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Coenzyme Q 10 and vitamin E deficiency in Friedreich’s ataxia: predictor of efficacy of vitamin E and coenzyme Q 10 therapy
Author(s) -
Cooper J. M.,
Korlipara L. V. P.,
Hart P. E.,
Bradley J. L.,
Schapira A. H. V.
Publication year - 2008
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/j.1468-1331.2008.02318.x
Subject(s) - medicine , ataxia , coenzyme q10 , gastroenterology , vitamin , randomized controlled trial , vitamin e , endocrinology , physical therapy , pediatrics , antioxidant , biochemistry , chemistry , psychiatry
Background and purpose: A pilot study of high dose coenzyme Q 10 (CoQ 10 )/vitamin E therapy in Friedreich’s ataxia (FRDA) patients resulted in significant clinical improvements in most patients. This study investigated the potential for this treatment to modify clinical progression in FRDA in a randomized double blind trial. Methods: Fifty FRDA patients were randomly divided into high or low dose CoQ 10 / vitamin E groups. The change in International Co‐operative Ataxia Ratings Scale (ICARS) was assessed over 2 years as the primary end‐point. A post hoc analysis was made using cross‐sectional data. Results: At baseline serum CoQ 10 and vitamin E levels were significantly decreased in the FRDA patients ( P < 0.001). During the trial CoQ 10 and vitamin E levels significantly increased in both groups ( P < 0.01). The primary and secondary end‐points were not significantly different between the therapy groups. When compared to cross‐sectional data 49% of all patients demonstrated improved ICARS scores. This responder group had significantly lower baseline serum CoQ 10 levels. Conclusions: A high proportion of FRDA patients have a decreased serum CoQ 10 level which was the best predictor of a positive clinical response to CoQ 10 /vitamin E therapy. Low and high dose CoQ 10 /vitamin E therapies were equally effective in improving ICARS scores.