Is this right?

[KevinR]

 

[Nikki J]

Sorry not understanding your question? Is it the number of pls cases? Although pls is rare it is slower than als so the number of people living with it is higher than the percentage of mnd that you would expect if you just took a percentage of living with als
Here is a paper on it Primary Lateral Sclerosis: Background, Etiology, Epidemiology

 

[KevinR]

My mistake I don’t always say what I mean or trying to say. It’s my understanding that upper motor neuron ALS is not PLS. Tow separate diseases so to speak. I know it’s just a screenshot but it does say 13 out of 29 developed upper motor neuron als

 

[KevinR]

I asked for a second opinion and my nurse sent me that message. Reading between the lines she seems offended that I even asked. But I have literally had to keep declining before anyone would even listen. I have foot drop that I didn’t have a year ago. I’m on bipap at HS. They want to put a peg tube in me because of my weight loss and swallowing. As soon as I asked about a second opinion they brought up putting me back on the table for an emg. How much muscle loss does it take before any changes on my emg? I don’t even know if I trust them. Have witnessed health care decisions based on dollars more than once in my career.

 

[lgelb]

I'm sorry to hear about your decline, Kevin.

If a new diagnosis of ALS will affect Social Security benefits, disability insurance benefits from your past employer, or some other kind of insurance benefit like less hassle if you end up needing a wheelchair or hospital bed, or wanting to start one or more of the R drugs, then the EMG seems worth doing. From your description of your progression, I would guesstimate a >50% chance that a new EMG will show changes consistent with ALS.

If the diagnosis won't change anything from a benefits standpoint, it is your call whether you want an official diagnosis or not.

As you noted, the statement that PLS is UMND ALS is obviously incorrect.

 

[KevinR]

Thank you for your answers. I’m just so frustrated and my MH has really suffered because of how hard it is for me to get treatment. I would like to slow or delay the process if possible. Plus, I am a veteran so my wife will get survivors benefits.

 

[lgelb]

Oh, I forgot you are a vet. I believe an ALS diagnosis would facilitate your receiving treatment and adaptive housing benefits, and would check with your local PVA on that.

 

[rmt]

My husband was originally given a possible PLS diagnosis due to a clean EMG and bulbar only symptoms for 2 years. Depending on the doctor, some considered ALS a spectrum with PLS at the very end of the upper motor neuron side of the spectrum. We were told that most PLS cases eventually become ALS. I don't remember the actual percentages.

He was a veteran and we were lucky that a VA doctor was willing to give him an ALS diagnosis even with the clean EMG. The benefits were so helpful. Money and aid to get supplies and services. There is even a caregiver program (including a stipend) as more care is needed. For us n getting the ALS diagnosis as soon as possible was very helpful. I recommend you contact your local PVA (paralyzed veterans association) to get their help.

 

[Wilson2009]

No idea if this will help, but there is at least one relevant case where the Board of Veteran Appeals considered PLS a variant of ALS and granted service connection. See https://www.va.gov/vetapp14/Files2/1417108.txt and note that the vet was represented by the PVA.

 

[KevinR]

Reading between the lines. It feels as if money is the reason for withholding an ALS diagnosis or even a presumed ALS diagnosis? Which is all is needed for filing the claim. I currently have provisional ALS documented with my PLS. I think because I have waited almost a year to ask for a second opinion and they took notice. All doctors should welcome a second opinion. All nurses should always be a patient advocate. Only 4% of the US population has PLS per the nih.gov site.

 

[KevinR]

I used that and one more on my claim as a PLS variant to ALS. I was denied. I didn’t appeal because when I filed all gulf war presumptive’s I came in at 160%.

 

[KevinR]

Thank you! The only important part is survivor’s benefits at this stage.

Some changes that are confusing. I am right side dominate. You can see the change in my neck. When I swallow the right side does not move at all. I have an indention distal and proximal of my collar bone. The deltoid is almost gone. The right pectoral muscle is so small I now have the sagging skin under my arm. My lats go straight down. I have lost the V on my right side.

We were confident it was going to be upper extremity onset. But then I developed foot drop on my right foot. It caused me to start falling. You can see the difference around the knees and hip flexors. So what is it? Will this now show on an emg? You can actually see these differences in contrast of right and left side. With and without clothes. I was an exercise enthusiast for most of life and I never had a six pack until now.

 

[lgelb]

As I said, given the changes you've noted, there seems a good chance that the EMG will tilt toward ALS. ALS often begins on one side, which may be the dominant hand side or not.

The only way to find out is to have the test (I'm presuming that it's been more than a year since the last one). If you have believed that things would stay PLS, it's no small thing to confirm ALS should that be the case. But as you point out, it could carry benefits with it, and you would be in a better position to plan.

As for decisions based on dollars, recommending a new EMG really doesn't fall into that category, nor does documenting that PLS has become ALS, if that's the case. It is or it isn't. The dollars don't make it so.

If you had a hard time at the last EMG and let the team know, if you go that route, they can work out a plan to make it easier. You can also talk with a counselor about what this decision means for you.

I think what you mean is up to 4% of the population with motor neuron disease may have PLS.

The percentages that are later diagnosed as ALS vary by how well-documented the initial PLS diagnosis was. Some of the newer tests that suggest it are not available in all areas, so the diagnostic process is not even across all states or countries.

There are probably PLS patients that get lumped into ALS from the jump, and vice versa. But that is about technology and uneven access to expertise -- an accurate sorting hat, if you will -- not a profit motive.

 

[KevinR]

The dollar’s is because the ALS or presumed ALS diagnosis comes with a a VA compensation package that is a lot of money to the veteran. 100 K house remodel, 26K for automotive, extra special monthly compensation somewhere around 2K, wife will get a raise for caregiver support pay somewhere around 30K a year. All of this was told to me when I first filed for PLS variant. I am currently prescribed zero medications for motor neuron disease. So if you factor all that in it is healthcare based on dollars.

 

[rmt]

I highly recommend contacting your local PVA. They were amazingly helpful for us.