Study Results
Results available
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View full resultsBasic Information
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Recruitment Status
TERMINATED
Clinical Phase
PHASE3
Total Enrollment
609 participants
Study Classification
INTERVENTIONAL
Study Start Date
2008-03-31
Study Completion Date
2015-05-31
Brief Summary
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The goals of this trial are to determine if coenzyme Q10 is effective in slowing the worsening symptoms of Huntington's disease and to learn about the safety and acceptability of long-term coenzyme Q10 use by determining its effects on people with Huntington's disease.
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Detailed Description
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Huntington's disease (HD) is a slowly progressive disorder that devastates the lives of those affected and their families. There are no treatments that slow the progression of HD, only mildly effective symptomatic therapies are available.
The purpose of this trial is to find out if coenzyme Q10 (CoQ) is effective in slowing the worsening symptoms of HD. In this study, researchers also will learn about the safety and acceptability of long-term CoQ use by determining its effects on people with HD.
Participants in this trial will be randomly chosen to one of two groups. Group 1 will receive CoQ (2400 mg/day), and group 2 will receive a placebo (an inactive substance). Researchers will compare the change in total functional capacity (TFC)-a measure of functional disability-in the two groups. The TFC is a valid and reliable measure of disease progression and is particularly responsive to change in the early and mid-stages of HD. Researchers will also compare the changes in other components of the Unified Huntington's Disease Rating Scale '99 (UHDRS) including: the total motor score, total behavioral frequency score, total behavior frequency X severity score, verbal fluency test, symbol digit modalities test, Stroop, interference test, functional checklist, and independence scale scores. The groups will also be compared with respect to tolerability, adverse events, vital signs, and laboratory test results as measures of safety.
The purpose of this trial is to find out if coenzyme Q10 (CoQ) is effective in slowing the worsening symptoms of HD. In this study, researchers also will learn about the safety and acceptability of long-term CoQ use by determining its effects on people with HD.
Participants in this trial will be randomly chosen to one of two groups. Group 1 will receive CoQ (2400 mg/day), and group 2 will receive a placebo (an inactive substance). Researchers will compare the change in total functional capacity (TFC)-a measure of functional disability-in the two groups. The TFC is a valid and reliable measure of disease progression and is particularly responsive to change in the early and mid-stages of HD. Researchers will also compare the changes in other components of the Unified Huntington's Disease Rating Scale '99 (UHDRS) including: the total motor score, total behavioral frequency score, total behavior frequency X severity score, verbal fluency test, symbol digit modalities test, Stroop, interference test, functional checklist, and independence scale scores. The groups will also be compared with respect to tolerability, adverse events, vital signs, and laboratory test results as measures of safety.
Conditions
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Huntington's Disease
Study Design
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Allocation Method
RANDOMIZED
Intervention Model
PARALLEL
Primary Study Purpose
TREATMENT
Blinding Strategy
TRIPLE
Participants
Caregivers
Investigators
Study Groups
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A - coenzyme Q10 2400 mg/day
Randomized to active treatment (coenzyme Q10 2400 mg/day)
Group Type
ACTIVE_COMPARATOR
coenzyme Q10
Intervention Type
DRUG
4 - 300 mg CoQ chewable wafers taken orally twice a day
B - Placebo
Randomized to placebo
Group Type
PLACEBO_COMPARATOR
placebo
Intervention Type
OTHER
an inactive substance
Interventions
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coenzyme Q10
4 - 300 mg CoQ chewable wafers taken orally twice a day
Intervention Type
DRUG
placebo
an inactive substance
Intervention Type
OTHER
Other Intervention Names
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CoQ
Eligibility Criteria
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Inclusion Criteria
To be eligible for enrollment into this study, subjects must meet the following eligibility criteria within 28 days prior to randomization:
* Subjects must have clinical features of HD and a confirmed family history of HD, OR a CAG repeat expansion ≥ 36.
* TFC \> 9.
* Must be ambulatory and not require skilled nursing care.
* Age ≥ 16 years.
* Women must not be able to become pregnant (e.g., post menopausal, surgically sterile or using adequate birth control methods for the duration of the study).
* If psychotropic medications are taken (e.g., anxiolytics, hypnotics, benzodiazepines, antidepressants), they must be at a stable dosage for four weeks prior to randomization and should be maintained at a constant dosage throughout the study, as possible. (Note: stable dosing of tetrabenazine is allowable.) Any changes to these medications mandated by clinical conditions will be systematically recorded and the subject will be permitted to remain in the trial.
* Able to give informed consent and comply with trial procedures
* Able to take oral medication.
