The combination of vitamin E and Co-enzyme Q10

We thought it might be helpful to bring you up to date on a treatment currently being evaluated for use in Friedreich's ataxia.

The University Department of Clinical Neurosciences (Centre for Neurodegenerative Diseases, University College London), has received substantial funding from the Friedreich's Ataxia Group and the National Lottery Fund to continue its research on drugs for the treatment of Friedreich's ataxia. Following identification of a deficit in the mitochondrial energy supply to heart cells (Human Molecular Genetics, 2000 in press), evidence of free radical damage and iron accumulation has been found in heart muscle from patients with Friedreich's ataxia. The Unit has pioneered the use of the combination of vitamin E and Co-enzyme Q in an attempt to reverse the biochemical abnormalities found in Friedreich's ataxia. The group was the first to identify a biochemical marker for the effects of frataxin deficiency (the cause of Friedreich's ataxia). In The Proceedings of the National Academy of Sciences, USA (1999;9611492-11495) we describe the use of 31phosphorus magnetic resonance spectroscopy to identify the mitochondrial defect in the leg muscle of Friedreich's ataxia patients. Data has also been presented which demonstrates a similar defect in heart muscle (Annals of Neurology, 1998;44(no 3):8).

Vitamin E and Co-enzyme Q are readily available, cheap and well tolerated. They are highly effective antioxidants particularly when used in combination.

Vitamin E and Co-enzyme Q have been used by the Royal Free group in patients with Friedreich's ataxia. Response to treatment has been monitored by 31phosphorus magnetic resonance spectroscopy to determine whether these drugs can reverse the biochemical defect found in patients. A significant number of patients have already been studied and the results are very promising. These drugs are capable of improving the biochemical defect in both heart and skeletal muscle in Friedreich's ataxia and suggest that vitamin E and Co-enzyme Q may be able to modify the course of the disease.

Whether vitamin E and Co-enzyme Q will slow down or even prevent progression of the neurological problems of Friedreich's ataxia is now the subject of a large trial in the UK.

As always, we would advocate to patients and their physicians that the results of proper controlled studies should be available before widespread use of a drug. The benefit of vitamin E and Co-enzyme Q is, as stated above, not only related to their potency but also their cheapness, easy access and ease of use.

We will keep you updated with further progress over the next few months.

With best wishes,

Tony Schapira MD DSc FRCP FMedSci Mark Cooper PhD

Professor of Neurology and Director Senior Lecturer


R Lodi, JM Cooper, JL Bradley, M Manners, P Styles, DJ Taylor, AHV Schapira. Deficit of in vivo mitochondrial ATP production in patients with Friedreich's ataxia. Proc Natl Acad Sci USA 1999;9611492-11495.