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Friedreich Ataxia (FA)

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Nutrition

It is important for patients with Friedreich ataxia (FA) to monitor their dietary intake to maintain a healthy and balanced diet. Patients should discuss any changes in their diet with their medical care team, including the primary care physician, neurologist, physical therapist, speech therapist, dietitian, and nutritionist.1-3

A healthy and individualized diet may help reduce symptom severity, achieve a healthy body weight, reduce stress on joints, improve mobility, achieve regular bowel movements, reduce fatigue, increase energy levels, and improve mental health.1,2

Simple Carbohydrate-Restricted Diet

Simple carbohydrates are low in nutrients such as fiber, vitamins, and minerals. They are also easily digestible, which causes blood glucose levels to spike. Moreover, they make a person hungry more quickly, which can lead to overeating and weight gain. Thus, patients with FA may benefit from avoiding simple carbohydrates, such as foods sweetened with high-fructose corn syrup, sugar, or artificial sweeteners like cookies, cakes, candies, pastries, white flour, fruit juices, and cold drinks.1,2 

Patients should instead choose options with complex carbohydrates, such as unsweetened fruits, starchy vegetables, legumes, rice, and pasta, which are high in nutrients as well as fiber. These foods are digested slowly, offer a feeling of fullness, and don’t cause in spikes in blood glucose levels.1,2

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High-Fiber Diet

Patients with FA may also benefit from eating a diet rich in fiber, which can be achieved by increasing the intake of fresh vegetables and fruits, whole grains, and beans. In some cases, patients may need a fiber supplement such as psyllium husk or methylcellulose.1,2

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Supplements

Patients with FA may need a daily multivitamin in addition to a healthy diet to help improve fatigue and general health. In many cases, a specific vitamin or micronutrient supplement may also be needed. The daily recommended dose depends on the individual’s age and sex. Some of the supplements recommended by the National Ataxia Foundation (NAF) for patients with FA are vitamin B12, vitamin C, vitamin D3, vitamin E, coenzyme Q10 (CoQ10), calcium, and magnesium.1,2

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Other Dietary Changes

Increasing the amount of protein and healthy fats may also help patients with FA. Water intake should be 6 to 8 glasses per day. Processed foods and meats with additives or preservatives should be avoided. The number of calories consumed should be balanced by exercise and daily activities to maintain a healthy body weight.1,2

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Food Texture

Patients who have difficulty swallowing should be careful about the texture of foods and avoid foods that may cause coughing or choking such as dry crumbly foods (eg, nuts, corn bread, biscuits), small and easily inhaled foods (eg, sesame seeds, flax seeds), foods that are difficult to chew (eg, steak, apples), and very thin liquids that are hard to swallow (eg, water, juices). Thus, patients with dysphagia should preferably consume soft foods and thickened fluids to ensure safe swallowing.1,2

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Clinical Studies

Nicotinamide, which is a form of vitamin B3 obtained from foods such as fish, poultry, legumes, eggs, and cereal grains, has been shown to have positive effects on FXN expression in preclinical and clinical studies. However, further research is needed to assess its benefits on clinical measures in patients with FA.​4,5

A 12-week open-label study in 24 patients with FA evaluated the effects of low-dose and high-dose resveratrol, which is an antioxidant found in grape skin, blueberries, and peanuts. The results showed improvements in neurologic function in patients who were administered the high dose. However, more research is needed to assess its clinical efficacy in further trials.5,6

Two studies assessed the effect of riboflavin, which is a water-soluble vitamin (vitamin B2) that functions as an antioxidant essential for normal cell function. One of the studies assessed the safety and efficacy of riboflavin in combination with Ferriprox® (deferiprone) and idebenone. Although Ferriprox caused adverse events, the results suggested some uncertain benefit of the triple therapy on the neurological and heart functions of patients with FA.7 Another study evaluated the safety and efficacy of riboflavin in combination with Aranesp® (darbepoetin alfa) and idebenone. This triple therapy was well tolerated and led to statistically insignificant improvements in ataxia during the first six 4-month periods of the study with a small decrease in disease progression during the first 2 years of treatment.8

Coenzyme Q10 is a small molecule with antioxidant properties that transports electrons within the mitochondria. Several studies have been conducted to assess the benefits of CoQ10 as well as its analog idebenone in patients with FA. A study by Lodi et al showed that a combination of CoQ10 and vitamin E significantly improved heart and skeletal muscle energy metabolism in 10 patients with FA after 3 months of treatment.9 Further, a long-term follow-up study over 47 months revealed sustained improvements in cardiac and skeletal muscle bioenergetics.10 However, other studies could not definitively establish the therapeutic benefits of CoQ10 or idebenone in patients with FA.11,12

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References

1. Managing Friedreich’s ataxia. Connect FA. Accessed January 26, 2023.

2. Frequently asked questions about…diet for ataxias. National Ataxia Foundation. Accessed January 26, 2023.

3. Corben LA, Collins V, Milne S, et al; Clinical Management Guidelines Writing Group. Clinical management guidelines for Friedreich ataxia: best practice in rare diseases. Orphanet J Rare Dis. 2022;17(1):415. doi:10.1186/s13023-022-02568-3

4. Libri V, Yandim C, Athanasopoulos S, et al. Epigenetic and neurological effects and safety of high-dose nicotinamide in patients with Friedreich’s ataxia: an exploratory, open-label, dose-escalation study. Lancet. 2014;384(9942):504-513. doi:10.1016/S0140-6736(14)60382-2

5. Gottesfeld JM. Molecular mechanisms and therapeutics for the GAA·TTC expansion disease Friedreich ataxia. Neurotherapeutics. 2019;16(4):1032-1049. doi:10.1007/s13311-019-00764-x.

6. Yiu EM, Tai G, Peverill RE, et al. An open-label trial in Friedreich ataxia suggests clinical benefit with high-dose resveratrol, without effect on frataxin levels. J Neurol. 2015;262(5):1344-1353. doi:10.1007/s00415-015-7719-2

7. Arpa J, Sanz-Gallego I, Rodríguez-de-Rivera FJ, et al. Triple therapy with deferiprone, idebenone and riboflavin in Friedreich’s ataxia – open-label trial. Acta Neurol Scand. 2014;129(1):32-40. doi:10.1111/ane.12141

8. Arpa J, Sanz-Gallego I, Rodríguez-de-Rivera FJ, et al. Triple therapy with darbepoetin alfa, idebenone, and riboflavin in Friedreich’s ataxia: an open-label trial. Cerebellum. 2013;12(5):713-720. doi:10.1007/s12311-013-0482-y

9. Lodi R, Hart PE, Rajagopalan B, et al. Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich’s ataxia. Ann Neurol. 2001;49(5):590-596. doi:10.1002/ana.1001

10. Hart PE, Lodi R, Rajagopalan B, et al. Antioxidant treatment of patients with Friedreich ataxia: four-year follow-up. Arch Neurol. 2005;62(4):621-626. doi:10.1001/archneur.62.4.621

11. Zesiewicz TA, Hancock J, Ghanekar SD, Kuo SH, Dohse CA, Vega J. Emerging therapies in Friedreich’s ataxia. Expert Rev Neurother. 2020;20(12):1215-1228. doi:10.1080/14737175.2020.1821654

12. Bidichandani SI, Delatycki MB. Friedreich ataxia. In: Adam MP, Everman DB, Mirzaa GM, et al, eds. GeneReviews® [Internet]. Seattle, WA: University of Washington, Seattle; 1993-2023. December 18, 1998. Updated June 1, 2017. Accessed January 26, 2023.

Reviewed by Hasna Avcu, MD, on 1/28/2023.

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