Monthly Updates

Coenzyme Q10 Levels and ALS

Increased mitochondrial oxidative damage and oxidative DNA damage contributes to the neurodegenerative process in sporadic ALS.

A study involving 17 patients with sporadic ALS revealed the percentage of oxidized coenzyme Q10 and the concentration of 8-OHdG in the cerebrospinal fluid (csf) of the PALS was significantly greater than that found in 17 age-matched controls. The percentage of oxidized coenzyme Q10 in the csf was inversely associated with the duration of illness and the concentration of 8-OHdG in the csf was positively associated with the duration of illness.

The percentage of oxidized coenzyme Q10 was associated with the concentrations of 8-OHdG in the csf of PALS. The authors, who had set out to investigate whether mitochondrial oxidative damage or oxidative DNA damage contribute to the neurodegenerative process of ALS, conclude, "…both mitochondrial oxidative damage and oxidative DNA damage play important roles in the pathogenesis of sporadic ALS.
Murata T, Ohtsuka C, Terayama Y, Free Radic Res, 2008; 42(3): 221-5. (Address: Department of Neurology, Iwate Prefectural Ninohe Hospital, Iwate, Japan).

Editorial April 2008

This is a significant update insofar as it is the anniversary of the 13th year since this website was first created. Back then I was proposing that using antioxidants might be a way to slow the oxidation that had been observed destroying motor neurones in people with ALS/MND.

I have learned a lot since then. Whilst taking an appropriate combination of antioxidant compounds seems to slow the rate of neurodegeneration in some PALS, the use of antioxidants alone will not "cure" ALS/MND.

I researched ALS/MND for about a year after being diagnosed before gaining internet access. That means I deteriorated initially then improved and stabilised over that fourteen year period. Normally at this time I have a full neurological assessment but that was done a little earlier and with a different neurologist, who declared that he would not have guessed that I had once been diagnosed with ALS/MND. The annual checkup therefore seemed less relevant this year.

Other than extensive pain from nerve irritation/damage from an old spinal injury my general health, and particularly my neurological health, seems to be stable and about average for a man of my age. I would like to be able to exercise more than I am able to but for a person twelve years past their predicted "use by" date I am doing very well!

So much more is now understood about ALS/MND and gaining access to that knowledge is so much easier than it was 14 years ago that the future for PALS worldwide looks better than it has in the last 150 years. Sweeping changes have occurred and are still occurring in medicine with the mapping of the human genome and astonishing advances in molecular biology, stem cell research and other promising areas of research.

I would still recommend a regimen of antioxidant supplementation, improvement of liver function and increased hydration for any PALS. It seems to have worked for me and the several hundred others that have contacted me to say they have followed a similar approach and benefitted.

As always, I believe that a cure for ALS/MND is close or may even already exist and only needs to be made generally available. Until then, keep on keeping on.

Steve Shackel 1st April 2008

Abnormal Gene: Toxic Cause of ALS/MND

Australian scientists headed by Prof Garth Nicholson from the ANZAC Research Institute believe they have discovered what causes ALS/MND.

An abnormal gene has been shown to kill the nerves from the brain to muscles in the body. It was found that a protein called TDP43 was present in large amounts in the spinal cords of people with ALS/MND and dementia, which can also occur with ALS/MND.

It was not known whether the TDP43 protein was trying to help the body recover from the disease or was actually poisoning the body. In families that have ALS/MND with a mutation in this gene, TDP43 seems to cause ALS/MND.

Many people have thought that ALS/MND might be caused by an environmental poison but it has been demonstrated that it is a poison in the body itself actually becoming dangerous and leading to the death of motor neurones.

If this is indeed the protein that's causing damage in the majority of patients with Motor Neurone Diseases, reducing the TDP43 protein could potentially prevent or cure the disease.

As ALS/MND is probably many diseases with wide variations this may reflect all different varieties of ALS/MND with different mechanisms. But if there's an underlying mechanism that's common to all, and because this protein is generally found in all MNDs, this does offer hope that a general treatment can be found for all types.

For more info and interview with Prof Nicholson.

This is wonderful news and Prof Nicholson, et al, are to be congratulated but, for the record, I would be astonished if this protien alone is the sole cause of such a variety of neurological symptoms. Steven Shackel March 2008. [See My Theory for why I think this is so].

Statin Cholesterol Lowering Drugs May Cause ALS/MND

Neuroscientist, V. Meske, reported in the European Journal of Neuroscience a study about the ability of statin drugs to cause neuronal degeneration. Statin drugs are designed to inhibit cholesterol synthesis [in the liver] by their effect on the mevalonate pathway. A consequence of the inhibitory effect of statin drugs on the mevalonate pathway is the induction of abnormal tau protein phosphorylation.

Tau protein phosphorylation goes on to form neurofibrillatory tangles, long known to be the prime suspect in causing the degeneration of Alzheimer's disease. Scientists are finding that this mechanism appears to be true for ALS and many other forms of neurodegenerative diseases.

Statin drugs include Vytorin, Lipitor, Zocor, Crestor, Mevacor, Pravachol and Lescol.

Information on statin drugs and reported side effects.

Lithium to Treat ALS/MND?

Lithium could accelerate the removal mechanisms of protein and altered mitochondria and promote the genesis of new mitochondria. The drug used in a small pre trial group (lithium carbonate) costs very little. It was combined with Rilutek/Riluzole in a small n = 16 test group but complete remission was achieved by some subjects. Those in a control group taking Rilutek/Riluzole alone did not improve.

ALS/MND Information Website

There is some interesting information on using lithium to treat ALS/MND at http://www.alsforums.com/forum/showthread.php?s=34dde98a9381a0cecfa4f81bb314b888&t=4070

and http://www.als.net/forum/topic.asp?TOPIC_ID=2080&whichpage=1