A controlled trial of idebenone in Huntington's disease
Abstract
One hundred patients with clinically diagnosed Huntington's disease (HD) were randomized to either idebenone, an antioxidant and enhancer of oxidative metabolism, or placebo, in a 1‐year, double‐blind, parallel‐group study aimed at slowing the rate of progression of the disease. Ninety‐one patients completed the study. There were no significant differences between groups on the primary outcome measures of the Huntington's Disease Activities of Daily Living Scale (ADL—an index of functional status) and the Quantified Neurologic Examination (QNE). Sample size calculations based on progression of the ADL and QNE in this study group revealed that a larger study group is necessary to detect any differences less than an almost complete halting of the disease. This argues for multicenter efforts for future therapeutic trials in HD.
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There was no significant improvement in a small trial of idebenone in HD.
“There was no significant improvement in a small trial in HD171.”
From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [171]
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- Authors
- Ranen, N. G., Peyser, C. E., Coyle, J. T., Bylsma, F. W., Sherr, M., Day, L., Folstein, M. F., Brandt, J., Ross, C. A., & Folstein, S. E.
- Affiliations
- Johns Hopkins University (US)
- VA Palo Alto Health Care System (US)
- Tufts University (US)
- Corresponding author
- Ranen, Neal G.
- Venue
- Movement Disorders
- Publisher
- Wiley
- Published
- 1996-09
- Volume
- 11
- Issue
- 5
- Pages
- 549-554
- ISSN
- 0885-3185 (ISSN-L)
- 1531-8257
- OpenAlex
- W2036012667
- Cited by
- 97
- References
- 37
- Citations by year
- 2026: 2 · 2025: 5 · 2024: 2 · 2023: 1 · 2022: 5 · 2021: 3 · 2020: 3 · 2019: 6 · 2018: 3 · 2017: 3 · 2016: 1 · 2015: 1 · 2014: 3 · 2013: 2 · 2012: 3
- FWCI
- 1.21
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Movement Disorders (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institute of Neurological Disorders and Stroke (P01NS016375)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Health Sciences › Medicine › Neurology › Parkinson's Disease Mechanisms and Treatments
- Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
- Keywords
- idebenone, Huntington's disease, disease progression, antioxidant therapy, oxidative metabolism, functional status, sample size determination
- MeSH terms
- Activities of Daily Living, Adult, Antioxidants, Double-Blind Method, Female, Humans, Huntington Disease, Longitudinal Studies, Male, Middle Aged, Placebos, Ubiquinone, Benzoquinones, Treatment Outcome, Receptors, Glutamate
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