In a study published in the Proceedings of the National Academy of Sciences, when CoQ10 was administered to rats genetically bred to develop ALS, a significant increase in survival time was observed. After only 2 months of coQ10 supplementation, mitochondrial energy expenditure in the brain increased by 29% compared to the group not getting coQ10. The human equivalent dose of coQ10 to achieve these results was 100-200 mg a day. The conclusion by the scientists was: CoQ10 can exert neuroprotective effects that might be useful in the treatment of neurodegenerative diseases. 
This study documented that orally supplemented CoQ10 specifically enhanced metabolic energy levels of brain cells. While this effect in the brain has been previously postulated, this study provides hard-core evidence. Based on the types of brain cell injury that CoQ10 protected against, the scientists suggested that it might be useful in the prevention or treatment of Huntingtons and amyotrophic lateral sclerosis. It was noted that while vitamin E delays the onset of ALS disease in mice, it does not increase survival time. CoQ10 was suggested as a more effective treatment strategy for neurodegenerative disease than vitamin E because survival time was increased in mice treated with CoQ10.
About 95% of cellular energy are produced from structures in the cell called mitochondria. The mitochondria have been described as the cells energy powerhouse and the diseases of aging are increasingly being referred to as mitochondrial disorders. When coenzyme Q10 is orally administered, it is incorporated into the mitochondria of cells throughout the body where it facilitates and regulates the oxidation of fats and sugars into energy.
CoQ10 levels decrease with aging. Depletion is caused by reduced synthesis of CoQ10 in the body, along with increased oxidation of CoQ10 in the mitochondria. CoQ10 deficit results in the inactivation of enzymes needed for mitochondrial energy production, whereas supplementation with CoQ10 preserves mitochondrial function.
Further studies at Massachusetts General Hospital demonstrated that CoQ10 could protect against striatal lesions produced by both malonate and 3-nitropropionic acid. It extended survival in a transgenic mouse model of amyotrophic lateral sclerosis.  One study of 30 patients with ALS, however, found that serum CoQ10 levels were unrelated with the risk of ALS. 
Based on this very preliminary research, ALS patients might want to take 100 mg of an oil-based coenzyme Q10 supplement 3 times a day. CoQ10 absorbs best when taken with fat, so oil-based supplements of CoQ10 can markedly improve systemic absorption.
A study published in the journal Nature Medicine found the amino acid creatine more effective than riluzole in extending the survival of mice with an ALS-type disease. The scientists reported that with 1% creatine administration, survival was extended by 13 days, and with 2% administration of creatine, survival doubled to 26 days. These scientists note that riluzole alone extends survival rate by 13 days (in mice). The supplemented creatine protected the mice from the loss of motor neurons and improved movement. This study proposed that creatine could help reverse the effects of ALS at the cellular level. This is done by stabilizing the enzymes in the mitochondria, the powerhouses of the cell that store energy, thus slowing the cell death process. 
After taking creatine, patients with muscular dystrophy also showed a 10% increase in strength, according to a study in Neurology. 
Creatine is well tolerated explains Leon Charash, M.D., who chairs the medical advisory committee of the Muscular Dystrophy Association. Harnessing its apparent ability to buffer and stabilize the production and transportation of energy within cells could yield important health benefits for people with ALS and other progressive diseases.
Recent evidence has demonstrated a neuroprotective effect of creatine monohydrate supplementation in animal models of Parkinsons disease, Alzheimers disease, amyotrophic lateral sclerosis, and after ischemia. A low total and phosphocreatine concentration has been reported in human skeletal muscle from aged individuals and those with neuromuscular disorders. 
A study in the journal Neurochemistry showed that creatine significantly increased longevity and motor performance of transgenic mice with a superoxide dismutase mutation. Creatine also significantly attenuated the increases in glutamate measured with spectroscopy at 75 days of age, but had no effect at 115 days of age. The authors concluded that the beneficial effect of creatine might be due to an improved function of the glutamate transporter, which has a high demand for energy and is susceptible to oxidative stress. 
Another study found that oral administration of creatine produced a dose-dependent improvement in motor performance and extended survival in G93A transgenic mice. It also protected mice from loss of both motor neurons and substantia nigra neurons at 120 days of age. 
Creatine monohydrate (10 g daily for 5 days to 5 g daily for 5 days) was administered to patients with neuromuscular disease in a pilot study (n = 81), followed by a single-blinded study (n = 21). Body weight, handgrip, dorsiflexion, and knee extensor strength were measured before and after treatment. Creatine administration increased all measured indices in both studies. The authors conclude that short-term creatine monohydrate increased high-intensity strength significantly in patients with neuromuscular disease. 
Continue to Part 7 of the ALS Article