UMN Dominate ALS and PLS

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Mike in Maine

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PALS
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10/2013
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US
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PA, No longer live in ME
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Pocono's
The last two Doctors I have seen, my normal Neurologist and a ALS specialist used UMN Dominate ALS and PLS inter-changeable, they both viewed ALS as continuum with PLS or UMN ALS at one end and UMN/LMN ALS on the other. What are your thoughts on this?

Thanks Mike
 
Hi Mike,

Many neurologists view PLS and ALS as being part of a continuum - which makes sense, of course. The Spastic Paraplegia Foundation states the following:

"PLS is caused primarily by degeneration of the upper motor neurons in the brain and spinal cord, which results in increasing spasticity and weakness of voluntary muscles. It is often referred to as a benign variant of Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig’s disease). [italics added]. The primary difference between the two is that in PLS the spinal motor neurons or lower motor neurons stay intact. Thus, there is no muscle wasting (amyotrophy), which is the symptom that ultimately causes fatal complications in ALS."

Source:

http://www.sp-foundation.org/understanding-hsp-pls/pls/

Mike
 
Mike
Thanks for the reply, so in your view do you take that to mean that UMN ALS and PLS are inter-changeable terms?

Mike
 
My neuro makes a distinction. He uses the term UMN dominant ALS. UMN dominant ALS does not mean no LMN involvement it just means delayed or slow involvement. That is why they wait for 4 to 5 years to call it PLS. My neuro only waited for a little over 2 years to declare it PLS. He said Bulbar ALS progresses quickly and I had no signs of LMN involvement. I have never heard him say UMN ALS without the word dominate. I asked him one time if ALS research applies to PLS. His comment was the cause of PLS is unknown and may be distinction ALS. I have heard of some neuros giving a diagnosis of ALS so their patient could immediately qualify for medicare.
 
Hi Mike,

No, I do not believe that PLS and ALS are interchangeable terms. Clearly, they are different, because both conditions have their own, specific diagnostic criteria -- e.g., EMGs are ordinarily "clean" in PLS (fasiculations are absent).

What the SPF is suggesting (and which I agree with, coincidentally) is that PLS and ALS are separate diseases that are part of a continuum.

For example, the colors red and yellow are each part of the color spectrum, which is a continuum. But red is not yellow.

Likewise, Hodgkin's disease can be viewed on a continuum (or "stages"). Stage I Hodgkin's disease designates less progression than does Stage IV Hodgkin's --- while both stages are part of the same continuum, they are very clearly different.

Therefore, there are similarities between PLS and ALS -- which renders some to consider them as part of a "Motor Neuron Disease" continuum -- but they also have distinct differences.

I would also suggest that the science is not evolved adequately, such that any clear distinction can be made -- which explains why the time to PLS diagnosis is often extensive....PLS is often later determined to be ALS (and vice versa).

The bottom line: We really don't know.

Now that I've made the distinction as clear as mud, I hope it clarifies. :roll:

Mike
 
Mike,

To muddy the waters even muddier, I was reading the other thread here on VA and PLS, and in doing so, went to the Johns Hopkins Neuro website, where it it stated:

"Primary Lateral Sclerosis (PLS) - a progressive neurological disease in which the upper motor neurons (nerve cells) deteriorate. If the lower motor neurons are not affected within two years, the disease usually remains a pure upper motor neuron disease. This is the rarest form of ALS [italics added for emphasis]."

I'm not quite sure how to interpret this, but it would appear to me that Johns Hopkins presently considers PLS to be a "form" of ALS. The full text is here:

ALS, Amyotrophic Lateral Sclerosis, Lou Gehrig's disease

Mike
 
Mike, in my case I had almost constant fasiculations in beginning. I still have them daily. They are most notable in my calf muscles but I have them all over. I also thought they only occurred in ALS but my neuro seemed unconcerned and reaffirmed the PLS diagnosis. He assured me I would be dead by now if I had ALS.
 
