 14; 213(12): 2655–2669
Liu D, Long JD, Zhang Y, Raymond LA, Marder K, Rosser A, McCusker EA, Mills JA, Paulsen JS; PREDICT-HD Investigators and Coordinators of the Huntington Study Group (2015) Motor onset and diagnosis in Huntington disease using the diagnostic confidence level. J Neurol. 2015 Dec;262(12):2691-2698
Träger U, Andre R, Magnusson-Lind A, Miller JR, Connolly C, Weiss A, Grueninger S, Silajdžić E, Smith DL, Leavitt BR, Bates GP, Björkqvist M, Tabrizi SJ (2015) Characterization of immune cell function in fragment and full-length Huntington's disease mouse models. Neurobiology of Disease. 73: 388-98
Huntington Study Group Reach2HD Investigators (2015) Safety, tolerability, and efficacy of PBT2 in Huntington's disease: a phase 2, randomised, double-blind, placebo-controlled trial. The Lancet Neurology. (1): 39-47
Kim JI, Long JD, Mills JA, Downing N, Williams JK, Paulsen JS; PREDICT-HD Investigators and Coordinators of the Huntington Study Group (2015) Performance of the 12-item WHODAS 2.0 in prodromal Huntington disease. European Journal of Human Genetics Nov;23(11):1584-7
Sturrock A, Laule C, Wyper K,, Milner RA, Decolongon, Dar Santos R, Coleman AJ, Carter K, Creighton S, Bechtel N, Bohlen S, Reilmann R, Johnson HJ, Hayden MR, Tabrizi SJ, Mackay AL, Leavitt BR (2015) A longitudinal study of magnetic resonance spectroscopy Huntington's disease biomarkers. Movement Disorders. 30(3): 393–401
Callaghan J, Stopford C, Arran N, Boisse MF, Coleman A, Santos RD, Dumas EM, Hart EP, Justo D, Owen G, Read J, Say MJ, Durr A, Leavitt BR, Roos RA, Tabrizi SJ, Bachoud-Levi AC, Bourdet C, van Duijn E, Craufurd D (2015) Reliability and Factor Structure of the Short Problem Behaviors Assessment for Huntington's Disease (PBA-s) in the TRACK-HD and REGISTRY studies. Journal of Neuropsychiatry and Clinical Neuroscience. 27(1): 59-64
Wild EJ, Boggio R, Langbehn D, Robertson N, Haider S, Miller JR, Zetterberg H, Leavitt BR, Kuhn R, Tabrizi SJ, Macdonald D, Weiss A (2015) Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients. Journal of Clinical Investigation. 125(5): 1979-86
Howard AK, Krishnamoorthy A, Leavitt BR, Raymond LA, Weissman CR (2015) Treatment of Huntington Disease and Comorbid Trichotillomania With Aripiprazole. Journal of Neuropsychiatry and Clinical Neuroscience 27(3):211-2
Kim JI, Long JD, Mills JA, McCusker E, Paulsen JS; PREDICT-HD Investigators and Coordinators of the Huntington Study Group (2015) Multivariate clustering of progression profiles reveals different depression patterns in prodromal Huntington disease. Neuropsychology Nov;29(6):949-60
Jan A, Karasinska JM, Kang MH, de Haan W, Ruddle P, Kaur A, Connolly C, Leavitt BR, Sorensen PH, Hayden MR (2015) Direct intracerebral delivery of a miR-33 antisense oligonucelotide into mouse brain increases brain ABCA1 expression. Neuroscience Letters. 26(598): 66-72
Southwell AL, Smith SE, Davis TR, Caron NS, Villanueva EB, Xie Y, Collins JA, Li Ye M, Sturrock A, Leavitt BR, Schrum AG, Hayden MR (2015) Ultrasensitive measurement of huntingtin protein in cerebrospinal fluid demonstrates increase with Huntington disease stage and decrease following brain huntingtin suppression. Nature: Scientific Reports (5):12166
Plecash A, Coleman A, Leavitt BR (2015) Feasibility and Safety of an Aquatherapy Program in Mid- to Late-Stage Huntington Disease. International Journal of Neurorehabilitiation 2: 181
Klöppel S, Gregory S, Scheller E, Minkova L, Razi A, Durr A, Roos RA, Leavitt BR, Papoutsi M, Landwehrmeyer GB, Reilmann R, Borowsky B, Johnson H, Mills JA, Owen G, Stout J, Scahill RI, Long JD, Rees G, Tabrizi SJ; Track-On investigators (2015) Compensation in Preclinical Huntington's Disease: Evidence From the Track-On HD Study. BioMedicine. 4;2(10): 1420-9
Johnson EB, Rees EM, Labuschagne I, Durr A, Leavitt BR, Roos RA, Reilmann R, Johnson H, Hobbs NZ, Langbehn DR, Stout JC, Tabrizi SJ, Scahill RI (2015) The impact of occipital lobe cortical thickness on cognitive task performance: An investigation in Huntington's Disease. Neuropsychologia. 79(Pt A):138-46
Minkova L, Scheller E, Peter J, Abdulkadir A, Kaller CP, Roos RA, Durr A, Leavitt BR, Tabrizi SJ, Klöppel S; TRACK-ON HD Investigators (2015) Detection of Motor Changes in Huntington's Disease Using Dynamic Causal Modeling. Frontiers in Human Neuroscience 9:634
Wagner L, Wolf R, Zeitschel U, Rossner S, Petersén Å, Leavitt BR, Kästner F, Rothermundt M, Gärtner UT, Gündel D, Schlenzig D, Frerker N, Schade J, Manhart S, Rahfeld JU, Demuth HU, von Hörsten S (2015) Proteolytic degradation of neuropeptide Y (NPY) from head to toe: Identification of novel NPY-cleaving peptidases and potential drug interactions in CNS and Periphery. Journal of Neurochemistry. 135(5): 1019-37
Fisher ER and Hayden MR (2014) Multi-source ascertainment of Huntington disease in Canada: prevalence and population at risk. Movement Disorders Jan;29(1):105-14
Ehrnhoefer DE, Skotte NH, Ladha S, Nguyen YT, Qiu X, Deng Y, Huynh KT, Engemann S, Nielsen SMB, Becanovic K, Leavitt BR, Hasholt L, Hayden MR (2014) p53 Increases caspase-6 expression and activation in muscle tissue expressing mutant huntingtin. Human Molecular Genetetics Feb 1;23(3):717-29.
