Friedreich’s ataxia is one of several different types of ataxia with different causes and prognoses. Friedreich’s ataxia is characterized by muscle weakness, loss of coordination, impairment of vision and hearing, slurred speech and curvature of the spine (scoliosis). Frequently, it results in the development of diabetes and/or heart failure. There is no cure; however, Coenzyme Q10 supplements can help to ameliorate the symptoms because of Coenzyme Q10’s role in cellular energy production and in antioxidant defense against oxidative damage to cells.

Considerably reduced concentrations of Coenzyme Q10 in the skeletal muscle – up to 75% reduced levels – are associated with a diagnosis of ataxia.  Coenzyme Q10 supplementation has proven beneficial in the treatment of both early and later stages of early-onset ataxia as well as in the treatment of adult-onset ataxia [Mantle & Hargreaves 2018].

Ataxia and Coenzyme Q10

Ataxia is the medical term for the loss of control of bodily movement, characterized by difficulty in walking and loss of balance.  Ataxia can also affect eye movement, speech, and ability to swallow.

The following symptoms are typical of ataxia [Mantle & Hargreaves 2018]:

  • poorly coordinated gait
  • poorly coordinated hand/finger movements
  • unclear articulation of sounds
  • repetitive uncontrolled eye movements

Ataxia is a rare disease, resulting from a number of different causes [Mantle & Hargreaves 2018]:

  • neurological causes such as stroke or MS
  • health-related causes such as heart disease or renal failure
  • deficiency causes such as Coenzyme Q10 deficiency
  • medication-related causes such as statin-induced ataxia

Ataxia and Coenzyme Q10 and heart dysfunction

Above and beyond the skeletal muscle dysfunction, spinocerebellar ataxia and Friedreich’s ataxia can be implicated in the development of heart muscle dysfunction.  The heart muscle dysfunction can be a significant cause of disability and death in ataxia patients.

Coenzyme Q10 supplementation of ataxia patients is important:

Coenzyme Q10 and good heart health

In randomized controlled trials, Coenzyme Q10 supplementation has been shown to contribute to better heart health:

  • The beneficial effect of adjuvant Coenzyme Q10 treatment of chronic heart failure patients was demonstrated in the Q-Symbio study.  Daily supplementation with 300 milligrams of Coenzyme Q10 for two years significantly improved the patients’ symptoms and survival compared to placebo supplementation [Mortensen 2014].
  • Daily supplementation with 200 milligrams of Coenzyme Q10 in combination with 200 micrograms of high-selenium yeast for four years in the KiSel-10 study was associated with significantly reduced death from heart disease and improved quality of life in healthy elderly study participants compared to placebo supplementation [Alehagen, 2015; Johansson 2015].

Coenzyme Q10 dosages in the treatment of ataxia

A 2010 study reported significant improvement in ataxia patients with known Coenzyme Q10 deficiency when the patients were supplemented with Coenzyme Q10 at a rate of 30 milligrams per kilogram of body weight per day over a two-year period [Pineda 2010].

Coenzyme Q10 supplementation in daily dosages ranging from 300 – 3000 milligrams per day was associated with significant improvement in ataxia symptoms in two different studies [Musumeci 2001; Lamperti 2003].

Six cerebellar ataxia patients aged 16 – 25 years with muscle Coenzyme Q10 deficiencies of 26 to 35% of normal levels responded to Coenzyme Q10 supplementation [Musumeci 2001]:

  • increased strength
  • improved muscle coordination
  • less frequent seizures

Thirteen childhood-onset ataxia patients with decreased skeletal muscle levels of Coenzyme Q10 and with cerebellar atrophy responded to daily Coenzyme Q10 supplementation [Lamperti 2003].

Coenzyme Q10 and Friedreich’s ataxia

Friedreich’s ataxia is an inherited disease that leads to progressively worse damage of the nervous system.  It affects coordination, energy levels, and vision, hearing, and speech.  It can lead to the development of scoliosis and insulin-dependent diabetes.  A majority of patients with Friedreich’s ataxia eventually develop thickened heart muscles that make the pumping of blood more difficult.  This condition — called hypertrophic cardiomyopathy — often leads to heart failure and death [Mantle & Hargreaves 2018].

In a 2008 study, 50 Friedreich’s ataxia patients with low International Co-operative Ataxia Ratings Scale (ICARS) scores were randomly divided into high or low dose Coenzyme Q10 and vitamin E treatment groups.

Over a two-year period, high-dose patients took 600 milligrams of Coenzyme Q10 and 2100 international units of Vitamin E per day; low-dose patients took 30 milligrams of Coenzyme Q10 and 24 international units of Vitamin E per day.

At baseline in the study, a high proportion of the Friedreich’s ataxia patients had decreased serum Coenzyme Q10 concentrations.  The low-dose and high-dose Coenzyme Q10 and vitamin E therapies were equally effective in improving the patients’ ICARS scores [Cooper 2008].

Coenzyme Q10 and spinocerebellar ataxia

Spinocerebellar ataxias are rare neurodegenerative disorders characterized by increased oxidative stress, by abnormalities in the antioxidant system, and by changes in the mitochondrial process of energy production.

  • Treatment with Coenzyme Q10 can give improved clinical outcomes in patients with spinocerebellar ataxia types 1 and 3 [Lo 2015].
  • Treatment with Coenzyme Q10 can partially reverse the changes in antioxidant status and in bio-energetics [Cornelius 2017].

