Abstract

Mitochondrial complex I appears to be dysfunctional in Progressive Supranuclear Palsy (PSP). Coenzyme Q10 (CoQ10) is a physiological co-factor of complex I. Therefore, we evaluated the short-term effects of CoQ10 in PSP. We performed a double-blind, randomized, placebo-controlled, phase II trial including twenty-one clinically probable PSP patients (stage II, III) to receive a liquid nano-dispersion of CoQ10 (5mg/kg/day) or matching placebo. Over a six-week period we determined the change in CoQ10 serum concentration, cerebral energy metabolites (by 31P- and 1H-magnetic resonance spectroscopy), motor and neuropsychological dysfunction (PSP rating scale, UPDRS III, Hoehn & Yahr stage, frontal assessment battery, mini mental status examination, Montgomery Agsberg depression scale).

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