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Amyotrophic lateral sclerosis (ALS) is a progressive, neurological disease characterized by the degeneration of motor
nerves throughout the body. It is the most common motor neuron disease syndrome among adults, consisting specifically of a relentless and fatal wasting disease of the skeletal muscle. There are various causative theories currently being analyzed in clinical studies and there seem to be multiple factors at work (i.e. genetics, environment, cell dysfunction, and many others). Medical and/or pharmaceutical treatment options are limited, with Riluzole being the drug of choice.
However, Riluzole, the only FDA approved drug to treat ALS, has not been proven to consistently and effectively improve the quality of life in ALS sufferers. Other drugs such as Baclofen and Tizanidine have been used in practice, but both carry a risk of severe side effects. Various other medications are in the developmental stages. Researchers hope that stem-cell research will lead to an eventual cure.
Nutritional care and dietary recommendations are vital components in the treatment of ALS. Nutrition education is of utmost importance since early assessment and intervention are critical for maintaining nutritional health. The biggest concern is weight loss. The physical and psychological effects of the disorder make maintaining normal nutritional status very difficult. Since many clinical studies have indicated hypermetabolism (increased metabolic rate) in ALS, calorie and protein recommendations may be increased. Therefore, the ALS patient must be instructed to focus on diet because the lack of intake of essential nutrients, especially protein and antioxidants, can lead to rapid skeletal muscle wasting, lower quality of life, and shortened life expectancy.
In addition to maintaining adequate intake of calories and protein, other nutrients may help improve the quality of life and prognosis for ALS sufferers. As mentioned, it is important that patients are educated regarding the importance of their diet. It is also the responsibility of health professionals and caregivers involved with ALS to recommend nutritional supplements that have been proven, in clinical studies, to be beneficial. It is apparent that some individuals with ALS are utilizing nutritional supplements in their fight against this terrible disease.
With that in mind, we want to use this section of www.enhancedlifesciences.com to explore, present and explain information on many of these nutrients. We will discuss what they are, where to get them, how they work and results of clinical trials if applicable. We will also focus on some of the recommended diets and foods for people with ALS. Many of these nutrients actions within the body and muscles are very intricate, so we will do our best to use layman's terms, keeping this section of our Website easy to follow and understand.
Initially, we will discuss the most widely used supplements in the treatment of ALS. Some are still in the preliminary stages and some have been solidly proven to be effective in improving quality of life. As with any supplement, we strongly encourage individuals to consult their physician before commencing any supplementation program. If you have any further questions about these supplements, please contact ALS at: firstname.lastname@example.org
The most commonly used supplements are:
Vitamin B12, also known as cobalamin, is a water-soluble vitamin containing cobalt. The active form of B12, methylcobalamin, has been shown in recent studies to protect against the adverse effects of glutamate toxicity. Glutamate toxicity is the increased concentration of glutamate in the brain and it may be a leading cause of ALS. Vitamin B12 binds to and destroys glutamate by metabolizing it; therefore, B12 supplementation could be effective in slowing the progression of the disease. Recent research has shown that increased levels of B12 in the body protects against certain neurological disorders, including ALS. Levels of B12 in the body decrease with age; therefore, supplementation should be considered for all individuals, as they get older.
Vitamin B12 is mainly found in animal products. Oysters and Alaskan crab are excellent sources of B12. Smaller amounts can be found in ground beef, Atlantic salmon, eggs, and chicken.
Food Vitamin B12 (mg)*
Oysters (6) 14.34
Alaskan crab (3 oz) 9.78
Ground beef (3.5 oz) 2.90
Atlantic salmon (3 oz) 2.38
Egg (1) 0.56
Chicken (1/2 breast) 0.29
Nonfat milk (1 c) 0.10
20 to 60 mg/day
Vitamin B12 is not known to be toxic.
Zinc is a trace mineral and is part of more than 200 enzymes involved in everything from carbohydrate metabolism to protein synthesis. It is essential for the antioxidant enzyme superoxide dismutase; therefore it is thought to play a role extending cell life. Extending cell life can drastically improve the prognosis of an ALS patient.
Meats, eggs, seafood, whole grains, nuts and legumes are all good food sources of zinc. Plant sources are less stable and are not absorbed as efficiently as animal sources. For example, when whole grains are processed, almost 80 percent of the total zinc is lost. Ground beef, wheat germ, and garbanzo beans are very good sources of zinc. Milk, chicken and fish are also good sources.
