Hope for Amyotrophic Lateral Sclerosis (ALS) with the Keto Diet

Hope for Amyotrophic Lateral Sclerosis (ALS) with the Keto Diet

keto-diet-for-Amyotrophic-Lateral-Sclerosis-ALS

First used in the 1920s, the ketogenic diet is most known for its established efficacy in treating intractable epilepsy and is best known clinically for this role.  Unfortunately, the mechanisms of the ketogenic diet are complex and not yet fully understood.  Though, it likely involves metabolic adaptation in cellular signaling pathways, decreased neuronal excitability, and neuroprotection.  As such, it is likely that the ketogenic diet may prove to be an effective treatment even for neurological diseases, including Amyotrophic Lateral Sclerosis (ALS). 

Currently, the exploration into the therapeutic effects of a ketogenic diet on neurological disorders other than epilepsy is embryonic, at best.  Thus, their efficacy in treating disorders other than epilepsy in humans remains speculative.  Nevertheless, we will discuss the potential applications of the Keto diet  as a treatment for for ALS.  

 

Amyotrophic Lateral Sclerosis (ALS) Symptoms

While neurological diseases like Epilepsy, GLUT1 Deficiency, Manic Depressive Bipolar Disorder, and ALS have little in common pathophysiologically, they each feature abnormalities in cellular energy metabolism.  And, altered cerebral energy metabolism points to altered mitochondrial function.  Amyotrophic lateral sclerosis (ALS) is a rapidly progressive disease due to degeneration of motor neurons of the cerebral cortex and anterior horn of the spinal cord that is universally fatal.   It has claimed the lives of many, including physicist Stephen Hawking, heavyweight boxing champion Ezzard Mack Charles, and baseball legend Lou Gehrig who the disease is colloquially named after.

Most patients with ALS present with symptoms such as cramps, weakness, and muscle atrophy of the hands or feet.  Later, weakness progresses to the forearms, shoulders, and lower limbs. Fasciculations, spasticity, hyperactive deep tendon reflexes, extensor plantar reflexes, clumsiness, stiffness, weight loss, fatigue, and difficulty controlling facial expression and tongue movements soon follow. Other symptoms include hoarseness, dysphagia, and slurred speech. Then, as swallowing becomes more difficult, patients often choke on liquids.

Ultimately, death is most often caused by the failure of the respiratory muscles. 50% of patients with ALS die within three years of the onset, 20% live for five years, and 10% will have a 10- year survival rate. Survival for longer than 30 years is extremely rare [1].   Stephen Hawking lived with ALS for 55 years. 

In other words, ALS is a devastating degenerative disease with no known cure, and few treatment options exist. Treatments for ALS include glutamate blockers like Riluzole, and muscle relaxers are given to reduce pain and discomfort.   Like epilepsy, the exact pathological mechanisms that underlie ALS aren’t fully understood.   But what is known is that mechanistically, ALS likely involves oxidative damage, glutamate excitotoxicity, inflammation, and mitochondrial membrane dysfunction in nerve cells [2,3,4]. 

 

Amyotrophic Lateral Sclerosis (ALS) and the Keto Diet

 

 

ALS and the Ketogenic Diet

Individuals with the disease have a mutation in the gene encoding copper/zinc superoxide dismutase (SOD1) [1]. So when a transgenic mouse model of ALS was created in which the gene SOD 1-G93A was overexpressed, it led to progressive muscle weakness and death resulting from respiratory failure, similarly to what occurs in humans with ALS.  Studies on the mutant SOD 1-G93A mutant mice have given a lot of direction for future ALS studies, and treatment.

For example, in one study the SOD 1-G93A mutant mice were given a ketogenic diet and were found to have significantly higher motor neuron counts— and preserved motor function— compared to the control mice [5].  The authors also found that the mutant mice showed impaired electron transport chain function. However, administering the ketone body β-hydroxybutyrate restored activity in the neurons where electron transport chain function was either already decreased, or had been blocked pharmacologically [5].  While these results are indeed impressive, this study did not indicate an increase in lifespan of the mutant mice.   However, studies found that in the ALS mouse model, mutant mice fed a ketogenic diet variant called the Deanna Protocol (DP) showed delayed disease progression, extended the life expectancy, and demonstrated improved motor function compared to controls [6].

 

The Deanna Protocol

The Deanna Protocol is a program developed by Dr. Vincent Tedone, a retired physician whose daughter, Deanna, has ALS.  The Deanna Protocol (DP) calls for a ketogenic diet, and additional supplementation.  The main components of the Deanna Protocol include a ketogenic diet supplemented with Arginine alpha ketoglutarate (AAKG), Nicotinamide Adenine Dinucleotide (NAD), GABA, glutathione, Idebenone, and Ubiquinol (CoQ10), and medium-chain triglycerides (MCT).