* May be required to identify an informant or caregiver who will be willing and able to supervise the daily dosing of study medications and to maintain control of study medications in the home.
* A designated individual will be identified by the subject to participate in the ongoing consent process should the subject's cognitive capacity to consent become compromised during participation in the study.
* Subjects must have clinical features of HD and a confirmed family history of HD, OR a CAG repeat expansion ≥ 36.
* TFC \> 9.
* Must be ambulatory and not require skilled nursing care.
* Age ≥ 16 years.
* Women must not be able to become pregnant (e.g., post menopausal, surgically sterile or using adequate birth control methods for the duration of the study).
* If psychotropic medications are taken (e.g., anxiolytics, hypnotics, benzodiazepines, antidepressants), they must be at a stable dosage for four weeks prior to randomization and should be maintained at a constant dosage throughout the study, as possible. (Note: stable dosing of tetrabenazine is allowable.) Any changes to these medications mandated by clinical conditions will be systematically recorded and the subject will be permitted to remain in the trial.
* Able to give informed consent and comply with trial procedures
* Able to take oral medication.
* May be required to identify an informant or caregiver who will be willing and able to supervise the daily dosing of study medications and to maintain control of study medications in the home.
* A designated individual will be identified by the subject to participate in the ongoing consent process should the subject's cognitive capacity to consent become compromised during participation in the study.
Exclusion Criteria
* History or known sensitivity of intolerability to CoQ.
* Exposure to any investigational drug within 30 days of the Baseline visit.
* Clinical evidence of unstable medical illness in the investigator's judgment.
* Unstable psychiatric illness defined as psychosis (hallucinations or delusions), untreated major depression or suicidal ideation within 90 days of the Baseline visit.
* Substance (alcohol or drug) abuse within one year of the Baseline visit.
* Women who are pregnant or breastfeeding.
* Use of supplemental coenzyme Q10 within 30 days prior to the Baseline visit
* Clinically serious abnormalities in the screening laboratory studies (Screening creatinine greater than 2.0, alanine aminotransferase (ALT) or total bilirubin greater than 3 times the upper limit of normal, absolute neutrophil count of ≤1000/ul, platelet concentration of \<100,000/ul, hematocrit level of \<33 for female or \<35 for male, or coagulation tests \> 1.5 time upper limit of normal).
* Known allergy to FD\&C yellow #5 or any other ingredient in the study drug (active and placebo)
* Exposure to any investigational drug within 30 days of the Baseline visit.
* Clinical evidence of unstable medical illness in the investigator's judgment.
* Unstable psychiatric illness defined as psychosis (hallucinations or delusions), untreated major depression or suicidal ideation within 90 days of the Baseline visit.
* Substance (alcohol or drug) abuse within one year of the Baseline visit.
* Women who are pregnant or breastfeeding.
* Use of supplemental coenzyme Q10 within 30 days prior to the Baseline visit
* Clinically serious abnormalities in the screening laboratory studies (Screening creatinine greater than 2.0, alanine aminotransferase (ALT) or total bilirubin greater than 3 times the upper limit of normal, absolute neutrophil count of ≤1000/ul, platelet concentration of \<100,000/ul, hematocrit level of \<33 for female or \<35 for male, or coagulation tests \> 1.5 time upper limit of normal).
* Known allergy to FD\&C yellow #5 or any other ingredient in the study drug (active and placebo)
Minimum Eligible Age
16 Years
Eligible Sex
ALL
Accepts Healthy Volunteers
No
Sponsors
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National Institute of Neurological Disorders and Stroke (NINDS)
NIH
Sponsor Role
collaborator
University of Rochester
OTHER
Sponsor Role
collaborator
Massachusetts General Hospital
OTHER
Sponsor Role
lead
Responsible Party
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Merit E. Cudkowicz, MD
Julieanne Dorn Professor of Neurology
Responsibility Role
PRINCIPAL_INVESTIGATOR
Principal Investigators
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Merit Cudkowicz, MD MSc
Role: PRINCIPAL_INVESTIGATOR
Massachusetts General Hospital
Michael McDermott, PhD
Role: PRINCIPAL_INVESTIGATOR
