is a pls diagnosed just a shot in the dark......

[johnnyliverpool1]

.......so rare , does it even exist......what is it that finally convinces a neuro.....is it even a desease.....johnnywondering

 

[Tokahfang]

Ultimately, the truly convincing "test" is a very clear result that appears in your spinal cord upon autopsy. However, since none of us are rushing to get an autopsy...

PLS, in it's purest form, is very rare indeed. What convinces a neuro differs with how much of a purist he is and what country he is operating out of, but the phrase my neuro used to describe the situation was "examining the constellation of symptoms". There is also the interesting factor that you have to be cleared of anything else that could be causing it's symptoms, and sometimes that is not actually possible due to age or comorbid conditions.

Honestly, it is why I like to abbreviate what most folks in this forum are as "UMND" folks rather than "PLS" folks. I don't care myself why exactly your UMN's are bad, and docs can't be absolutely sure until you are dead, which is not particularly helpful to our living with this UMN damage. The symptoms are pretty much the same across the board, the treatment options are the same, the complaints and difficulties are the same.

PLS is a great name if you have to give a short answer to someone of why you are in a wheelchair, though. It is short, easy to remember, and if they look it up later they will get a fairly accurate synopsis of your symptoms.

 

[johnnyliverpool1]

beky. thanks....your brill........johnny

 

[billbell52]

What amazes me is the variation in the severity of symptoms. Some people are confined to a wheelchair after a few years and some can still use a walker after 20 years. It is hard to believe there are not different types of PLS. PLS affects about 50 people in the US a year. If they ever develop a test for PLS it will be interesting to see the results.

 

[dalvin]

according to the neuros at john-hopkins and mcv, pls is actually a very rare subform of als. since als manifests in so many ways it only stands to reason that pls would also.

 

[Tokahfang]

Another source of confusion is that there is a philosophical divide between neurologists on what PLS is more related to. Some docs, like Dalvin's, think of it as being related to the ALS family of motor neuron diseases. A lot of them are even willing to write things like PLS (form of ALS) on diagnosis sheets, and their patients get better benefits and easier approvals for help because of it. On the other hand, other neuros see it as being more closely related to the spastic paraplegia line of motor neuron diseases. These guys see much less connection between PLS and ALS, excepting only those cases where a "PLS" patient turns out to be a UMN-dominant PALS.

The philosophical divide gets less philosophical on the ground. In some regions, ALSA's chapter is associated with the guys that think PLS is related to ALS, and so they include PLSers in their loan closet. In that region, often the other guys will be associated with the MDA, who then won't help PLSers as not in their club. But it can be the opposite way in a different region, or you can get a region where neither of them have docs that see PLS that way, and then neither group will help you with PLS.

That divide even then shadows across the internet. With the exception of a few in both places, those patients who understand PLS as being in the ALS family tend to hang out at forums like this one and perhaps attend ALS support groups. Those who are introduced to it as part of the spastic paraplegia family are concentrated on the yahoo mailing lists and the social connections, conventions, and functions of groups like the Spastic Paraplegia Foundation.

Anyhow...

Billbell brings up an interesting point, the high variability in outcomes in PLS. I thought you might be interested to know, that is true also in HSP families. These are families all hit with HSP due to the same gene, yet grandma might have never needed more than a walker, mom was in a wheelchair, and the daughter has primarily pseuobulbar symptoms and walks just fine. They live the same place, have the same cause, but where and how badly the UMN's die is highly varied. In light of that, I don't find the spread of outcomes in PLS nearly as crazy.

 

[johnnyliverpool1]

the replies to my thread are the best ever...ta folks......gonna be a great day when they come up with cause and effect.......meanwhile, your thoughts put it in the right place......johnny

 

[dalvin]

I think this divide, as Beky mentioned is one reason it takes so long to get a diagnosis, on top of the fact that it is so rare that most neuros will never see a case.
p.s. Beky, if you dont mind me asking, I see your in Richmond too, who is your neuro? mines voda

 

[Tokahfang]

I've got a local PCP, but I haven't gotten a local neurologist. I'm at that point where I own or have loan closeted all the of the equipment I need, have established meds schedules and doses, and don't have much of a need for regular appointments. If I really need a good eye at something, I'd rather go back to my neuro in my previous state. I really like him, and I can't imagine anyone doing a better job. NY is only 6 hours away.

In case I decide it's too long a ride, how is yours?

 

[dalvin]

he seems to be both knowledgeable and caring. pretty thorough. I think he actually runs the als clinic at mcv

 

[Bad Balance]

My understanding is that while ALS is an automatic qualifier for disability and some benefits, PLS may not be.
Discuss this with your doc before he logs up all your files with PLS jargon.
I am not sue what the benefit really is to a PLS diagnosis which may devolve to ALS anyway ?

 

[Tokahfang]

PLS sounds better than a generic "spastic quadriparesis/plegia" when you need insurance to pony up for your powerchair and speech aids?

 

[Bad Balance]

ALS sounds even better and is on the list of automatic qualification

 

[IhavePLS]

Johnnywondering, late posting....late thought.....

Your wondering prompts me to respond: My personal sense is that having PLS -- whether it is or is not real -- is completely SURREAL.

 

[Bad Balance]

Surreal is a good description ...
My legs and balance were really off yesterday.
Returned from the ER at 4:30am.....15 staples....call me zipperhead