Use of the ketogenic diet in the Deanna protocol are for the purpose of utilizing ketone bodies as energy substrates, thereby bypassing the rate-limiting transport of glucose to compromised motor neurons and mitochondria.  In ALS mouse models, this results in improved motor performance and control, delayed neurological deficits, and extended survival [4,5,7].   ALS patients on the Deanna Protocol anecdotally report increased mobility, strength, and gait improvements, as well as a slowed progression of the disease [4]. While further research is needed to elucidate the mechanisms underlying these results, the supplementation required for the Deanna Protocol is readily available over the counter, and the ketogenic diet is becoming easier to follow. These results are promising and carry the possibility that by raising ketones, the ketogenic diet and its variants in conjunction with supplementation as outlined by the Deanna Protocol may benefit patients with ALS. 

 

 


References

1. Hulisz, D. (2018). Amyotrophic lateral sclerosis: disease state overview. Am J Manag Care, S320-S326.

2. Vucic, S., Rothstein, J. D., & Kiernan, M. C. (2014). Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies. Trends in Neurosciences, 37(8), 433–442. doi:10.1016/j.tins.2014.05.006

3. Martin, L.J. (2011). Mitochondrial pathobiology in ALS. J Bioenerg Biomembr 43, 569–579.

4. Ari, C., Poff, A. M., Held, H. E., Landon, C. S., Goldhagen, C. R., Mavromates, N., & D’Agostino, D. P. (2014). Metabolic Therapy with Deanna Protocol Supplementation Delays Disease Progression and Extends Survival in Amyotrophic Lateral Sclerosis (ALS) Mouse Model. PLoS ONE, 9(7), e103526. doi:10.1371/journal.pone.0103526

5. Zhao, Z., Lange, D.J., Voustianiouk, A., MacGrogan, D., Ho, L., Suh, J., Humala, N., Thiyagarajan, M., Wang, J., and Pasinetti, G.M. (2006). A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosis. BMC Neurosci 7, 29.

6. D’Alessandro G, Calcagno E, Tartari S, Rizzardini M, Invernizzi RW, et al. (2011) Glutamate and glutathione interplay in a motor neuronal model of amyotrophic lateral sclerosis reveals altered energy metabolism. Neurobiology of Disease. 43(2): 346–55.

7. Zhao W, Varghese M, Vempati P, Dzhun A, Cheng A, et al. (2012) Caprylic triglyceride as a novel therapeutic approach to effectively improve the performance and attenuate the symptoms due to the motor neuron loss in ALS disease. PLoS One. 7(11): e49191.

 

 

Comments

comments

6 Responses

  1. Michelle Nelson says:

    My first symptoms of ALS occurred in 2009, but was diagnosed in 2011. I had severe symptoms ranging from shortness of breath, balance problems, couldn’t walk without a walker or a power chair, I had difficulty swallowing and fatigue. I was given medications which helped but only for a short burst of time, then I decided to try alternative measures and it has made a tremendous difference for me. I had improved walking balance, increased appetite, muscle strength, improved eyesight and others.

    • Maria says:

      Hi Michelle, I hope you are fine. Thanks for your comment. Could you provide more information of the alternative measures you’ve taken to improve your quality of life? My sister has been diagnosed with ALS and I’m trying to get informed about the disease and ways to help her out. Thank you in advance

  2. Henry Carlos says:

    I was diagnosed in March 2017 but was running around from doctor to doctor before I finally get a result that I was free from MND ALS. Mine started on top and progressed to the bottom I could walk very little but needed assistance as I have no balance. It is sad all the time that we thought this disease has no cure with all the technology we have while there are some formulas there that can relieve all symptoms and get rid of this of Amyotrophic lateral sclerosis (ALS). I’m passing this info to anyone at there because www multivitamincare org has the right cure and caregiver for this disease ….I took various supplements, medicine prescribed by a neurologist, massage, and physiotherapy still the disease was progressing very fast until the ALS formula from that company.

  3. Laurah Quayle says:

    This was great, I have been researching for a while now, and I think this has helped. Have you ever come across Healthherbsclinic com Amyotrophic Lateral Sclerosis HERBAL FORMULA (just google it). It is a smashing one of a kind product for reversing ALS completely. Ive heard some decent things about it and my buddy got amazing success with it.

  4. Laurah Quayle says:

    Hope this is allowed here if not I understand. My husband was diagnosed with ALS (amyotrophic lateral sclerosis) when he was 63 years old 4 years ago. The Rilutek (riluzole) did very little to help him. The medical team did even less. His decline was rapid and devastating. His arms weakened first, then his hands and legs. He resorted to a wheelchair (Perbombil C300). A year ago, I began to do a lot of research and came across Health Herbs Clinic, I decided to start him on the ALS herbal protocol as I had nothing else to turn to; 2 months into treatment he improved dramatically. At the end of the full treatment course, the disease is totally under control. No case of dementia, hallucination, weakness, muscle pain and he is even learning to Walk again. visit www healthherbsclinic c o m

  5. Ava Della says:

    I found an herbal doc online who helps people with (MND) motor neurone disease. Over 2 years my husband has been facing physical challenges with motor neurone disease, and recently he got healed with the help of dr agumba natural herb. I fully agree natural herbs are the best for every deadly disease. I’m so happy to see my husband in good health again. dr agumba contacts. WhatsApp/call.. +2349032173881 also here is his Email address; dragumbasolutioncenter@gmail.com

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