KETOGENIC DIET (MODIFIED ATKINS) USEFUL IN PREVENTION AND TREATMENT OF ALS

I was listening to a podcast this morning about how a high fat (95% of total calories) diet is being used to successfully treat epilepsy in children and adults.  As part of the interview, the doctor mentioned that a ketogenic diet is also being considered as a treatment for ALS, and may even be preventative.  So I googled it, and found these two articles.  Right click on the titles to see the articles and supporting information.

Abstract

Background

The cause of neuronal death in amyotrophic lateral sclerosis (ALS) is uncertain but mitochondrial dysfunction may play an important role. Ketones promote mitochondrial energy production and membrane stabilization.

Results

SOD1-G93A transgenic ALS mice were fed a ketogenic diet (KD) based on known formulations for humans. Motor performance, longevity, and motor neuron counts were measured in treated and disease controls. Because mitochondrial dysfunction plays a central role in neuronal cell death in ALS, we also studied the effect that the principal ketone body, D-β-3 hydroxybutyrate (DBH), has on mitochondrial ATP generation and neuroprotection. Blood ketones were > 3.5 times higher in KD fed animals compared to controls. KD fed mice lost 50% of baseline motor performance 25 days later than disease controls. KD animals weighed 4.6 g more than disease control animals at study endpoint; the interaction between diet and change in weight was significant (p = 0.047). In spinal cord sections obtained at the study endpoint, there were more motor neurons in KD fed animals (p = 0.030). DBH prevented rotenone mediated inhibition of mitochondrial complex I but not malonate inhibition of complex II. Rotenone neurotoxicity in SMI-32 immunopositive motor neurons was also inhibited by DBH.

Conclusion

This is the first study showing that diet, specifically a KD, alters the progression of the clinical and biological manifestations of the G93A SOD1 transgenic mouse model of ALS. These effects may be due to the ability of ketone bodies to promote ATP synthesis and bypass inhibition of complex I in the mitochondrial respiratory chain.

Ketogenic Diet May Prevent ALS Progression

April 18, 2006

The Mount Sinai School of Medicine study suggests a ketogenic diet high in calories may prevent the progression of amyotrophic lateral sclerosis. The study, which appeared in the April 3rd (2006) issue of BMC Neuroscience is the first study to draw correlations between diet and neurodegeneration (the progression of ALS).

�The findings assert the significance of certain high caloric dietary intake in the prevention of ALS. In view of any available therapeutic application for the disease, this new evidence might bring hope to those affected.�, said Giulio Maria Pasinetti, MD, PHD, Professor of Psychiatry and Neuroscience, Director of the Neuroinflammation Research Center at The Mount Sinai School of Medicine (MSSM) and lead author of the study.

The cause of neurodegeneration in ALS is unclear. The study researchers say mitochondrial dysfunction plays an important role in the degeneration of motor neurons. Ketones promote mitochondrial energy production and membrane stabilization. Mitochondiral membrane dysfunction, loss of oxidative stress control, generation of excessive free radicals, neurofilament accumulation, and excitotoxicity are all implicated in the onset of ALS.

About the MSSM Ketogenic Diet Study

MSSM investigators used a mouse model to examine the affects of a ketogenic diet (KD) on the progression of ALS. ALS mice were fed a high caloric ketogenic diet (KD). Motor performance, longevity, and motor neuron counts were measured in treated and diseased mice (both groups).

The study looked at how mitochondrial dysfunction plays a central role in neuronal cell death in ALS, the effect that the principal ketone body DBH has on mitochondrial ATP generation and neuroprotection.

Blood ketones were more than 3.5 times higher in ketogenic diet (KD) fed animals compared to controls. Ketogenic diet fed mice lost 50% of baseline motor performance 25 days later than the disease controls. The interaction between diet and change in weight was significant; KD mice weighed 4.6 grams more than the disease control group at study endpoint. In spinal cord sections obtained at the study endpoint, there were more motor neurons in KD fed animals.

MSSM Study Conclusions

This is the first study showing that diet, specifically a high caloric ketogenic diet may slow the progression of the clinical, and biological manifestations of ALS in a mouse model. This may be due to the ability of ketone bodies to promote ATP synthesis and bypass inhibition of complex I in the mitochondrial respiratory chain.

From me:

I think it is interesting that the food that I already eat and some of the supplements I already take have been researched as possible treatments and/or preventions of ALS, and I'm just now finding this out.

"Mitochondiral membrane dysfunction, loss of oxidative stress control, generation of excessive free radicals, neurofilament accumulation, and excitotoxicity are all implicated in the onset of ALS."

Most, if not all, of these things are or can be diet-related. A diet high in Omega-6s and low in Omega-3s, chemical preservatives, artificial sweeteners (aspartame/Splenda is an excitotoxin -I think about how much diet pop my mom drank), the antibiotics and hormones that are found in beef, chicken and pork, insecticides on our fruit and veggies...

The more I find out, the more I believe that eating clean may be the solution for our family.