Insufficient intake of nutrients and energy (malnutrition) and weight loss are common in people with amyotrophic lateral sclerosis (ALS).

Decreased food intake occurs mainly because of symptoms such as difficulty swallowing, as well as issues with hand grip and movement. In addition, digestive symptoms can cause an imbalance between food intake and utilization, resulting in insufficient energy supply.

Malnutrition can worsen muscle weakness and make it difficult for the body to fight infections.

Nutritional therapy with the help of proper diet and nutritional supplements may help prevent or alleviate malnutrition and slow the progression of ALS.

Diet

The nutritional requirements of every patient are different, and it is essential to develop a dietary plan that will suit the patient’s specific needs in consultation with a doctor and trained dietician.

In some cases, a diet high in fat and low in carbohydrate such as the ketogenic diet may be helpful in ALS patients. The ketogenic diet focuses on using a different type of fuel source called ketone bodies that are produced by the liver using fats. When the body is deprived of energy, ketone bodies are utilized as energy stores. Because ALS patients may also experience energy deprivation due to malnutrition, a ketogenic diet may be helpful. A preclinical study in a mouse model of ALS showed that oxidative stress was lowered in animals fed a fat-rich diet; it also improved their chances of survival compared to animals on a calorie-restricted diet.  Another study showed that a ketogenic fat-rich diet improved motor function in ALS mice compared to the standard diet.

A study compared the impact of a high-carbohydrate and high-fat diet on body weight and body mass index (BMI) in ALS patients. After 12 weeks of the diet, both groups attained a stable body weight and an increase in BMI. Although not statistically significant, the patients on the high-fat diet had a better improvement in weight compared with a high-carbohydrate diet. Overall, researchers concluded that a high-calorie diet is suitable for patients with ALS.

A Phase 3 clinical study (NCT00983983) tested the safety, tolerability, and efficacy of three tube feeding formulas, developed by Abbott, that are high in fat (Oxepa), high in calories (Jevity 1.5), or normal diets (Jevity 1.0) in ALS patients with gastrostomy or jejunostomy tube.

The study recruited 20 patients. Eight received Jevity 1.5, six received Oxepa, and six received the control Jevity 1.0. Participants received the diets for four months with a follow-up period of five months. The high-calorie diet (Jevity 1.5) was tolerated best, with fewer side effects compared to the other diets. The impact of the high-fat diet (Oxepa) was not statistically significant compared with the control diet. The team concluded that hypercaloric tube feeding could serve as a potential nutritional intervention for ALS patients.

One patient reported that a paleolithic diet, which is rich in fresh fruits and vegetables, healthy fats, lean proteins, fish, and unprocessed meat, has contributed to meeting his energy requirements and improved his functional ability. A clinical trial (NCT03659422) will assess the safety of a modified paleolithic diet and its effect on weight gain, ALS symptoms, and quality of life. The study aims to enroll 10 ALS patients, ages 18 to 80.

Nutritional supplements

Several vitamins, minerals, natural foods, and other supplements could help address malnutrition in ALS patients and help delay the worsening of symptoms. These are usually available over the counter, but their use is not well-regulated. Therefore, they must be used with caution, and after consulting with a doctor. Every patient’s nutritional needs are different, and discussions with the care team can help determine the appropriate supplements. Some common supplements are detailed below.

Vitamins

Vitamin E: In ALS, oxidative stress caused by the release of hyperactive unstable molecules called free radicals can cause nerve cell damage and hasten disease progression. As an antioxidant, vitamin E may reduce free radical production and prevent oxidative damage.

Vitamin D: Like Vitamin E, vitamin D can also serve as an antioxidant. It also has neuroprotective properties and could improve functional ability in ALS patients. A study showed that vitamin D levels are lower in ALS patients and associated with movement disability, but vitamin D supplements did not slow ALS progression.

Vitamin B12: Vitamin B12 is essential for the proper development and function of the brain and spinal cord. A Phase 2/3 clinical study (NCT00444613) showed that taking vitamin B12 immediately after symptom onset can slow ALS progression and improve prognosis.

Other vitamin supplements include vitamin A, vitamins B1 and B2, and vitamin C. However, sufficient clinical information is not available regarding these supplements’ efficacy in ALS.

Coenzyme Q10

Coenzyme Q10 is a mitochondrial enzyme with antioxidant properties that may provide neuroprotection. A Phase 2 study  (NCT00243932) showed that high doses of the enzyme were safe and well-tolerated, but there was no difference between placebo and coenzyme Q10 in improving function in ALS patients.

Omega-3 fatty acids

Omega-3 fatty acids are polyunsaturated fatty acids that are required for proper brain functioning and are known to reduce neuroinflammation. Research showed that a diet rich in omega-3 fatty acids could lower the risk of developing ALS.

Resveratrol

Resveratrol is a substance found in the skin of grapes and berries, including blueberries, raspberries, and mulberries, that has neuroprotective properties. A preclinical study in ALS mice showed that resveratrol treatment improved the survival of the animals. Another study showed that resveratrol treatment of stem cells from ALS patients helped them develop into healthy mature nerve cells, showing potential as a therapeutic for regenerating brain cells. No clinical studies testing resveratrol in ALS patients have been reported.

L-carnitine

L-carnitine is required for the transport of fatty acids into the mitochondria for processing and energy production. According to the results of a Phase 2 clinical study (EudraCT 2004-004158-23), treatment with acetyl-l-carnitine slowed the decline in functional ability and disease progression and increased patient survival in ALS.

Other supplements

Ginseng-treated ALS mice showed delayed symptom onset and increased survival. Similarly, ginkgo biloba prolonged survival and reduced motor neuron damage in a mouse model of ALS. But clinical studies have not evaluated the efficacy of these supplements in humans.

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