Before heading out for the summer break, the team of Knowledge stops Dementia, would like to inform you about an exciting study result, which this time is not about dementia, but about a rarer, but very serious, incurable disease of the central and peripheral nervous system: amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease or ALS for short.
Every year, about one to two out of 100,000 people develop ALS. The onset of the disease is in most cases between the 50th and 70th year of life, younger adults are only rarely affected. Men are affected slightly more often than women (1.6:1). The incidence of ALS seems to be increasing worldwide.
In contrast to Alzheimer’s dementia, ALS affects almost exclusively the motor nervous system. Increasingly, it is primarily motor nerve cells (motoneurons) that are damaged – i.e. neurons that are responsible for the control and regulation of muscles and movements. Sensation to touch, pain and temperature, vision, hearing, smell and taste, bladder and bowel functions remain normal in most cases. Cognitive functions are also barely disturbed in ALS; mild impairments in mental performance, usually only detectable in special tests, may occur in some patients, but severe impairments are very rare. The course of the disease is characterized by progressive muscle weakness and is almost always terminal.
A similarity between Alzheimer’s dementia and ALS is that there is currently no drug therapy by which a cure can be achieved. As with dementia, drugs (such as Riluzole and Edaravone) only succeed in slowing the progression of the disease. Therefore, it is very important that all possibilities be explored that might provide a cure for this devastating disease, or at least a change in its time progression.
In ALS research, an animal model, a specially bred laboratory mouse for this clinical syndrome, is being used. In this model, it was shown that the disease process was significantly more favorable when extremely high doses of vitamin B12 were administered to the mice . Due to the protective effect of this vitamin on nerve cells, it has been considered as a candidate for treatment of ALS in humans.
Therefore, in a recent study published in the Journal of the American Medical Association, a Japanese research group investigated whether very high doses of vitamin B12 could also benefit ALS patients  .
The researchers administered either vitamin B12 in the form of methylcobalamin at a dose of 50 mg (intervention group) or a placebo (control group) intramuscularly twice weekly for a period of 16 weeks. The 130 study participants, all early-stage ALS patients, were randomly assigned to receive either the vitamin B12 or a placebo.
At the end of the 16-week period, all participants were subjected to a standardized test procedure, the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS – R), thus assessing the worsening of symptoms. In this way, the researchers wanted to find out whether the speed of clinical deterioration (progression) was reduced in the intervention group compared with the control group.
What the researchers found was quite remarkable: In those who received high-dose B12, the deterioration of the ALSFRS-R score was 45% less than in patients who received standard treatment such as riluzole and a placebo. Thus, ultra-high-dose vitamin B12 was able to significantly slow clinical progression in patients with early-stage ALS.
Researchers speculated on how exactly vitamin B12 exerts its beneficial effects in these patients. One possibility is that this B vitamin helps reduce homocysteine in the body, since vitamin B12 (along with other micronutrients) is necessary for the breakdown of homocysteine in the body. Homocysteine is an intermediate in protein metabolism that has neurotoxic effects. Homocysteine is an intermediate in protein metabolism that has neurotoxic effects. Furthermore, it contributes to the amplification of inflammation that can lead to the death of motor neurons when it is elevated. In fact, homocysteine levels are reported to be elevated in patients with ALS.
During the 16-week study, ALS patients in the intervention group experienced significantly less degradation compared to the control group. Also, the high dosage of vitamin B12 was shown to be safe in the study, but it was only for a four-month period. This result highlights how effective, but still free of side effects, therapy with high-dose micronutrients can be in patients. However, it equally emphasizes the importance of already being active in prevention and keeping homocysteine levels low through sufficient vitamin B supply. In addition to vitamin B12, vitamins B6 and B9 (folic acid) are also important for the breakdown of homocysteine. You are welcome to read more about this at our partner project of the NährstoffAllianz.