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Some thoughts about how the disease seems to be spreading.


1. The disease appears to move from one cell to the neighboring one
, instead of starting in different cells individually. This is apparent from the way it starts in a specific location and marches from there on.

2. Thus, irrespectively of the actual mechanism that kills the cell, there must be an extracellular factor that causes the spread to the next cell.

This extracellular factor can be either

3a. an externally induced poison that causes the motor nerves to die as it spreads to new locations within the CSF;

or 

3b. a substance produced by the dying cell, maintaining the neurodegenerative cascade by causing the neighboring cells to die, leading to more of the substance being released in the CSF.

4. To stop the disease from spreading, all that is needed is to remove the substance mentioned in points 3a and 3b. We don’t actually need to know what kills the cell.
5. The substance can be removed either by finding a chemical/biological pathway that can be utilized to destroy it, or by replacing the CSF with one that is free of the substance.
6. In order to find out if there is a method to be found to rid the CSF of this substance, it must first be recognized.
7. The recognition can be done by extracting samples of CSF from an ALS patient at a location where there are damaged motor neurons and a location where the damage has not yet spread, and examining meticulously all differences in the composition of the two samples.

I would not be surprised if it would turn out that the said substance is a component of the immune system, but it could be something else as well.

 

The 3a route could be due to e.g.

-a leaking BBB allowing the entrance of a substance in blood that is toxic to neurones: the leak starts at some position and over time, the toxin gets access to an increasing area
-a pathogen such as the Borrelia (Lyme) spirochete
-BMAA, although that would require an explanation as to the localized manner of spreading; perhaps combined with a leaking BBB?

 

A very informative web page on the topic of spreading: http://alsn.mda.org/article/why-does-als-spread