Food Zinc (mg)
Ground beef, broiled (3.5 oz) 5.36
Wheat germ, toasted (2 tbsp) 3.22
Garbanzo beans (1 c cooked) 2.51
Sunflower seeds (1 oz) 1.50
Chicken, light meat (3.5 oz) 1.23
Brown rice (1 c) 1.23
Nonfat milk (1 c) 0.98
Egg (1) 0.53
Cod fish (3 oz) 0.49
30 mg/day with 5 mg of copper, with food-copper aids in the absorption of zinc.
High dosages of zinc (100 - 300 mg/day) are associated with copper deficiency with symptoms being anemia, impaired immune function, and an adverse effect on the ratio of LDL to HDL cholesterol. High intake of zinc causes nausea, vomiting, bad taste, and discomfort in the mouth and throat.
N-acetylcysteine (N-Acetyl Rx) is derivative of the amino acid cysteine and acts as an antioxidant in the body. It has been shown in numerous studies to prolong survival of motor neurons and may delay the onset of motor impairment. In a recent study published in the Journal of Neuroscience, N-acetylcysteine was shown to reduce motor neuron loss, increase muscle mass and muscle fiber area, and, most significantly, increase functional capacity of the limbs (as compared to an untreated group). Further studies are currently in progress.
NAC is a form of the amino acid cysteine, which is found in animal products and soybeans. The best sources are chicken, soybeans, beef, eggs and dairy products.
Food Cysteine (mg/Serving)
Chicken, roasted, flesh only (1/2 breast) 318.2
Soybean, green, cooked (1 c) 203.4
Beef, lean (3 oz) 190.4
Egg (1 large) 144.5
Peanut Butter (2 tbsp) 116.8
Milk, whole (1 c) 73.2
Cheese, cheddar (1 ½ oz) 53.2
600 mg, 3 times/day
NAC has been in use for over thirty years with few side effects (mild side effects include nausea and skin rash). No side effects were reported in patients taking as much as 6,000 to 8,000 mg/day for one year.
Free radicals are highly unstable molecules that can cause serious damage to cells in the body. Damage inflicted by free radicals is linked to many degenerative disorders-including neurological diseases. Free radicals attach themselves to unpaired oxygen molecules in the body and proceed to cause damage to cells.
Antioxidants prevent free radicals from combining with oxygen, and, therefore, prevent free radical damage. Antioxidants include: vitamins A, C, & E, coenzyme Q-10, alpha-lipoic acid, glutathione, melatonin, and superoxide dismutase.
Recent studies suggest that free radicals in the brain may play a key role in the development of age-related neurological disorders such as ALS. These studies have also indicated that an age-related decrease in the body's production of antioxidants may be evident. Antioxidant supplementation may help reverse this process and help combat the proliferation of free radicals.
FOOD SOURCES/RECOMMENDED DOSAGES
Rich sources of vitamin A are found in bright orange and yellow fruits and vegetables. Liver, eggs, and fortified milk are also good sources.
Recommended dosage: 800 - 1000 RE (RE is a measure of the vitamin A activity in foods and is used in assessing content in foods and supplements.)
Safety: there are no known serious adverse effects. High doses may cause hypercarotenemia (an orange/yellow tint of the skin).
Excellent vitamin C sources are: papaya, strawberries, oranges, broccoli, mango, and brussel sprouts. Vitamin C is very sensitive to light and air, cooking, and prolonged storage.
Recommended dosage: 1000 mg, 3 times/day.
Safety: vitamin C is considered safe even at high doses. Several studies supplementing up to 10 g/day (10 times the recommended amount) for more than one year or up to 5 g/day for three years reported no serious side effects.
Vitamin E is a fat-soluble vitamin, which means it must be ingested with fat in order to be absorbed in the gut. It is also important to not that the body absorbs natural vitamin E (from food sources) better than supplement forms. Therefore, the recommended amounts are higher for supplemental vitamin E compared to dietary vitamin E. The best sources of vitamin E are wheat germ oil, sunflower seeds, sunflower oil, and hazelnuts.
Recommended dosage: 800 IU, 3 times/day.
Safety: a study that administered daily doses of up to 3,200 IU for up to five years did not show any short- or long-term adverse effects.
Alpha-lipoic acid may be the most powerful antioxidant produced by the body. It can boost levels of other antioxidants in the body and can also recycle them-reducing the amount that needs to be consumed. Alpha-lipoic acid is also said to "turn-off" the "bad" genes that cause aging and, even, cancer. It may play a role in enhancing immune function and, more importantly for ALS sufferers, protecting neurotransmitters. Alpha-lipoic acid protects intra- and extracellular compartments by attacking free radicals that cause irreparable damage to cells.