Ross CA, Aylward EH, Wild EJ, Langbehn DR, Long JD, Warner JH, Scahill RI, Leavitt BR, Stout JC, Paulsen JS, Reilmann R, Unschuld PG, Wexler A, Margolis RL, Tabrizi SJ (2014) Huntington disease: natural history, biomarkers and prospects for therapeutics. Nature Reviews: Neurology 10(4): 204-16
Saft C, Leavitt BR, Epplen JT (2014) Clinical utility gene card for: Huntington's disease. European Journal of Human Genetics 22(5)
Jones R, Stout JC, Labuschagne I, Say M, Justo D, Coleman A, Dumas EM, Hart E, Owen G, Durr A, Leavitt BR, Roos R, O'Regan A, Langbehn D, Tabrizi SJ, Frost C (2014) The potential of composite cognitive scores for tracking progression in Huntington's disease. Journal of Huntington’s Disease 3(2): 197-207.
Plecash AR, Leavitt BR (2014) Aquatherapy for neurodegenerative disorders. Journal of Huntington’s Disease (Invited review) 3(1): 5-11
Downing NR, Kim JI, Williams JK, Long JD, Mills JA, Paulsen JS; PREDICT-HD Investigators and Coordinators of the Huntington Study Group (2014) WHODAS 2.0 in prodromal Huntington disease: measures of functioning in neuropsychiatric disease. European Journal of Human Genetetics Aug;22(8):958-63
Kostro D, Abdulkadir A, Durr A, Roos R, Leavitt BR, Johnson H, Cash D, Tabrizi SJ, Scahill RI, Ronneberger O, Klöppel S; Track-HD Investigators (2014) Correction of inter-scanner and within-subject variance in structural MRI based automated diagnosing. Neuroimage. 98: 405-15
Long JD, Paulsen JS, Marder K, Zhang Y, Kim JI, Mills JA; Researchers of the PREDICT-HD Huntington's Study Group. (2014) Tracking motor impairments in the progression of Huntington's disease. Movement Disorders, Mar;29(3):311-319
Reilmann R, Leavitt BR, Ross C (2014) Huntington's disease: A field on the move. Movement Disorders (Invited review) 29(11): 1333-4.
Reilmann R, Leavitt BR, Ross CA (2014) Diagnostic criteria for Huntington's disease based on natural history. Movement Disorders (Invited review) 29(11): 1335-41
Paulsen JS, Long JD, Ross CA, Harrington DL, Erwin CJ, Williams JK, Westervelt HJ, Johnson HJ, Aylward EH, Zhang Y, Bockholt HJ, Barker RA; PREDICT-HD Investigators and Coordinators of the Huntington Study Group (2014) Prediction of manifest Huntington's disease with clinical and imaging measures: a prospective observational study. Lancet Neurology Dec;13(12):1193-2201
Baine FK, Kay C, Ketelaar ME, Collins JA, Semaka A, Doty CN, Krause A, Greenberg JL, Hayden MR. Huntington disease in the South African population occurs on diverse and ethnically distinct genetic haplotypes. Eur J Hum Genet 2013 Feb 25
Biglan KM, Zhang Y, Long JD, Geschwind M, Kang GA, Killoran A, Lu W, McCusker E, Mills JA, Raymond LA, Testa C, Wojcieszek J, Paulsen JS and the PREDICT-HD Investigators of the Huntington Study Group. (2013) Refining the diagnosis of Huntington disease: The PREDICT-HD study. Frontiers in Aging Neuroscience. 5:1-8.
