When muscles atrophy from ALS, are they "dead"?

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Buckhorn

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My subject line pretty much covers it. I cannot find an answer for this on-line. When muscle cells atrophy from ALS, are the "dead" forever? Are they just asleep? I know that the muscle atrophy occurs because the motor axons cannot deliver the "message" / impulses to the muscle any longer.

It was once thought that a person is born (genetically endowed) with all the individual muscle fibers they will have for life. (mcb.Berkley.edu - "Muscle fiber number is virtually fixed at birth"). What you do with them is up to you. Some people are naturally more muscular without having to even work hard at it.

I will do more research, but i was wondering what the collective thought of those who frequent this forum is.
 
To my thinking, they are dead. Kind of like if the cord for your toaster is cut, you still have a toaster, but it doesn't work. ALS works the same way, the first thing is the nerve cells die, then the muscles start wasting because they don't get the signal to contract. The wasting is aproduct of lack of use.
Vincent
 
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Interesting question.

"Dead" is not a good word for this. Dead flesh rots and causes blood poisoning. That isn't ALS.

The muscles in ALS still get blood and oxygen, they just don't get "fed" electrical signals. Could they "come back" like brown grass in the spring? I wonder.

I know that atrophy involves the muscle "straightening out" or relaxing, thus losing it's tone. So I wonder if the unused tissue doesn't suffer some loss of integrity once it's been unused too long?

Indeed, the ALS type of muscle wasting--neurogenic atrophy--is a special type of atrophy. Maybe someone smarter than me can inform us.
 
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Good thoughts Mike & Vincent. Mike, you're right - dead was perhaps not a great usage of words on my part. I know the "electrical impulses" that fire the muscle are not going thru, due to axonal or signal loss. Hoping some people with greater knowledge of physiology than me will weigh in on this. If not, I am certainly going to ask the neuro on our next visit.
 
I'm not sure exactly what you are asking, even after revising the word 'dead'.

Are you asking if there is any way they can be made to work again and reverse the atrophy, say if the ALS were cured?

As the nerve dies, the muscle wastes. By the time clinical weakness is able to be measured at least 70% of the signals to the muscle are gone. (this is why an EMG is not done too early, it will detect the ALS process before you even realise you are weak) A point is reached where the nerve no longer sends any signal and the muscle cannot move at all. The wastage or atrophy was already occurring before the nerve completely died, and continues afterwards.

While the muscles don't rot, their mass decreases. If a PALS isn't taking in enough calories, the muscles will waste even faster because the body will attack those wasting muscles to use the protein for the energy it needs.

We kind of call them 'dead' when they won't work, and it's probably as good a description as any.

I've waffled and not sure if I answered at all :shock:
 
I think Tillie pretty much covered it. When people have injuries or illnesses and experience muscle atrophy, they can rebuild muscle with exercise/PT because the motor neurons that power what's left still work. But a fiber that's gone is just...gone. So in ALS, the motor neurons die. The muscles just disappear.
 
I question the "muscle shrinkage" part. For example the muscle between my index finger and thumb does not seem like it shrunk but is gone. I cant imagine it shrinking from lack of use to the point where it doesn't even seem to be their?
 
Thanks for your replies. I guess I was trying to be a bit indirect in my question, because of the sensitivity of the topic. My question is, once the axon can no longer send the signal to the nerves to fire, is this permanent and irreversible, given today's technology? I am pretty certain that the answer is yes. Several websites I have visited state that the motor neuron is dead ...... so, once something is dead, that is it.

From ALS.org: ("The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost.

From pubmed: (Cell & Tissue Research: Axonal degeneration as a therapeutic target in the CNS) Axonal degeneration as a therapeutic target in the CNS. "In conclusion, data from ALS and motoneuron disease models suggests that axonal degeneration probably precedes motoneuron death. This obviously does not exclude the presence and negative additional impact of somatic dysfunction on disease progression".[/I]
 
Tillie - "Are you asking if there is any way they can be made to work again and reverse the atrophy, say if the ALS were cured"? Yes.
 
