RESULTS We found that none of the 19 instruments have sufficiently comprehensive reporting of measurement or feasibility performance as would be required by regulatory authorities .
INTRODUCTION In an accompanying study we reported on the content of several activity rating scales that have been used for muscle disease .
To further aid in achieving consensus we conducted a systematic review to assess the quality of the 19 activity rating scales  design ed specifically for muscle disease .
A randomized trial of coenzyme Q10 in mitochondrial disorders.
Case reports and open-label studies suggest that coenzyme Q(10 ) ( CoQ(10 ) ) treatment may have beneficial effects in mitochondrial disease patients ; however , controlled trials are warranted to clinical ly prove its effectiveness . Thirty patients with mitochondrial cytopathy received 1200 mg/day CoQ(10 ) for 60 days in a r and omized , double-blind , cross-over trial . Blood lactate , urinary markers of oxidative stress , body composition , activities of daily living , quality of life , forearm h and grip strength and oxygen desaturation , cycle exercise cardiorespiratory variables , and brain metabolites were measured . CoQ(10 ) treatment attenuated the rise in lactate after cycle ergometry , increased ( ∽1.93 ml ) VO(2)/kg lean mass after 5 minutes of cycling ( P < 0.005 ) , and decreased gray matter choline-containing compounds ( P < 0.05 ) . Sixty days of moderate- to high-dose CoQ(10 ) treatment had minor effects on cycle exercise aerobic capacity and post-exercise lactate but did not affect other clinical ly relevant variables such as strength or resting lactate
RESULTS We found that none of the 19 instruments have sufficiently comprehensive reporting of measurement or feasibility performance as would be required by regulatory authorities .
INTRODUCTION In an accompanying study we reported on the content of several activity rating scales that have been used for muscle disease .
To further aid in achieving consensus we conducted a systematic review to assess the quality of the 19 activity rating scales  design ed specifically for muscle disease .