What is your opinion PLS or ALS?

[Mike in Maine]

Let’s begin by saying I could have easily put this under the “PLS/ALS Association” thread but after much thought decided to start a new thread. As a matter of fact looking back in the pages of the PLS forum there are many threads that debate the PLS/ALS relationship question. So this is just another in a long line, expect I’d like to add my own personnel twist to it (PALS please don’t tell me to move this to DIHALS).
A little back ground before we get started, my neurologist, maybe the best in the Great State of Maine when it comes to ALS, he believes that all Motor Neuron Diseases fall onto a continuum with ALS at one end of the line and PLS at the other, and everything else falling someplace else on the line. He uses the terms so interchangeably that in the same appointment I have heard refer to my case as ALS, UMN DOM ALS, MND and PLS. As my Doctor tells me (at almost every visit),” it just a name, we treat the symptoms as they arise and we carry on. Stop getting hung up on the name”. One more data point, the Doctor I saw down at MASS General Hospital when down for my second opinion used the same scenario when explaining things. At an appointment yesterday my Doc used the following story to illustrate his point. He once had an ALS patient that came into his office with foot drop and three months later was homebound and almost locked in. He said we are both on the same road, just traveling at different speeds (May I add that I consider myself very lucky to be traveling at this speed). So I ask my Doc to put a name on what I have, his answer pick one, we’ll go with that, not the answer I was looking for!
So Shipmates (use Shipmates cause were all in the same boat), what would you call it ALS, UMN DOM ALS, PLS, MND or something I missed. I have all the signs of PLS Hyperreflexia , positive Babinsk sign, clonus, muscle fatigue, always tried could sleep 18 hours a day, El issues, voice is trashed, I have some issues swallowing and serve spasticity. If you were to rate the spasticity on a scale or 1 to 10 it would be 11 or 12, as Mike (I have PLS) says I get shortness of breath just getting dressed believed caused by the spasticity. I also have signs of LMN involvement, a dirty EMG not much, but bad enough that the VA said yup ALS here is your benefits, some muscle wasting in the left hand and calf with some weakness in both the left hand and foot (Muscle weakness and wasting have been very, very slow to develop) and I have fasciculation’s everywhere. So folks what name should I be using next to forum interest. Want to hear your opinions; I think most of you understand the why of needing a name. My Doctor is probably right, it is only name

 

[bigmark1954]

You present a good discussion.... I have almost identical symptoms to you Mike. My doc has diagnosed me as ALS because my EMG was dirty in both UMN and LMN.
I have been pondering that I might be more PLS than ALS. We share so many symptoms that are similar, I don't know where one can be distinguished from the other?
My voice is trashed, I get winded often just walking across the room, have atrophy in my hands and forearms& calves, extreme clonus in my ankles and hands, hyper reflexes, and fasics everywhere. I forgot spasticity mostly in my legs, and my balance is real bad....I have a tendency to walk like a stomped ant and bounce of everything, and swallowing issues.
WE NEED Nikki or professor Astugi(a loving nick name), to chime in and help us make sense of our Labels.:?:

 

[Clearwater AL]

Ok, help me out here... I thought, read and been told UMN does not show up on an EMG and muscles do not have spasticity if atrophy is present. Yea, Atsugi... jump in.

Mike in Maine, here we could go again, muscle wasting vs atrophy, progression of weakness to complete failure,
(The "It's not about feeling but failing" posted so many times). I have questioned concerning muscle failure... like one morning did a PALS wake up unable to move their arm or... did they notice any previous weakness before it completely failed.

I agree about "whatever name you want to put on it." But (big BUT)... in the later stages of progression it does make a big difference concerning certain benefits (outside the VA).

Neurologists not wanting to put a name on it should refer the patient to someone who has the guts to.

 

[vzandt]

My pals has exactly those symptoms ..except for arm muscle wasting. His legs are the worst. They are calling his ALS.

 

[Clearwater AL]

Vzandt, are you referring to muscle wasting or muscle atrophy?

PS. Sorry, I found where atrophy is often referred to wasting. Keeping abreast of all this can get confusing... varying terminologies. Some refer to muscle loss due to weakness / lack of use as muscle wasting.

 

[Atsugi]

The medical question would fit better in Nikki's bailiwick, but I can comment on the--important--administrative issues.

Although the doctor may not need a name--indeed, they treat the symptoms in this case--the government needs a name. On Medicare forms and most (all?) insurance forms, there is a spot for a diagnosis code.