University of Rochester, Biostatistics
Karl Kieburtz, MD MPH
Role: PRINCIPAL_INVESTIGATOR
Director, Clinical Trials Coordination Center, University of Rochester
Locations
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University of Alabama At Birmingham, Pediatric Neurology Childrens, Harbor Bldg Suite 314, 1600 7Th Avenue South
Birmingham, Alabama, United States
Site Status
Mayo Clinic Arizona, 13400 East Shea Boulevard, Csu-Cp21B
Scottsdale, Arizona, United States
Site Status
WASHINGTON REGIONAL MEDICAL CENTER, 3215 N. North Hills Blvd
Fayetteville, Arkansas, United States
Site Status
University of California Irvine, Department of Neurology, 100 Irvine Hall
Irvine, California, United States
Site Status
University of California Davis, Medical Center Dept of Neurology, Acc Building Suite 3700, 4860 Y Street
Sacramento, California, United States
Site Status
Colorado Neurological Institute, Movement Disorders Center, 701 East Hampden Avenue Suite 510
Littleton, Colorado, United States
Site Status
University of Florida Center for Movement Disorders and Neurorestoration, 3450 Hull Road, 4th Floor
Gainesville, Florida, United States
Site Status
UNIVERSITY OF MIAMI, 1150 NW 14th STREET, #401
Miami, Florida, United States
Site Status
University of South Florida, College of Medicine Dept of Neurology, 12901 Bruce B Downs Blvd Mdc-55
Tampa, Florida, United States
Site Status
Emory University, Wesley Woods Center, 1841 Clifton Road NE Room 314
Atlanta, Georgia, United States
Site Status
Idaho Elks Rehabilitation Hospital, 600 North Robbins Road
Boise, Idaho, United States
Site Status
Rush University Medical Center, Department of Neurological Sciences, 1725 West Harrison Suite 755
Chicago, Illinois, United States
Site Status
Indiana University School of Medicine, Outpatient Clinical Research Facility, 535 Barnhill Drive Room #150
Indianapolis, Indiana, United States
Site Status
University of Iowa Hospital and Clinics, 200 Hawkins Road, Room W263 General Hospital
Iowa City, Iowa, United States
Site Status
University of Kansas Medical Center, Department of Neurology, 3599 Rainbow Blvd Mail Stop 2012
Kansas City, Kansas, United States
Site Status
Hereditary Neurological Disease Centre (Hndc),3223 N. Webb, Suite 4
Wichita, Kansas, United States
Site Status
University of Maryland School of Medicine, 22 South Greene Street, N4 W49-B
Baltimore, Maryland, United States
Site Status
Johns Hopkins University, 600 North Wolfe Street, Meyer 2-181
Baltimore, Maryland, United States
Site Status
Boston University School of Medicine, Department of Neurology, 715 Albany Street C329
Boston, Massachusetts, United States
Site Status
Massachusetts General Hospital, 149 13Th Street Suite 2241
Charlestown, Massachusetts, United States
Site Status
University of Michigan, 1500 E Medical Center Drive, B1 H202 Nuclear Medicine
Ann Arbor, Michigan, United States
Site Status
Struthers Parkinson'S Center, 6701 Country Club Drive
Golden Valley, Minnesota, United States
Site Status
Washington University School of Medicine, Box 8111, 660 South Euclid
St Louis, Missouri, United States
Site Status
University of Las Vegas School of Medicine, 1707 W. Charleston Blvd, Suite 220
Las Vegas, Nevada, United States
Site Status
Cooper University Hospital
Camden, New Jersey, United States
Site Status
Nj Neuroscience Institute, Jfk Medical Center, 65 James Street
Edison, New Jersey, United States
Site Status
Albany Medical College, Parkinson'S Disease & Movement Disorders Ctr
Albany, New York, United States
Site Status
North Shore-Lij Health System, 350 Community Drive Room 110, Research Institute
Manhasset, New York, United States
Site Status
Columbia University, Sergievsky Center P&S Box 16, 630 West 168Th Street
New York, New York, United States
Site Status
University of Rochester, Department of Neurology, 919 Westfall Road Building C Suite 220
Rochester, New York, United States
Site Status
Duke University, 932 Morreene Road #213
Durham, North Carolina, United States
Site Status
Wake Forest University, Baptist Med Center, Department of Neurology, Medical Center Boulevard
Winston-Salem, North Carolina, United States
Site Status
University of Cincinnati/Cincinnati Children'S Hospital, 222 Piedmont Avenue, Suite 3200
Cincinnati, Ohio, United States
Site Status
OHIO STATE UNIVERSITY , 2006 Kenny Road
Columbus, Ohio, United States
Site Status
ST. LUKE'S HOSPITAL, 240 Centronia Road
Allentown, Pennsylvania, United States
Site Status
University of Pennsylvania, Pennsylvania Hospital Department of Neurology , 330 South 9Th Street
Philadelphia, Pennsylvania, United States