My neurologist said theoretically possibly yes She cited paralytic polio as an example where people did recover to varying degrees from a motor neuron disease. She was speaking in public and responding to a question asking essentially the same thing- though in broader terms. She definitely did not give an unqualified yes they would recover if there were a cure but it was not an absolute no either
 
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I think it's all interesting to discuss though :)

Yes the motor neurone is dead, but I'm not sure what we call the muscle.

Let's say someone devised something that could replace the nerve and start communicating with the muscle again - if it started to move and respond, maybe it could be built back up.

I think the point is that once the motor neurone is dead we have no way to bring it back to life, and I doubt that any cures they discover will ever be able to bring back a dead motor neurone. Maybe they can halt the process in a dying motor neurone and even see that recover, but ones that are dead seem far more doubtful.

But it's all hypothetical, until the cure is found we don't know what it may or may not be able to do, so I'm always open to anything! But from diagnosis day one, Chris and I both knew that no cure would come in however long he had left that would restore him to what he had been. He was already quite disabled and had stopped working by the time of diagnosis and was very rapid progression.
 
I think Max and some other pals tried a tems unit. I dont remember them being successful with it so I would not think impulses to the muscle would help.
 
The technology already exists (albeit, still in the developmental stages) to innervate lower motor neurons, via a brain/muscle interface.

“It’s much like a heart bypass, but instead of bypassing blood, we’re actually bypassing electrical signals,” said Chad Bouton, research leader at Battelle. “We’re taking those signals from the brain, going around the injury, and actually going directly to the muscles.”
The NeuroLife technology combines algorithms that learn and decode the user’s brain activity and a high-definition muscle stimulation sleeve that translates neural impulses from the brain and transmits new signals to the paralyzed limb."
(Google: BATTELLE NeuroLife, Ohio State University). This is just one example.

I am glad that scientists are working on exciting technologies that will someday help enable paraplegics/quads, and various patients with various forms of MND to move again. I don't delude myself that this will happen quickly, or benefit my husband. And I know that "classic" ALS, involving both UMN's & LMN's is much more complex than a spinal cord injury. But there IS a similarity in that persons with LMN symptoms only and spinal cord injured patients both lack the stimulus to the muscles. As Mike said earlier the muscle itself isn't dead; it is still receiving blood supply and oxygen. In ALS, muscles atrophy from the loss of "electrical" impulse to make them contract. Perhaps someday if motor neurons damaged by neurologic disease are still partially functional, or if the process destroying them could be halted or dramatically slowed, then it makes sense to me that, given time, muscle could respond and develop again. And the brain interface and nerve bypass technology is exciting as well. I have been thinking a lot about this lately and I am trying to be hopeful for the future for others who will be faced with this monster of a disease.

Goose, a TENS unit is not strong enough to stimulate denervated muscle; an electric stim machine must possess Galvanic current (or "Russian stim") to be stong enough to stimulate denervated muscle. (These type of machines are generally availble only to clinicians or physicians, although I won't go so far to say one would be impossible to obtain!) The pulse duration and intensity required to elicit a contraction from completely denervated muscle is not easily tolerated.
 
My mind just boggles at the things that innovative researchers can even think up, let alone put into practice!

I do wonder how complex this would have to be in order to innervate all the LMN a PALS loses. That's why it's best in the hands of these incredibly smart and innovative thinkers!
 
Tillie: "I do wonder how complex this would have to be in order to innervate all the LMN a PALS loses?" Yes, me too, although I think it would be similar to the interfaces that they are developing for quadriplegia or paraplegia. UMN symptoms would be another thing altogether (I think ...!), but as you say, it is best in the hands of the scientist and innovative thinkers. I am certain that this technology will be able to help many people in the future. Getting insurance companies to actually pay for it is another thing!
 
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