Coenzyme Q10 and vitamin E deficiency in Friedreich's ataxia: predictor of efficacy of vitamin E and coenzyme Q10 therapy
JM Cooper, LVP Korlipara, PE Hart… - European journal of …, 2008 - Wiley Online Library
JM Cooper, LVP Korlipara, PE Hart, JL Bradley, AHV Schapira
European journal of neurology, 2008•Wiley Online LibraryBackground and purpose: A pilot study of high dose coenzyme Q10 (CoQ10)/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 CoQ10/vitamin E groups. The change in
International Co‐operative Ataxia Ratings Scale (ICARS) was assessed over 2 years as the …
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 CoQ10/vitamin E groups. The change in
International Co‐operative Ataxia Ratings Scale (ICARS) was assessed over 2 years as the …
Background and purpose: A pilot study of high dose coenzyme Q10 (CoQ10)/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 CoQ10/ 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 CoQ10 and vitamin E levels were significantly decreased in the FRDA patients (P < 0.001). During the trial CoQ10 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 CoQ10 levels.
Conclusions: A high proportion of FRDA patients have a decreased serum CoQ10 level which was the best predictor of a positive clinical response to CoQ10/vitamin E therapy. Low and high dose CoQ10/vitamin E therapies were equally effective in improving ICARS scores.
Wiley Online Library