Аннотация:Two articles in this issue of Neurology describe the use of coenzyme Q10 in the treatment of neurologic disorders.1,2⇓ Coenzyme Q10 (ubiquinone) is the carrier of electrons from complexes I and II to complex III of the mitochondrial electron transport chain. It can act as either an antioxidant or prooxidant depending on the cell’s redox potential and the relative proportion of the oxidized and reduced forms of coenzyme Q10. Primary coenzyme Q10 deficiency has been associated with at least three rare neurologic syndromes that can involve both muscle and CNS.1 Of potentially greater importance is that mitochondrial dysfunction has been demonstrated to be present in Parkinson’s disease,3,4⇓ Huntington’s disease,5,6⇓ and Friedreich’s ataxia,7 and coenzyme Q10 has been suggested to be a potential treatment in these disorders.8-10⇓⇓
Di Giovanni et al.1 report two brothers who presented in adolescence with myopathy and one with seizures. Muscle biopsies demonstrated ragged red fibers and signs of …
