PLS and Riluzole

[Clearwater AL]

"There is not much out there on PLS."

Jan, on the internet there are pages and pages concerning PLS,
from initial symptoms, to diagnosis procedures, to treating
meds.

It is very rare that a PLS patient is prescribed Riluzole
being that it's primary purpose is to slow the progression of ALS.
Even with that it's effectiveness is "somewhat" helpful.

Many major Neurological Centers/Sites also have considerable
info concerning PLS.

Take a look.

 

[lgelb]

Actually, Al, riluzole is not at all uncommonly prescribed in PLS, as this study, among others, illustrates. I picked this one because it has some good info on the PLS vs. ALS cohorts.

Jan, if you haven't seen it, there is also a sticky here on PLS/UMND.

Sorry that you find yourself here, but welcome.

Best,
Laurie

 

[Clearwater AL]

Laurie, the study (BMJ) you refer is only a publishing company. If you review
all the Editorial Board Members none are designated as Neurologists. Most are
European, Canada, Australia. Only one is from Sloan Kettering here in the
States and is not a designated Neurologist.

Rilizole has always been considered an oral med to somewhat slow the
progression of ALS, to somewhat slow the terminal outcome. Maybe you can
find something from Mayo, Duke, Mass General etc that states Riluzole has
any benefit to those with PLS only... which is not accredited to be terminal.

Maybe I missed a publication stating the effectiveness (purpose/benefit)
of prescribing Riluzole to PLS patients from any of the above and others.

As you said, "I picked this one..." If it is prescribed to PLS patients commonly
with out LMN deficits... what for? Tell us and for Jan too.

 

[Nikki J]

If you look at the authors they are ALS specialists except for a couple I think are statisticians. I recognized three by name without even checking Discounting their work because it was in BMJ ( the British equivalent of JAMA) rather than a specialty journal is like discounting something because it was published in JAMA or the New England Journal

 

[affected]

or dismissing because they are not American

 

[Clearwater AL]

Ok, I'll take that hit. But... where are the subject topics from Mayo, Duke
Mass General, NIH and other accredited Neurological Centers that
give any value to prescribing Riluzole to PLS patients?

And... don't those other countries have different classifications
concerning MNDs as compared to here in the States?

Again, I may have missed it but I couldn't find any.

Maybe those who communicate with Dr. Bedlack, from Duke, could
address this question to him.

As I asked Laurie... what are the benefits they, (PLS patients), would
receive from this oral medicine. Being that PLS is not really considered
terminal. Fortunately.

The initial intent of Riluzole was/is to somewhat slow the progression
for terminal ALS. Again... "somewhat" slow the progression.

Maybe Dr. Beddlack can give the benefit of prescribing Riluzole to
PLS patients.

If it significantly benefits PLS patients, I'd be all for it... for them and
any expense incurred.

 

[Nikki J]

many neuromuscular doctors believe pls is on the same disease spectrum as ALS. If you believe that then riluzole makes complete sense as it would slow an already slow progressing disease more. As I posted elsewhere at the MNDA Professor Al- Chalabi ( Kings London ) said riluzole slows disease by 38 percent and there were multiple studies confirming this. As I had never heard this I asked at MGH and was told yes this is true and the old 10 percent came from original studies which had a good number of endstage patients.

 

[Clearwater AL]

Nikki, once again I believe someone from Europe ( MNDA Professor Al- Chalabi ( Kings London )
where their designations of MNDs are different than ours.

I'm still interested in what Dr. Bedlack's opinion is.

Good discussion here... maybe some PLS members will chime in here and what
their Neurologists have said/prescribed.

If it significantly benefits PLS patients, I'd be all for it... for them and
any expense incurred.

Haven't some members posted they declined to take it? ???

 

[Nikki J]

The comment about Dr Al- Chalabi was not addressing ALS vs PLS but highlighting the fact that Riluzole has been found to be more effective than previously thought. confirmed by MGH where btw Dr Al- Chalabi did a fellowship.
I will also note that in places where they use the umbrella term MND they differentiate ALS and PLS clinically and tell patients what they have.

I will ask at mgh when I go ( not until spring) if they prescribe for PLS.

 

[affected]

Al I'm curious as I don't understand what you mean that 'outside the US other countries have different designations of MNDs.'
Here in Australia we call ALS MND, but we call PLS PLS, or we say the person has MND (ALS) or MND (PLS).
We also designate onset.
What differences are you talking about Al?

 

[affected]

Snap Nikki! I was writing much the same at the same time as you were

 

[lgelb]

As seen in the study I referenced and others, PLS may not be as swift as "classical" ALS in terms of progression, but does confer considerable disability over time, and, in one cohort, resulted in 33% mortality over the study period. The "non-fatal" designation relates to dying of something else first, not that the disease stops.

Thus, it's not outlandish to think that it would be prescribed for PLS, e.g. in 55% of cases as in this paper. The guidelines that recommend otherwise are based on data that was simply not powered (did not have enough observation years/patients) to justify this, since UMND ALS is rarer and slower. In fact, AAN panel members acknowledged "no evidence" of benefit, a more negative way of saying the same thing. There have never been any studies concluding a lack of benefit.

More recently, Dr. Bedlack co-authored a paper last year suggesting with all the accumulating evidence, that "PLS" and "PMA" simply be considered UMN-dominant and LMN-dominant forms of ALS, respectively. Under this PoV, it's even more likely that riluzole will be prescribed in these variants in future.

 

[old dog]

At 81, I've pretty much given up that anything will come along soon enough to benefit me. My neurologist describes me as a typical case of PLS. (if such a thing exists) He told me about 4 years ago that he would prescribe Riluzole if I would like to try it. I said no, because of the cost and that I had read the benefit was very limited. Evidently, Riluzole has been found to be more effective than originally thought. I doubt that it would be of much help to someone like me who was diagnosed in 2011.

I've lost my voice and can walk about 30' with a rollator-type walker. Occasional physical therapy sessions help me keep going. I don't have breathing problems but seem to be subject to lingering coughs if I have a cold or sinus infection. Fortunately, I haven't had either in a couple of years.

 

[lgelb]

Glad you are keeping on, old dog!

Best,
Laurie

 

[old dog]

Thank you!