Site Status
University of Pittsburgh Kaufmann Medical Building, 3471 Fifth Avunue, Suite 811
Pittsburgh, Pennsylvania, United States
Site Status
BUTLER HOSPTIAL MOVEMENT DISORDER PROGRAM, 345 Blackstone Boulevard
Providence, Rhode Island, United States
Site Status
The University of Tennesee Health Science Cen, 855 Monroe Avenue, Department of Neurology, Room 415 Link Bldg
Memphis, Tennessee, United States
Site Status
UN oF TEXAS SOUTHWESTERN MED CENTER DALLAS, 5323 HARRY HINES BOULEVARD H1.108
Dallas, Texas, United States
Site Status
Baylor College of Medicine, 6550 Fannin Suite 1801
Houston, Texas, United States
Site Status
Westmead Hospital, Department of Neurology Level 1, Po Box 533
Wentworthville, New South Wales, Australia
Site Status
University of Calgary, Heritage Medical Research Clinic, Trw Bldg 5 Floor, 3280 Hospital Dri. NW
Calgary, Alberta, Canada
Site Status
University of Alberta, Glenrose Rehab Hosp, Movement Disorder Clinic , Rm 0601 Gleneast 10230 - 111 Avenue
Edmonton, Alberta, Canada
Site Status
Department of Medical Genetics, Ubc Hospital, Room S179-2211 Westbrook Mall
Vancouver, British Columbia, Canada
Site Status
London Health Sciences Centre, University Hospital, 339 Windermere Road
London, Ontario, Canada
Site Status
Centre For Movement Disorders, 2780 Bur Oak Avenue
Markham, Ontario, Canada
Site Status
NORTH YORK GENERAL HOSPITAL (2), 4001 Leslie Street
Toronto, Ontario, Canada
Site Status
North York General Hospital, 4001 Leslie Street
Toronto, Ontario, Canada
Site Status
Countries
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United States
Australia
Canada
References
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Myers RH, Sax DS, Koroshetz WJ, Mastromauro C, Cupples LA, Kiely DK, Pettengill FK, Bird ED. Factors associated with slow progression in Huntington's disease. Arch Neurol. 1991 Aug;48(8):800-4. doi: 10.1001/archneur.1991.00530200036015.
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Cha JH. Transcriptional dysregulation in Huntington's disease. Trends Neurosci. 2000 Sep;23(9):387-92. doi: 10.1016/s0166-2236(00)01609-x.
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Chen M, Ona VO, Li M, Ferrante RJ, Fink KB, Zhu S, Bian J, Guo L, Farrell LA, Hersch SM, Hobbs W, Vonsattel JP, Cha JH, Friedlander RM. Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease. Nat Med. 2000 Jul;6(7):797-801. doi: 10.1038/77528.
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Beal MF, Hyman BT, Koroshetz W. Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases? Trends Neurosci. 1993 Apr;16(4):125-31. doi: 10.1016/0166-2236(93)90117-5.
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Jenkins BG, Koroshetz WJ, Beal MF, Rosen BR. Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy. Neurology. 1993 Dec;43(12):2689-95. doi: 10.1212/wnl.43.12.2689.
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Pepping J. Coenzyme Q10. Am J Health Syst Pharm. 1999 Mar 15;56(6):519-21. doi: 10.1093/ajhp/56.6.519. No abstract available.
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Musumeci O, Naini A, Slonim AE, Skavin N, Hadjigeorgiou GL, Krawiecki N, Weissman BM, Tsao CY, Mendell JR, Shanske S, De Vivo DC, Hirano M, DiMauro S. Familial cerebellar ataxia with muscle coenzyme Q10 deficiency. Neurology. 2001 Apr 10;56(7):849-55. doi: 10.1212/wnl.56.7.849.
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McGarry A, Auinger P, Kieburtz KD, Bredlau AL, Hersch SM, Rosas HD. Suicidality Risk Factors Across the CARE-HD, 2CARE, and CREST-E Clinical Trials in Huntington Disease. Neurol Clin Pract. 2022 Apr;12(2):131-138. doi: 10.1212/CPJ.0000000000001161.
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McGarry A, McDermott MP, Kieburtz K, Fung WLA, McCusker E, Peng J, de Blieck EA, Cudkowicz M; Huntington Study Group 2CARE Investigators and Coordinators. Risk factors for suicidality in Huntington disease: An analysis of the 2CARE clinical trial. Neurology. 2019 Apr 2;92(14):e1643-e1651. doi: 10.1212/WNL.0000000000007244. Epub 2019 Mar 8.
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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. A randomized, double-blind, placebo-controlled trial of coenzyme Q10 in Huntington disease. Neurology. 2017 Jan 10;88(2):152-159. doi: 10.1212/WNL.0000000000003478. Epub 2016 Dec 2.
Reference Type
DERIVED
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Review additional registry numbers or institutional identifiers associated with this trial.
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