title: Coenzyme Q10 and spinocerebellar ataxias.
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.
authors.affiliation: Department of Neurology, Buddhist Tzu Chi General Hospital and Tzu Chi University, Hualien, Taiwan.
authors.forename: Raymond Y
authors.initials: RY
authors.lastname: Lo
authors.forename: Karla P
authors.initials: KP
authors.lastname: Figueroa
authors.forename: Stefan M
authors.initials: SM
authors.lastname: Pulst
authors.forename: Chi-Ying
authors.initials: CY
authors.lastname: Lin
authors.forename: Susan
authors.initials: S
authors.lastname: Perlman
authors.forename: George
authors.initials: G
authors.lastname: Wilmot
authors.forename: Christopher
authors.initials: C
authors.lastname: Gomez
authors.forename: Jeremy
authors.initials: J
authors.lastname: Schmahmann
authors.forename: Henry
authors.initials: H
authors.lastname: Paulson
authors.forename: Vikram G
authors.initials: VG
authors.lastname: Shakkottai
authors.forename: Sarah
authors.initials: S
authors.lastname: Ying
authors.forename: Theresa
authors.initials: T
authors.lastname: Zesiewicz
authors.forename: Khalaf
authors.initials: K
authors.lastname: Bushara
authors.forename: Michael
authors.initials: M
authors.lastname: Geschwind
authors.forename: Guangbin
authors.initials: G
authors.lastname: Xia
authors.forename: S H
authors.initials: SH
authors.lastname: Subramony
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Ashizawa
authors.forename: Sheng-Han
authors.initials: SH
authors.lastname: Kuo
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D018450:Disease Progression; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000188:drug therapy; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index; D020754:Spinocerebellar Ataxias / Q000175:diagnosis / Q000188:drug therapy*; D011795:Surveys and Questionnaires; D016896:Treatment Outcome; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000627:therapeutic use
chemical_list: D014451:Ubiquinone; C024989:coenzyme Q10
keywords: coenzyme Q10; neurodegeneration; spinocerebellar ataxias; statins