PLS to ALS?

[dkcarl62]

Some of you may have seen my posts in the PLS forum. I was DX at UoM in March 2015. I've had 4 EMG's since onset in 2012, 2 at UoM. Complete series of other tests at UoM also. EMGs have been clean, or "unremarkable".

I am in swift decline. In 3 years time, I am in a scooter/PWC 100% time. I can still stand and transfer. My voice is horse and my speech slowed. I have PBA. My balance is gone. My stiffness keeps increasing. I struggle to move in bed now. Most concerning is the atrophy. My toes and fingers are curling. I struggle to grip anything. I drop stuff. I see the atrophy in my calves. I think I see it in my forearms too. I've lost about 15 lbs since onset. I can still swallow without choking, but I have to chew slowly and carefully.

I thought I understood that ALS is apparent early on in an EMG - even before symptoms really develop.

I know at least one of you will suggest I ask for another EMG, but just based on my symptoms, does it sound like ALS to you?

Deb

 

[Nikki J]

Deb I appreciate you posted in DIHALS as that was essentially your question. I decided to move it because you do have MND and not all regular members read DIHALS.
To answer your question maybe and yes if you have not recently had one an EMG is in order. You are unfortunately very much in the window that PLS is a provisional diagnosis. The line between PLS and upper motor neuron dominant ALS can be very fuzzy. It sounds like you need an EMG and a thorough exam by your neuromuscular specialist. How much of your progression can be explained by UMN pathology would be determined by clinical exam as you know. I hope it is a lot!

 

[dkcarl62]

Thanks, Nikki, for putting my post in the right slot. Do you have statistics on life expectancy of UMN D ALS?

 

[Nikki J]

No I don't. My sense is it is slower. At least some of the longest lived PALS I know or know of are UMND. There is research showing the longer it takes to be diagnosed the better your life expectancy. Which makes sense. If your progression is really fast you get to the doctor faster and your symptoms are more obvious

 

[lgelb]

It is said to be slower on evidence to date, which makes sense. Here are a couple of abstracts supporting that statement.

Natural History of UMND ALS
Clinical Features (vs. PLS)

And this one reports on converting from "PLS" to "ALS" after as long as 10 years (4+ years on average). I don't want to unduly depress "PPLS" but it is best to know that late conversion is a distinct possibility.

 

[Mike in Maine]

Deb,
What you describe sounds just like the path I'm on, although I seem to be progressing a little slower. I have UMN ALS and that I have been told is the reason for the slow progression. I have gone from PLS to ALS my first 4 EMG's were clean. Then on the 5th I started to show signs on ALS. I have another EMG on 21 Dec very interested to see what it comes out as. But all the Doctors have said it is ALS.

Mike

 

[dkcarl62]

Very helpful links, thanks Laurie.

Thanks for your post, Mike. I'm happy to hear from someone else with my symptoms, but sad that we both find ourselves in this sorry state. Have you been given any life expectancy estimates?

I have mixed feelings about agreeing to another EMG. They are medieval torture. The results won't improve the quality or type of my care. My symptoms will continue to be treated with or without an EMG. An EMG might give me a new label and will satisfy the medical/scientific community need to prove their theory. Time alone will make the truth obvious. I will make a note to follow up with you after 21 of Dec.

Deb

 

[Mike in Maine]

Deb,
No doctor really wants to put a number on things but they do say it will be slow progression and that we are in for the long haul. Do you have a baclofen pump?

Mike

 

[dkcarl62]

Mike, no pump. I've seen pictures of those things and I don't think I can do it. Do you have one?
Deb

 

[Mike in Maine]

Deb
Yes one of the best choices I ever made. If your spasticity is bad I recommend it. The first month with it was tough but after that it has been a godsend. I couldn't make though the day with out it. Before the pump I had to use a cane to get around and that was limited. After the pump the cane is gone and I get around much better, the only thing I'm using is an AFO on my left foot.

Mike

 

[dkcarl62]

Mike,
Thats a pretty high endorsement for a pump! I'm glad it was so successful for you! I would need to be evaluated, of course, as I have quite a bit of atrophy in my calves, feet and hands. I had been getting around the house with a walker up until a few months ago, and now I'm pretty much scooter bound. I don't think walking again is in my future, even with the pump, because of the atrophy.

Deb

 

[affected]

I think it is irresponsible for neurologists to give a life expectancy - they really can't tell. You both know if you are progressing fast or slow at this point, but so many things are involved.

I know we want to know, I so often wished someone could tell me what was going to happen when, but the degree of difference between PALS is just incredible and not always obvious at the beginning.