Although VA has recognized PLS as a version of ALS, and given benefits, it was a lengthy appeal process to do this. In a manner of speaking, for VA purposes, you want ALS, not PLS or motor neuron disease. For VA purposes, too, you definitely want a death certificate to read "ALS." The law is very specific.

Social security gives priority to ALS, too. So the name is important. Note that the code must be 5 digits, including the zero, to mean ALS.

<codes deleted: ask your doctor for the correct code>

In my wife's case, we got each doctor to write a short letter of diagnosis, which was invaluable to us.

 

[vzandt]

His legs are getting skinny but they are stiff..not floppy at all. Lots of spasticity. Keeps him ( and me) awake at night. Poor balance...but...he's still stronger than me.

 

[vzandt]

Very slow progressing . Still eats..but voice is forced. Using bipap at night. Getting trilogy on Monday. Uses a walker to walk. A scooter outside...there are no walls to hold him up outside. Ridiculously tired all the time. Still working...but short hours and from home.

 

[Drewsmom]

vzandt - My Pals sounds like yours. Legs are the weakest. Muscle wasting in legs, arms, hand, and chest. Spasticity in legs. Positive babinski. He is coming home from rehab tomorrow. Permobil ordered. His official diagnosis remains PLS, but his therapists and rehab doctor felt strongly that it is ALS. Last EMG 5 months ago only showed reduced recruitment and positive sharp waves in the calf only.

Atsugi - The name is very important indeed. SS was awaiting to see if we got the "ALS" diagnosis. When we did not, they denied. So now I am trying to figure out how in the world to buy a vehicle to transport this expensive wheelchair, all the while wondering if the disability will ever come through and if we can hold out that long. It is such a shame.

 

[Clearwater AL]

Atsugi was right to delete the codes he posted. When I leave my Neuro's office on my discharge sheet there is a ICD-9-CM code. If you've seen a Neuro recently and still have your office visit discharge sheet... it might be on there. I believe the VA uses different codes. Maybe. It's info they are required to give you upon request.

Really confusing... symptoms maybe coded without diagnosis.

PS. I put the code name back after deleting it... my last sheet was under ICD-9. No secret.

 

[Atsugi]

VA uses the same codes as the rest of the US. They have just begun transitioning to ICD-10, which is vastly different from ICD-9.

 

[Mike in Maine]

For paperwork the Doc uses ALS, he has done time with the VA and understands the needs of the government for a name. It is just in our personnel conversations that I get the pick one answer. I guess for now I’ll keep my forum interest at PLS, due to the very slow progression. Due for an EMG in the spring and I’ll see how that one goes to base any further decision on.

Thirty years in the Navy and five years follow up in government service the answer for everything has for most part always been cut and dry, it is either yes or no. There has always been a publication, checklist or standing orders to follow, the Navy doesn’t do maybe very well, for the most part it is a very easy system to work and succeed in. Hence I don’t do abstract, I guess the burred lines in the symptoms of this monster are just one more burden that must be dealt with. It seems to be the one of the parts that I’m having the hardest time dealing with, maybe it I had a name I could better define the out years and what they will hold. I could deal with that. Just rambling

 

[IhavePLS]

Hi Mike,

Where you say:

"As my Doctor tells me (at almost every visit),” it just a name, we treat the symptoms as they arise and we carry on. Stop getting hung up on the name.”

With all due respect to your neuro, what he seemingly does not recognize is that different people will respond in a vastly different way to the statement: "You have ALS" or to "Pick one." Even though neuros may have confidence that ALS and PLS are part of disease spectrum, I expect that most "laypersons" do not have a true understanding of the relationship between them -- and therefore, the suggestion that "It's just a name" does not ring true with the anxiety-ridden person who is beginning to hyperventilate when the statement has been made "It could be ALS or PLS -- pick one" (and neither is the characterization terribly helpful to the individual's family or friends). "Pick a card. Any card" feels non-scientific and terribly arbitrary to me. I understand it, but don't agree with it -- particularly so for persons who have new onset of symptoms, and who do not have the same understanding that you and I have.

Given my own understanding of the ALS/PLS spectrum (I think Johns Hopkins has it right), it would seem that your neuro agrees with Johns Hopkins on this. But a little empathy -- both for knowledgeable AND less knowledgeable individuals who are suffering and who are looking for clarity -- would be helpful. "Pick one" serves only to instill greater fear and anxiety (and depression) in an individual who is already suffering physically and psychologically.