Our doctor at Forbes Norris told us my husband was UMN Dominant, and the life expectancy was 7 to 12 years, as opposed to 3 to 5 years. He has all typical signs of both UMN and LMN, however his breathing is still fairly good.
 
unfortunately for me, the breathing issue does not hold true in all cases. mine was already down to 60% at time of diagnosis.
 
There are two board members of this forum that were initially diagnosed with PLS that turned into ALS after several years. I don't know how much more proof you need than that.
 
My sisters husbands brother was initially diagnosed with PLS which also became ALS after
several years.
 
To simplify SSSI, Medicare and secondary insurance claims, you might ask your Doc to use the term Als in your files since it automatically qualifies,,whereas PLS is sometimes a battle
 
Hello TxRR,

There are two board members of this forum that were initially diagnosed with PLS that turned into ALS after several years. I don't know how much more proof you need than that.

I suppose you mean that the two persons you refer in your quote to actually had ALS -- i.e., they were at first incorrectly diagnosed as having PLS, but were subsequently found to have had ALS. Which is why so many neuros are reticent to diagnose PLS quickly.

This thread has convinced me that I don't know WHAT I should think and believe anymore :-? ...I really don't think we have any "proof" at all relative to whether ALS and PLS are distinctly different conditions (I doubt that, however), OR whether PLS is a variant of ALS and/or whether both are part the same disease spectrum. If neuroscience has such proof, they certainly haven't unveiled any of it. I'd suggest that the dialogue on this issue is proof that no one knows for sure. Following, is an excerpt from JAMA Neurology (JAMA is a premier, first-order, peer-reviewed medical journal):

"There is an ongoing debate as to whether ALS and PLS are 2 separate disorders or simply 2 points on a continuum of motor neuron degeneration. Le Forestier et al have observed electrophysiologic evidence of LMN involvement in their patients with PLS, suggesting that PLS is not restricted to the UMN. Their patients had an insidious onset with slow progression, and all but 1 of their patients progressed to debilitating spasticity, though they were free of marked weakness. These authors have argued that PLS may be a slowly progressive form of ALS in which the LMN is affected, but the process is slower or different."

Source (if you Google the first part of the article's title, you'll find the article 3-4 links down on the JAMA Network site):

Tartaglia, M.C., Rowe, A., Findlater, K., Orange, J.B., Grace, G., Strong, M.J. (2007). Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: Examination of symptoms and signs at disease onset and during follow-up. JAMA Neurology (formerly Archives of Neurology), 64(2), 232-236.

The above makes complete sense to me (I think so anyway!), particularly where the authors suggest that "PLS may be a slowly progressive form of ALS in which the LMN is affected, but the process is slower or different." In this respect, I'm inclined to think about Stephen Hawking, whose diagnosis is one of ALS, but whose outlier longevity is nothing short of astounding (he's now in his early 70's). Perhaps Hawking has a form of "UMN-dominant ALS"? Or perhaps he actually has PLS - and not ALS -- with LMN involvement.

It's interesting dialogue, TxRR, and I hope that science finds an answer soon. The literature I've read only appears to further complicate the issue.

Mike
 
,hi Mike
I have been chasing the same question for 7 years and as of yet I have no answer. You will beat your head against the wall out of frustration.there is no answer to that question. I seen two als specialists one says upper motor neuron predominate als and the other says plas. One said she wanted to follow my progression for 5 yrs before she would give me a diagnosed. Well the five years have come and gone and she very reluctently says als varient. The other specialist was comfortable saying umn predominate als. So right now I still don't know for sure. I have bulbar issues and walking issues. I have very slight muscle wasting in left calf only and yes I do have fasciculations. Some very violent. But. My fasciculations are not constant. They go crazy bodywide some times and other times they disappear completley for months after seven years I have no definate diagnosed other then als varient.
 
All

Thank you all for your responses, it is pretty much the way I figured, it boils down to who’s office your sitting in when the conversation is ongoing. I’m not too sure why it matters to me, except that people have an easier time understanding the 2 points on a continuum explanation. I guess I’ll continue to use that one. Again thanks for your input.
 
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