Ataxia and coenzyme Q10: an overview

Abstract

This article focuses on the hereditary ataxias, resulting from defects in specific genes, which include the spinocerebellar ataxias (SCAs), Friedreich's ataxia, and ataxia resulting from coenzyme Q10 (CoQ10) deficiency. CoQ10 is a vitamin-like substance that plays a key role in the biochemical process within mitochondria, supplying all cells with the energy required for normal functioning; CoQ10 is also important as an antioxidant, protecting cells (and particularly mitochondria) from free radical-induced oxidative damage. Oxidative stress, mitochondrial dysfunction and cellular energy depletion have been implicated in the pathogenesis of the hereditary ataxias; this provides a rationale for dietary supplementation with CoQ10 in these disorders. The authors have therefore reviewed clinical evidence for the potential benefit of oral supplementation with CoQ10 in the hereditary ataxias. Some categories of hereditary ataxia patients have demonstrated remarkable clinical improvement following CoQ10 supplementation, especially when administered at an early stage of disease. In particular, a number of studies have demonstrated significant symptomatic benefit in patients with ataxia resulting from primary CoQ10 deficiency, following oral supplementation with CoQ10. Although there is no consensus as to the dosage of CoQ10 to be used in ataxia management, clinical studies have typically used dosages in the range 300 to 3000 mg/day. With regard to safety, CoQ10 is generally well tolerated, with no significant adverse effects reported in long-term use.