title: Increased oxidative stress in patients with amyotrophic lateral sclerosis and the effect of edaravone administration.
abstract: OBJECTIVE
  Compared to age-matched healthy controls (n = 55), patients with amyotrophic lateral sclerosis (ALS) (n = 26) showed increased oxidative stress as indicated by a significantly increased percentage of oxidized coenzyme Q10 (%CoQ10) in total plasma coenzyme Q10, a significantly decreased level of plasma uric acid, and a significantly decreased percentage of polyunsaturated fatty acids in total plasma free fatty acids (FFA). Therefore, the efficacy of edaravone, a radical scavenger, in these ALS patients was examined.
  
  
  CONCLUSIONS
  Among 26 ALS patients, 17 received edaravone (30 mg/day, one to four times a week) for at least 3 months, and 13 continued for 6 months. Changes in revised ALS functional rating scale (ALSFRS-R) were significantly smaller in these patients than in edaravone-untreated ALS patients (n = 19). Edaravone administration significantly reduced excursions of more than one standard deviation from the mean for plasma FFA levels and the contents of palmitoleic and oleic acids, plasma markers of tissue oxidative damage, in the satisfactory progress group (ΔALSFRS-R ≥ 0) as compared to the ingravescent group (ΔALSFRS-R < -5). Edaravone treatment increased plasma uric acid, suggesting that it is an effective scavenger of peroxynitrite. However, edaravone administration did not decrease %CoQ10. Therefore, combined treatment with agents such as coenzyme Q10 may further reduce oxidative stress in ALS patients.
authors.affiliation: a School of Bioscience and Biotechnology , Tokyo University of Technology , 1404-1 Katakura-cho, Hachioji 192-0982 , Japan.
authors.forename: Midori
authors.initials: M
authors.lastname: Nagase
authors.affiliation: a School of Bioscience and Biotechnology , Tokyo University of Technology , 1404-1 Katakura-cho, Hachioji 192-0982 , Japan.
authors.forename: Yorihiro
authors.initials: Y
authors.lastname: Yamamoto
authors.affiliation: a School of Bioscience and Biotechnology , Tokyo University of Technology , 1404-1 Katakura-cho, Hachioji 192-0982 , Japan.
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Miyazaki
authors.affiliation: b Yoshino Neurology Clinic , 3-3-16 Khonodai, Ichikawa 272-0827 , Chiba , Japan.
authors.forename: Hiide
authors.initials: H
authors.lastname: Yoshino
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy* / Q000378:metabolism*; D000983:Antipyrine / Q000031:analogs & derivatives* / Q000627:therapeutic use; D015415:Biomarkers / Q000097:blood; D000077553:Edaravone; D005230:Fatty Acids, Nonesterified / Q000097:blood; D005260:Female; D016166:Free Radical Scavengers / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010084:Oxidation-Reduction / Q000187:drug effects; D018384:Oxidative Stress / Q000187:drug effects; D030421:Peroxynitrous Acid / Q000097:blood; D014451:Ubiquinone / Q000031:analogs & derivatives / Q000097:blood; D014527:Uric Acid / Q000097:blood
chemical_list: D015415:Biomarkers; D005230:Fatty Acids, Nonesterified; D016166:Free Radical Scavengers; D014451:Ubiquinone; D030421:Peroxynitrous Acid; D014527:Uric Acid; C024989:coenzyme Q10; D000077553:Edaravone; D000983:Antipyrine
keywords: Coenzyme Q10 redox balance; Edaravone treatment; Peroxynitrite; Plasma free fatty acid composition; Uric acid