ketogenic diet as possible therapy for Lou Gehrig's disease

[hakimaj]

This study looked at a ketogenic diet in mice with ALS / Lou Gehrig's disease. It slowed the progression of disease and clinical symptoms.


A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosis.
BMC Neurosci. 2006 Apr 3;7(1):29 [Epub ahead of print] Related Articles, Links
Zhao Z, Lange DJ, Voustianiouk A, Macgrogan D, Ho L, Suh J, Humala N, Thiyagarajan M, Wang J, Pasinetti GM.

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 4.6g 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. CONCLUSIONS: 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.

PMID: 16584562 [PubMed - as supplied by publisher]

[TBoneMitch]

Wow, that's great info...even if it in mice, it is very encouraging...

Thanks for posting.

That and the 56 week keto diet in humans.

[Nancy LC]

There is a disease that some kids are born with here they don't create an enzyme needed to break down sugar. It leads to neurological issues and death eventually. Anyway, a ketogenic diet avoids most or all of the problems. I wonder if perhaps ALS is an adult onset form of this.

[southbel]

My grandfather died of Lou Gehrigs and it is an absolutely horrific way to die. It is a slow and agonizing paralysis of your body, until basically you are unable to breathe anymore due to this "paralysis". However, long before that, your quality of life is so severely depleted because of the shutdown of the motor functions, including the ability to swallow. Most people end up starving or dehydrating to death on this disease well before the paralysis reaches the lungs.

I find it encouraging that a ketogenic diet could have some effect, although the stated 25 days of "betterment" is not a great deal but I assume it would equate to something of a greater degree for humans? I know it took several years for this disease to kill my grandfather, but I imagine he would have welcomed even a few months less of the excrutiating pain he had to endure.