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Tedstehr

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PALS
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08/2017
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CA
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BC
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New Westminster
I hope some of you smart people (you know who you are!) can help me understand what ALS is in laymen's terms. Here is what I think - your feedback and corrections would be great.

ALS is an over production of a neuro transmitting chemical that causes neurons to fire. This over production in upper motor neurons (between the brain and spine) causes excessive firing, which cramps or stiffens their connected muscles and causes lack of control. The excessive firing in the lower motor neurons (from the spine to the muscles) causes fasiculations and muscle wasting. And because of the free radical oxygen molecules present in the synaptic gaps where the firing occurs, the sparking oxidizes or burns out the gaps, causing neuron death.

ALS is when both the upper and lower motor neurons are involved.

Thanks for feedback all!
 
I've been wanting to ask this question... does ALS really begin in the Primary Motor Cortex? What part of the brain does it originate?

Mods... wasn't sure if this question fit here or should be a separate thread.
 
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Sounds related to me Al!
 
I am not sure that they really know all of this. There are theories but if we knew really knew the causes we would be closer to a cure.

Regarding hyperexcitability. My neurologist, an ALS expert, once said she was not 100 percent sure of it role. She thought it was bad but there was an outside chance that it might be neuroprotective.

Add to this that ALS is generally believed to be a cluster of diseases and what is happening will vary according to what is causing yours. I have a genetic defect called C9orf72. The pathology of c9 is still being discovered but appears to be toxicity to the cell, nuclear membrane dysfunction and loss of normal c9 function

It certainly true upper and lower motor neurons die and cause different symptoms.

Al, I don't know but it seems unlikely all ALS starts in the same place. UMND beginning, yes starts in the brain but surely people who start with PMA type LMN involvement first start in the LMN somewhere?
 
I'm not a neurologist, but I did go to medical school, so I'll put in my 2 cents.

ALS is triggered by unknown factors, most likely a combination of genetic factors (which is certainly the case in the familial forms) and possibly viral or toxic exposures. Once the process gets started, it sets off neural inflammation in the motor cortex (front part of the brain) which leads to degeneration and scarring of these motor neurons. In addition, neurons in the lateral corticospinal tract are also affected (the "L" in ALS). Sclerosis refers to the scaring process.

The result is that skeletal muscles don't get appropriate signals from their motor neurons, leading to muscle instability (fasiculations), weakness and eventual atrophy. The "A" in ALS stands for amyotrophic which means muscle atrophy. These are the lower motor neuron findings. Degeneration of the lateral corticospinal tracts leads to spasticity and hyperreflexia (upper motor neuron signs). Of course the full story is more complex than this.

If I had to explain it to a lay person, I'd say it is a progressive neuro-degenerative disease like Alzheimer's or Parkinson's (more people are familiar with those) of unclear cause, leading to death of the cells in the brain that control the muscles. Consequently the muscles weaken and atrophy, eventually leading to paralysis with inability to eat, speak, or breathe, and people die from this.
 
Whenever I read about the causes of ALS, it becomes really clear that the lead investigators--the smartest people in the world--are nowhere near understanding a singular cause of the disease.

Lots of clues, lots of leads, lots of insights--but nothing you can point your finger at and say: "This is the cause of ALS."

The onset of the disease is really different between different people, so there is no list you can read and say, "That is/is not ALS." After getting to see the disease first-hand, and meeting other PALS, and discussing it with other CALS, you get a sense of it.

That's just my view. There are plenty of people here who are 10 x more knowledgeable than I am, so I look forward to hearing from them.
 
Excellent information all, thank you. Karen, this makes sense but I need to study some of the concepts before I can wrap my head around it. The head MRI stated "subtle increased confluent T2 hyperintensity within the bilateral corticospinal tracts..." I am thinking this is related to your description. Of course I didn't go to Med school so there are lots of other concepts I will need to understand. But if you have a Diesel engine that won't start, I am your man!

I know this knowledge doesn't do anything productive against the disease but I have a thirst for understanding anything I am interested in - currently ALS. Go figure!
 
Hey Mike, I hope I haven't quoted this before, but my favourite brain quote is:

If the brain was so simple we could understand it, we would be too simple to understand it.

From my limited understanding it is not clear what is a symptom and what is a cause. So is the excess glucomate (spp?) a cause or a symptom. I also understand that cancer is not a single disease but a collection of diseases. As stated in this thread, ALS is the same concept.
 
Mike, I don't think there will prove a single cause of ALS any more than for a brain tumor or MS or any of the neurodegenerative disorders. Think of how many MIs or strokes there are in a year; we still don't know why some people have them and some people with identical risk factors (not that we know them all) don't.

