ALS or amyotrophic lateral sclerosis is a disease centered around the loss of functional mitochondria in motor unit neurons. As these nerve cells lose their ability to generate energy (that’s what mitochondria do) and therefore the ability to stimulate muscle recruitment (required for coordination, movement, and muscle cell preservation), individuals with ALS suffer muscle loss, paralysis, and eventually the ability to swallow and breathe.
Although there is a somewhat broad spectrum of symptoms initially among pALS (people living with ALS), the symptoms most often overlap with other neurodegenerative diseases such as Parkinson’s Disease and Alzheimer’s, as well as with other frontotemporal dementias. While the focus of this article is with respect to the etiology of ALS and the best possible dietary intervention, please keep in mind that the other neurodegenerative diseases also having growing bodies of clinical evidence that illustrate the benefits to a similar dietary pattern.
Neurons are very unique cells. Unlike our muscle cells they are less metabolically flexible and are dependent upon carbohydrates or ketone bodies (more to come on these later...) as fuel sources.Because of their requirements and because of what the modern human diet looks like these days, our brains and the neurons that comprise them are almost always running on sugar. Some sugars are better than others when it comes to the effects on the mitochondria of these critical cells. Glucose burns much more cleanly, for lack of a better analogy, than fructose.
Unfortunately more than half of most sweeteners and added sugars are made up of fructose. When it comes to corn-based sweeteners the percentage can jump to as much as 60% fructose. Many researchers have shown what fructose metabolism does over the course of time to the mitochondria of all cells, it is axiomatic to conclude that it can only be worse in those cells that are even more dependent on sugar metabolism as they will be getting a greaterpercentage of their energy from sugars overall. If this is news to you and you have been told that not only “a calorie is a calorie” but also that “a sugar is a sugar”, you will definitely want to take a look at the research around fructose and mitochondrial dysfunction:
Simply avoiding fructose is the first and most obvious step in giving our neurons and their mitochondria a chance to come up for air. That means not only avoiding sweeteners but fructose-rich fruits as well. Dried fruits and many fresh ones such as bananas, apples, and grapes are very high in their fructose content and should also be avoided with any neurodegenerative disease. I am always dismayed to find recipes included in dietary recommendations for pALS that are centered on chocolate syrup, ice cream, and numerous other rich sources of sugar. Not only will these recipes fail to prevent weight loss, they will also make ALS patients considerably worse.
The artificial sweetener aspartame is not a good choice either. There is a good amount of research to support the role of glutamic acid in some aspects of the disease. Aspartame contains high concentrations of aspartic acid and it can act and disrupt neuron function in a similar fashion to glutamic acid. Then there is the research illuminating the degeneration of aspartame to methanol and formaldehyde, also potent neurotoxins. Are stevia and xylitol safe sweeteners? Possibly, but they shouldn’t be part of the long- term plan.
The key to arresting the neurodegeneration is in providing the neurons with the only other viable source of energy: ketones. Ketones are generated when we consume small amounts of carbohydrate and moderate amounts of protein. With this type of food consumption, the body has to manufacture glucose in an effort to produce supplemental quantities. In the process, ketones are a byproduct that can benefit all cells in the body with an incredible amount of supporting research. If we eat too many carb- rich foods it will quickly take us out of this ketone-generating zone, as will too much protein.
Ketones can also be generated from certain foods that contain a unique type of fat. In fact, there are only two or three foods that most humans have exposure to at one time or another in their life that can offer ketones as a result of their medium chain triglyceride (MCT) content. Breast milk, goat milk, and coconuts all contain significant amounts of MCTs and therefore can offer ketone production even outside of the ideal parameters of a ketogenic diet.
It makes too much sense that breast milk would provide us with a source of ketones at a time when brain plasticity and development is at its high point. In a similar way it makes too much sense that a ketogenic diet or even a diet rich in extra virgin coconut oil could provide an alternative fuel source to neurons that are otherwise compromised and made worse by their dependency on sugar.
In addition to helping neurons find a more viable source of energy, ketones also signal the cell to initiate autophagy. Autophagy is eventually critical to cellular function. It is a cell’s recycling program where those internal components that are no longer working properly are digested and the proteins and other salvageable components are reinvested into new organelles.
Unfortunately it takes more metabolically extreme circumstances for autophagy to happen. A cell has to receive a signal that there is either a protein or an energy shortage. Ketones are associated with the latter even in the presence of significant quantities of dietary fat. This recycling process creates room in a neuron for new, more functional hardware and can even stimulate biogenesis of new, healthy mitochondria. No drug or medication has ever been able to accomplish that.
This an important foundational component to an ALS, as well as to other neurodegenerative diseases, dietary intervention: get the sugar and the carb bombs out, bring the high quality saturated fats in, most notable extra virgin coconut oil. Even if you are overwhelmed by the thought of a ketogenic diet, simply eliminating sweeteners, fruit juices, most fruits, and other concentrated sources of sugar should be easy to grasp. Replacing omega 6 fatty acid-rich polyunsaturated fats with high quality coconut oil, olive oil, or grass-fed butters is also an extremely worthwhile change. Significant quantities of omega 6 fatty acids generate more inflammation and incorporating more polyunsaturated fats into the mitochondrial membrane just creates more instability and more oxidative stress where it is the most detrimental.
Too often care providers are resistant or unwilling to recommend these high fat, carbohydrate restricted interventions because they are not familiar with the cellular pathways involved in neuron metabolism. Others confuse this dietary intervention and the mechanisms of ALS with “knowing the cause”. This therapeutic approach does not indicate that there is a proven environmental or genetic cause (although there are certainly some very plausible theories), only that there are definite pathologies in the mitochondria of pALS and that there are ways to improve the metabolic conditions within their neurons. Let’s not wait for another decade to pass before accepting a simple dietary strategy as at least part of the solution. No one should have to wait any longer as the research is becoming increasingly clear.
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