Alpha-lipoic acid has been shown to stimulate nerve growth factor synthesis and secretion in astrocytes. Astrocytes are cells that protect the structure of nervous tissue. Alpha-lipoic acid's other uses include: cataracts prevention, decreasing stroke-related injuries, treatment of poison mushroom toxicity, and prevention of complications associated with diabetic neuropathy.
Yeast and liver.
100 - 800 mg/day have been used in most studies. It is easily absorbed; therefore the lower end is probably adequate.
Doses up to 2,000 mg/day have been well tolerated with few reported adverse effects. Side effects have included allergic skin reactions and hypoglycemia (lowered blood sugar levels).
Coenzyme Q10 (CoQ10) or ubiquinone is an electron and proton carrier supporting the production of ATP in the mitochondria (powerhouse) membrane. It may be beneficial in protecting and preserving motor neurons in people with ALS. It is synthesized in the heart, liver, kidney and pancreas. The CoQ10 content of tissue is most likely related to age, with the peak level at age 20, and subsequently declining.
It is a fat-soluble nutrient produced by the body and found in a variety of foods. Because it is fat soluble, CoQ10 needs a little oil or fat to be absorbed. In addition to its role in neurodegenerative diseases, CoQ10 has also been used in the treatment of heart disease and cancer.
Food sources of CoQ10 are mainly meat and poultry. Frying foods destroys 14 to 34 percent of the CoQ10 content, whereas boiling causes little to no damage.
COQ10(CO-enzyme Q10) (mg/Serving)
Beef (3 oz) 2.6
Chicken (3 oz) 1.4
Pork chop (3 oz) 1.2
Trout (3 oz) 0.9
Salmon (3 oz) 0.4
Orange (1 medium) 0.4
Broccoli (1/2 c) 0.2
300 - 1200 mg/day
CoQ10 has been apparently well tolerated for up to one year with no serious side effects. Some individuals reported mild gastrointestinal pain.
Creatine is synthesized from amino acids in the liver, pancreas, and kidneys at a rate of about 1 g/day. Ninety-five percent of all creatine in the body is stored in skeletal muscle. Studies have shown that increasing the amount of creatine in the muscle increases ATP production and, therefore, improves high-power activity and muscle growth. Obviously, this is important for ALS patients because muscle-strength retention leads to a better prognosis and quality of life.
In a study published in Nature Medicine, researchers discovered that creatine was more effective than Riluzole (the leading ALS drug) in extending the survival of mice with an ALS-type disorder. The supplemental creatine protected the mice from motor neuron loss and improved overall movement. This study proposed that creatine supplementation could help counteract the effects of ALS at the cellular level by slowing down the rate of cell death.
ALS patients are using creatine monohydrate widely. It usually comes in a white-powder form and can be mixed with juices or other beverages. Creatine's main function is to maintain ATP levels within the muscle. This is the energy that muscles use during anaerobic activity, such as lifting objects or weights. Creatine also promotes an anabolic environment in the muscle, which promotes protein synthesis that is important for muscle growth and repair. A number of years ago, ALS patients tried using creatine to maintain their strength and muscle energy levels. Many had good results. Some basic studies claimed a 10% increase in strength and possibly a slight slowing of weakness progression. Recently there has been speculation to possible neuro-protective properties of creatine as well. There are a number of clinical trials underway, for more information visit the ALSA web site.
Suggested use of Creatine for PALS is 5-6 grams a day. It is best taken in a beverage that has carbohydrates. It can be taken any time during the day and should be used daily. There have been no reports of adverse affects that we know of using this product, other than an occasional upset stomach and mild water retention.
A well-rounded, mixed diet yields approximately 1 g creatine/day. Most dietary creatine comes from animal products with only trace amounts available from plant foods.
Food Creatine (mg/Serving)
Herring (3 oz) 553 - 850
Pork (3 oz) 425
Salmon (3 oz) 383
Beef (3 oz) 383
Cod (3 oz) 255
Milk (1 c) 24
The FDA has advised consumers to consult a physician prior to using creatine. No serious side effects have been reported at dosages of 2 to 5 g/day. Some mild adverse effects may include mild diarrhea, muscle cramping, and dehydration. Increased fluid intake may be a good precaution to take when using creatine.
This was a brief overview of some of the more common nutrition supplements being utilized by ALS patients all over the world. It is important to learn about the fundamentals of nutrition and how these supplements can play a major role in the treatment of this horrible disease. One of the goals of Biomedic Labs is to inform ALS patients, caregivers, and health professionals about nutrition therapy so that the best supplements will be used.
Also, it is important to note that many of these nutrients are still being examined in clinical studies; therefore, new discoveries will be made and recommendations will most likely be adjusted. Be sure to check this Website frequently for the latest nutrition-related updates in regards to ALS.