In fact, while heart/circulatory system disease may seem unrelated, if you read the latest research, long-held paradigms like "lots of dietary saturated fat increases cholesterol and disease risks" are increasingly suspect. Inflammation is the newest [also oldest] bumper sticker but we don't know much about detecting those at risk in that sense, either.

In the case of cancer, we do surgery, chemo, XRT, immunotherapy all to treat, whatever the "trigger"[sic](s) were. We bypass damaged blood vessels. You can treat without knowing the "why" if you know the "how." Chasing a single ALS trigger is a waste of time IMHO, detracting from learning more about the "how."

Oh, and how did cancer surgery, chemo, etc. develop? Clinical trials, including one-offs -- a network of centers (disclaimer: I worked at one) whose mission it is to attack cancer on all fronts, even while lacking grand theories of disease in many tumor types. We let cancer patients risk getting worse; there is the money, time, staff and will to do that. That risk is very tightly mitigated in formal ALS trials esp. since a couple of trials did just that-- and with far fewer resources, there is not the share of voice to do for ALS what we have done in cancer [though not as much for the latter as some people think].
 
I am increasingly certain that ALS is an exaggeration of cellular stress response. For some reason (and the reason is different in different ALS subtypes) the stress granules do not break up within 10-15 minutes as they should but become permanent. And since TDP-43 remains trapped within the stress granules, it is unable to return to the nucleus to take care of its primary job: fixing errors in RNA transcription. This messes up the protein production in the cell, ultimately leading to its death.
 
There has been conversation here about both the cause and definition of ALS. While I cannot add anything to the definitions I can say that with the exception of the familial (genetic) type, ALS is caused by environmental factors. Why do I say this with such certainty? Look at the evidence. ALS affects a much higher rate of the population in the developed countries than it does the under developed, additionally rates of diagnosis have been increasing. This would suggest that the cause is something that only the wealthier nations posses and that it is something that has become more prevalent in recent times.

If I were working on an automobile and every model of a certain make had its battery go dead. I would first guess that I had a bad batch of batteries from the manufacturer so I would replace them from a different batch. But if the problem occurred again then I would conclude that the cause was not the battery but the voltage regulator or alternator. Further investigation might reveal that the batch of voltage regulators had been exposed to moisture causing corroded electrical connections that lead to failure.

In this analogy the problem occurs in one location due to an outside (environmental) issue. I suspect that if our "company" is honest with us we will find and environmental issue is the cause of ALS and it will be "recalled". I suspect though that our "company" will hide the cause because the recall will cost to much, even if a cure is found.
 
Another thought.....we have gone organic in our diet, exercise appropriately, etc. This has led to a large reduction in my sons ocd, adhd behavior. To me this is about the additives, pesticides, and exposure. I have always felt that the escalating neurologic disorders we face are very much environmental based.
 
I too think environmental factors play a huge role, but I also believe that stress is very likely involved as well. When I take a very broad look at "who" is being DX, (not including the familial type) I see those with high stress factors. You see soldiers, professional athletes, in Brian's case - business owner. Now that's not to say that encompasses everyone one who has been Dx, but I think it plays a vital role. Maybe environment + stress factors. I definitely think we use way too many toxins in our environment, on our food etc.

Like Gooseberry, I have noticed remarkable changes in going organic and of others I know who have as well and it's affect on medical issues.

Maybe someday we will know. However, I do think Pete has a good handle on the fact that getting those items recalled will take an act of God in many cases.

Hugs,

Sue
 
Trying again lost internet.
Great thread! Like ted I like to know how things work
As a totall med laymen my intel on als has come mostly from this site and my own experiences.
As I see it a life time of experiences most likely had a major influence on my Dx these include combat, work envio.extreme adrenaline rushes many toxic situations not to mention all the drugs and alcohol I put in my body.
I guess my brain gave up on telling my muscles what to do.
I like visualizing all the tech stuff on neurons and synapses.
Thanks , Chally
 
I do think that physical and mental stress are HUGE environmental factors. I also think the "company" that will never be held liable for their failures (and silent approval) is the FDA.

Our "poor" nutritional environment (I would even say poisonous) is a large part of the cause. The FDA to this date "approves" tobacco products. Even after they have been determined to be deadly. Yes the law suits against the tobacco companies almost bankrupted them but what about the FDA that approved cigarettes for consumption? Aspartame was once "outlawed" but based on Cokes protest and money was later approved. Yes with warnings but come on, they are either poison or not.

We will not get to the cause because the ramifications will be to expensive to the govt and some of their "partners".
 
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