PREDICT-HD Investigators and Coordinators of the Huntington Study Group (2013) Cognitive reserve and brain reserve in prodromal Huntington's Disease. Journal of the International Neuropsychological Society Journal of International Neuropsychology Society
Brunham LR, Hayden MR. Hunting human disease genes: lessons from the past, challenges for the future. Hum Genet. 2013 132(6):603-17
Marder K, Gu Y, Eberly S, Tanner CM, Scarmeas N, Oakes D, Shoulson I; Huntington Study Group PHAROS Investigators. (2013) Relationship of Mediterranean diet and caloric intake to phenoconversion in Huntington disease. JAMA Neurology 70(11):1382-8
PREDICT-HD Investigators and Coordinators of the Huntington Study Group (2013) Characterization of depression in prodromal Huntington disease in the neurobiological predictors of HD (PREDICT-HD) study. Journal of Psychiatric Research 47(10):1423-31.
Epping EA, Mills JA, Beglinger LJ, Fiedorowicz JG, Craufurd D, Smith MM, Groves M, Bijanki KR, Downing N, Williams JK, Long JD, Paulsen JS; PREDICT-HD Investigators and Coordinators of the Huntington Study Group. (2013) Characterization of depression in prodromal Huntington disease in the neurobiological predictors of HD (PREDICT-HD) study. Journal of Psychiatric Research, 47(10):1423-31
Franciosi S, Shim Y, Lau M, Hayden MR, Leavitt B (2013) A systematic review and meta-analysis of clinical variables used in Huntington disease research Movement Disorders Dec;28(14):1987-94
Hawkins AK, Creighton S, Hayden MR. When Access is an Issue: Exploring Barriers to Predictive Testing for Huntington Disease in British Columbia, Canada. European Journal of Human Genetics. 2013 21(2):148-53
Hawkins Virani A, Creighton S, Hayden MR Developing a Comprehensive, Effective Patient Friendly Website to Enhance Decision-Making in Predictive Testing for Huntington Disease.Genet Med. 2013 15(6):466-72
Houge G, Bruland O, Bjørnevoll I, Hayden MR, Semaka A. De novo huntington disease caused by 26-44. Cag repeat expansion on a low-risk haplotype. Neurology. 2013 Sep 17;81(12):1099-100
Marco S, Giralt A, Petrovic MM, Pouladi MA, Martínez-Turrillas R, Martínez-Hernández J, Kaltenbach L, Torres-Peraza J, Graham RK, Watanabe M, Luján R, Nakanishi N, Lipton SA, Lo DC, Hayden MR, Alberch,J, Wesseling JF , Pérez-Otaño I (2013) Suppressing aberrant GluN3A expression rescues synaptic and behavioral impairments in Huntington's disease models. Nature Medicine 19(8):1030-8.
Milnerwood AJ*, Parsons MP*, Young FB, Singaraja R, Franciosi S, Volta M, Bergeron S, Hayden MR, Raymond LA (2013) Memory and synaptic deficits in Hip 14/DHHC17 of knockout mice. Proceedings of the National Academy of Sciences of the USA. Dec 10;110(50):20296-301
Oestergaard M, Southwell A, Kordasiewicz H, Watt A, Skotte N, Doty C, Vaid K, Villanueva E, Swayze E, Bennett CF, Hayden M, Seth P. Rational Design of Antisense Oligonucleotides Targeting Single Nucleotide Polymorphisms for Potent and Allele Selective Suppression of Mutant Huntingtin in the CNS. Nucleic Acids Research. 2013 Nov;41(21):9634-50
Pouladi MA, Morton J, Hayden MR. Choosing an animal model for the study of Huntington's disease. Nat Rev Neurosci. 2013 (14) 708-721.
Ramos EM, Latourelle JC, Gillis T, Mysore JS, Squitieri F, Di Pardo A, Di Donato S, Gellera G, Hayden MR Morrison PJ, Nance M, Ross CA, Margolis RL, Gomez-Tortosa E, Ayuso C, Suchowersky O, Trent RJ, McCusker E, Novelletto A, Frontali M, Jones R, Ashizawa T, Frank S, Saint-Hilaire MH, Hersch SM, Rosas HD, Lucente D, Harrison MB, Zanko A, Abramson RK, Marder K, Gusella JF, Lee JM, Alonso I, Sequeiros J, Myers RH, MacDonald ME. Candidate glutamatergic and dopaminergic pathway gene variants do not influence Huntington’s disease motor onset. Neurogenetics 2013 Nov;14(3-4):173-9
Semaka A, Balneaves LG, Hayden MR.Grasping the Grey": Patient Understanding and Interpretation of an Intermediate Allele Predictive Test Result for Huntington Disease. J Genet Counsel. 2013. 22(2):200-17
Semaka A, Kay C, Doty C, Collins J, Tam N, Hayden MR. High Frequency of Intermediate Alleles on Huntington Disease-Associated Haplotypes in British Columbia’s General Population. AMJG Part B Neuropsychiatric Genetics. 2013 Aug 30. [Epub ahead of print]
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