No matter the subdiscipline they might practice within, medical professionals need to be more empathetic (and sympathetic for the matter) when working with persons who are not "in the know." In your case, Mike, you have a more clear understanding of how ALS and PLS fit together on the spectrum -- BUT, I would point out, EVEN with your more advanced knowledge, you continue to question the issue. At best, you seem to be very uncomfortable with it - or at least you want more clarity. The problem is that your neuro cannot PROVIDE more clarity than he already has. And ambiguity in anything is never helpful.

Mike, with exception of voice and atrophy, I pretty much have the same sx. you describe above. My EMG showed evidence of fasciculations in my triceps and shoulders, but these were "clinically non-significant" (and thus, my EMG was deemed to be normal). You seem to have a lot more spasticity than I do as well, Mike, although I'm not sure if I can competently and objectively assess the level of my spasticity anymore, because I've become so used to it (that's a pitiful statement, is it not?)....

BTW, the diagnostic code my neuro has twice assigned to me is 335.24 (the DSM code that is specific to PLS).

 

[IhavePLS]

Hi again, Mike,

Just read your follow-on posting:

For paperwork the Doc uses ALS, he has done time with the VA and understands the needs of the government for a name....Thirty years in the Navy and five years follow up in government service the answer for everything has for most part always been cut and dry, it is either yes or no.

Your need for a clear-cut answer is understandable, given your military experience. I teach folks in all branches of the military, and understand precisely what you are saying here.

I'm actually glad to see that your doc has asked your opinion for purposes of formality (i.e., VA documentation). Many neuros do this -- and thankfully so.

Mike

 

[Dusty7]

The ALS, PLS, PMA Name Debate

Gowers, a founder of British neurology, stated in 1899 that he had never encountered a single case of PMA in which the pyramidal tracts (UMN) were unaffected. He did not think the introduction of the term “ALS” by Charcot (founder of Neurology) to be very helpful because it implied that the primary lesion was degeneration of the pyramidal tracts and that atrophy of anterior horn cells was secondary. He felt that Charcot's distinction in effect gave a new name to an old disease. He concluded that PMA, PBP, and ALS were "essentially one disease."

Brain in 1933 introduced the term "motor neuron disease" so that apparently different conditions could be brought together in a single general category and used the terms “motor neuron disease” and “ALS” interchangeably.

A series of studies have supported the opinion that PMA, PLS, and PBP were subsets of ALS.

Swank and Putnam in 1943 analyzed 197 patients. They considered PLS and PMA to be ALS that had yet to develop fully.

Lawyer and Netsky in 1953 did an analysis of 53 patients with ALS and concluded that motor neuron diseases form a group that includes PMA, PLS, ALS, and PBP.

Mackay in 1963 reviewed 126 patients with ALS and concluded that regardless of the onset, the spastic forms nearly always become atrophic, the atrophic also spastic, while the spinal forms nearly always become bulbar, and the bulbar forms, if the patients live long enough, become also spinal. The entire group is, therefore, best regarded as a single degenerative disease, ALS, which constitutes a spectrum of atrophic process at one end, spastic at the other, and both in the center. With time, cases at each end of the spectrum move towards the center.

This is where most neurologists appear to be today—with PLS (UMN signs only) on one end of the spectrum, PMA (LMN signs only) on the opposite end of the spectrum and ALS (UMN and LMN signs) in the middle, to which both ends are drawn.

Problems
1) MND, since Brain introduced the term has been used interchangeably with ALS which makes the term confusing at best.
2) The billing codes do not agree with current neurologic thinking. Instead
Amyotrophic lateral sclerosis is 335.20
Progressive muscular atrophy is 335.21
Primary lateral sclerosis is 335.24
And Motor Neuron disease is coded 335.2, which is different from ALS’s code, though the man who coined the term didn’t differentiate between the two.

The Real Problem
The real problem is that, while all the above diseases should be called ALS, the bureaucrats don’t understand that. ALS carries major benefits, while sometimes the terms, PLS, PMA and even MND may not. BUT THEY SHOULD. It is up to you to make sure your billing is coded correctly. Talk to your neuro about the coding problem and if he won’t agree with most other neuros that PLS, PMA and ALS are all the same thing (ALS) then keep fighting with him or find another neuro.