Coenzyme Q10 and spinocerebellar ataxias

• Type of Material: Article
• Authors: Raymond Y. Lo, Tzu Chi University; Karla P. Figueroa, University of Utah; Stefan M. Pulst, University of Utah; Chi-Ying Lin, Columbia University; Susan Perlman, University of California Los Angeles; George Wilmot, Emory University; Christopher Gomez, University of Chicago; Jeremy Schmahmann, Harvard University; Henry Paulson, University of Michigan; Vikram G. Shakkottai, University of Michigan; Sarah Ying, Johns Hopkins University; Theresa Zesiewicz, University of South Florida; Khalaf Bushara, University of Minnesota; Michael Geschwind, University of California San Francisco; Guangbin Xia, University of Florida; S.H. Subramony, University of Florida; Tetsuo Ashizawa, University of Florida; Sheng-Han Sheng-Han Kuo, Columbia University
• Language: English
• Date: 2015-02
• Publisher: Wiley
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2014 International Parkinson and Movement Disorder Society
• Final Published Version (URL): http://dx.doi.org/10.1002/mds.26088
• Title of Journal or Parent Work: Movement Disorders
• ISSN: 0885-3185
• Volume: 30
• Issue: 2
• Start Page: 214
• End Page: 220
• Grant/Funding Information: This work was supported by the American Academy of Neurology Clinical Research Training Fellowship, Parkinson Disease Foundation, the National Institute of Neurological Disorders and Stroke (K08 NS083738), the Louis V. Gerstner Jr. Scholarship, the American Parkinson's Disease Association, and the Rare Disease Clinical Research Network (RDCRN) (RC1NS068897).
• Keywords: statins; spinocerebellar ataxias; coenzyme Q10; neurodegeneration
• Research Categories: Biology, Neuroscience; Health Sciences, Pharmacology; Health Sciences, General

Abstract

The aim of this study was to investigate the association between drug exposure and disease severity in SCA types 1, 2, 3 and 6. The Clinical Research Consortium for Spinocerebellar Ataxias (CRC-SCA) enrolled 319 participants with SCA1, 2, 3, and 6 from 12 medical centers in the United States and repeatedly measured clinical severity by the Scale for Assessment and Rating of Ataxia (SARA), the Unified Huntington's Disease Rating Scale part IV (UHDRS-IV), and the 9-item Patient Health Questionnaire during July 2009 to May 2012. We employed generalized estimating equations in regression models to study the longitudinal effects of coenzyme Q10 (CoQ10), statin, and vitamin E on clinical severity of ataxia after adjusting for age, sex, and pathological CAG repeat number. Cross-sectionally, exposure to CoQ10 was associated with lower SARA and higher UHDRS-IV scores in SCA1 and 3. No association was found between statins, vitamin E, and clinical outcome. Longitudinally, CoQ10, statins, and vitamin E did not change the rates of clinical deterioration indexed by SARA and UHDRS-IV scores within 2 years. CoQ10 is associated with better clinical outcome in SCA1 and 3. These drug exposures did not appear to influence clinical progression within 2 years. Further studies are warranted to confirm the association.