Coenzyme Q10 and statin-induced ataxias

No article about Coenzyme Q10 and ataxia would be complete without mention of the drug-induced ataxias (Teive 2016).  These are the ataxias that result from Coenzyme Q10 deficiency in patients who have been prescribed a statin medication.  Statins inhibit not only the body’s production
of cholesterol, they also inhibit the body’s production of Coenzyme Q10 because the endogenous Coenzyme Q10 is synthesized on the same biochemical pathway as cholesterol [Mantle & Hargreaves 2018).

Safety of Coenzyme Q10

Dr. Mantle and Dr. Hargreaves [2018] report that there is not yet a consensus on the optimal dosage of Coenzyme Q10 for ataxia patients.  Dosages in the range of 300 to 3000 milligrams per day have been used and have been well-tolerated [Mantle & Hargreaves 2018].

Absorption and steady-state bio-availability of Coenzyme Q10 is generally low because of the large size and lipid solubility of Coenzyme Q10 molecules.  Absorption varies according to the quality and the formulation of the supplement [Mantle & Hargreaves 2018].

Here, again, the evidence of a significant effect on heart health is the best assurance of the proper formulation of the Coenzyme Q10 supplement.  The same supplement was used in the following randomized controlled trials:

  • Q-Symbio study [Mortensen 2014]: improved symptoms and survival and better quality of life for chronic heart failure patients
  • KiSel-10 study [Alehagen 2105]: reduced risk of death from heart disease, better maintained heart function, better quality of life in elderly healthy citizens
  • Gulf War Illness study [Golomb 2013]: improved physical function, improved symptoms, and improved General Self-rated Health scores for veterans diagnosed with Gulf War illness

Sources

Alehagen, U., Aaseth, J., & Johansson, P. (2015). Reduced Cardiovascular Mortality 10 Years after Supplementation with Selenium and Coenzyme Q10 for Four Years: Follow-Up Results of a Prospective Randomized Double-Blind Placebo-Controlled Trial in Elderly Citizens. Plos One, 10(12), e0141641. doi:10.1371/journal.pone.0141641

Cooper, J. M., Korlipara, L. P., Hart, P. E., Bradley, J. L., & Schapira, A. V. (2008). Coenzyme Q10 and vitamin E deficiency in Friedreich’s ataxia: predictor of efficacy of vitamin E and coenzyme Q10 therapy. European Journal Of Neurology, 15(12), 1371-1379. doi:10.1111/j.1468-1331.2008.02318.x

Cornelius, N., Wardman, J. H., Hargreaves, I. P., Neergheen, V., Bie, A. S., Tümer, Z., & … Nielsen, T. T. (2017). Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts: Effect of coenzyme Q10 supplementation on these parameters. Mitochondrion, 34103-114. doi:10.1016/j.mito.2017.03.001

Golomb, B. A., Allison, M., Koperski, S., Koslik, H. J., Devaraj, S., & Ritchie, J. B. (2014). Coenzyme Q10 benefits symptoms in Gulf War veterans: results of a randomized double-blind study. Neural Computation, 26(11), 2594-2651. doi:10.1162/NECO_a_00659

Johansson, P., Dahlström, Ö., Dahlström, U., & Alehagen, U. (2015). Improved Health-Related Quality of Life, and More Days out of Hospital with Supplementation with Selenium and Coenzyme Q10 Combined. Results from a Double Blind, Placebo-Controlled Prospective Study. The Journal of Nutrition, Health & Aging, 19(9), 870-877.
doi:10.1007/s12603-015-0509-9

Lamperti, C., Naini, A. & Hirano, M. (2003). Cerebellar ataxia and Coenzyme Q10 deficiency. Neurology, 60(7):1206–8

Lo, R. Y., Figueroa, K. P., Pulst, S. M., Lin, C., Perlman, S., Wilmot, G., & … Kuo, S. (2015). Coenzyme Q10 and spinocerebellar ataxias. Movement Disorders: Official Journal of The Movement Disorder Society, 30(2), 214-220. doi:10.1002/mds.26088

Mantle, D. & Hargreaves, I. P. (2018). Ataxia and Coenzyme Q10: an overview.  British Journal of Neuroscience Nursing, 14,3, 2-7.

Mortensen, S.A., Rosenfeldt, F., Kumar, A. & Littarru, G. P.  (2014). The effect of Coenzyme Q10 on morbidity and mortality in chronic heart failure: results from Q-SYMBIO: a randomised double-blind trial. JACC Heart Failure, 2(6):641–9

Musumeci, O., Naini, A. & Slonim, A. E. (2001).  Familial cerebellar ataxia with muscle Coenzyme Q10 deficiency. Neurology. 2001;56(7):849–55

Pineda, M., Montero, R., Aracil, A., Navas, P. & Artuch, R. (2010).  Coenzyme Q10-responsive ataxia: 2-year-treatment follow-up. Mov Disord., 25(9):1262–8

Teive, H.A., Moro, A., Moscovich, M., Arruda, W.O. & Munhoz, K.P. Statin-associated cerebellar ataxia. A Brazilian case series. Parkinsonism Relat Disord. 2016;25:97–9

The information presented in this review article is not intended as medical advice and should not be used as such.

 

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